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CHAPTER 58

Congenital Heart Disease


Charles D. Fraser, Jr., Lauren C. Kane

OUTLINE
History and Other Considerations
Pathways for Practicing Congenital Heart Surgery
Anatomy, Terminology, and Diagnosis
Perioperative Care
Lesion Overview
Single Ventricle
Miscellaneous Anomalies
Summary

This chapter is designed to provide medical students, general venture inside the congenitally malformed heart. At first, the
surgery residents, and practicing general surgeons with a working parent was asked to serve as a biologic oxygenator using the tech-
tool to aid in their understanding of the features of anatomy and nique of controlled cross-circulation; soon thereafter, the mechan-
physiology in patients presenting for general surgical procedures ical, extracorporeal, heart-lung bypass pump was developed.2,3
in the setting of repaired or unrepaired congenital cardiac lesions. With the aid of this ability to support the patients circulation
The large scope and breadth of the evolving field of congenital during intracardiac exploration, surgeons have sequentially
heart surgery precludes an exhaustive treatise on all aspects of this attacked almost every described congenital cardiac anomaly. The
specialty. Several excellent and thorough textbooks of congenital prospect of meaningful survival for patients born with otherwise
heart surgery are referenced in this chapter, and the reader is devastating congenital cardiac lesions is now expected in most, if
encouraged to use them for additional in-depth understanding of not all, cases.
the lesions to be reviewed. A general surgeon practicing today As a result of this success story, there is now a large and growing
needs to be well versed in the basics of cardiac anatomy, physiol- population of adults with repaired or unrepaired CHD; estimates
ogy, and specific derangements associated with the various known in the United States for 2010 placed the number of adult patients
congenital cardiac lesions. Furthermore, few patients with complex surviving with repaired or palliated congenital cardiac lesions at
congenital cardiac lesions may be considered cured of their cardiac more than 1 million.4 There has been an increase of greater than
problem, even after successful reconstructive surgery. Thus, it is 50% in CHD prevalence since 2000, and by 2010, adults
imperative that a general surgeon who needs to perform a non- accounted for two thirds of patients with CHD in the general
cardiac operation on such a patient be familiar with the specific population.5 This reality has been associated with new challenges
issues of ongoing concern in patients with congenital cardiac in the ongoing medical maintenance of such patients, with par-
disease. ticular focus on the care of patients with congenital cardiac lesions
presenting for surgery for noncardiac illnesses. The evolving sub-
specialty of adult CHD points to the unique needs of this popula-
tion of patients.
HISTORY AND OTHER CONSIDERATIONS
The era of surgical treatment for congenital cardiac anomalies was
initiated in November 1944, when Alfred Blalock and associates PATHWAYS FOR PRACTICING
Vivien Thomas and Helen Taussig combined their unique talents
and vision to treat a young child dying of cyanotic congenital
CONGENITAL HEART SURGERY
heart disease (CHD).1 This palliative operation involved the surgi- Before embarking on a review of the field, it is worthwhile to
cal creation of a systemicpulmonary artery connection in the describe the setting in which patients with CHD seek and receive
patient, who had inadequate pulmonary blood flow. The proce- care in todays medical environment. With the development of
dure has since been recalled as miraculous and now more than 60 sophisticated methods of fetal ultrasound, a large percentage of
years later is known by the eponym Blalock-Taussig (BT) shunt. children requiring surgery for CHD are diagnosed during gesta-
The striking success of this simple concept and the reproducible tion (Fig. 58-1). A fetal diagnosis of complex CHD is extremely
nature of the operation in children with otherwise fatal cardiac helpful to parents and the medical management team. Fetal diag-
conditions have emboldened subsequent surgical innovators to nosis is particularly important in the setting of lesions dependent

1619
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1620 SECTION XI Chest

nursing staff. Focused pediatric cardiac intensive care units have


been developed to optimize the patients opportunity for
recovery.
Historically, pediatric cardiologists have provided the medical
management of patients born with CHD. Pediatric cardiology is
also evolving. With advances in catheter-based technology, inter-
ventional pediatric cardiologists are now addressing lesions previ-
ously treated with surgery. Examples include device closure of
FIGURE 58-1 Normal fetal ultrasound (four chamber; left) and fetal atrial septal defects (ASDs) and ventricular septal defects (VSDs),
ultrasound of a child with hypoplastic left heart syndrome (right). LV, occlusion of patent ductus arteriosus (PDA), and dilation and
Left ventricle; MV, mitral valve. stenting of stenotic vessels in the systemic and pulmonary circula-
tion. For a more in-depth review of this specialty, see the excellent
on persistent patency of the ductus arteriosus for postnatal sur- technical text by Mullins.9
vival. In these individuals, survival after delivery is predicated on The care for adults with CHD is in evolution. This issue is of
the maintenance of ductal patency through the intravenous infu- particular relevance to the general surgeon faced with operating
sion of prostaglandin E1 (PGE1) initiated in the delivery suite, on an adult patient with significant CHD. One overriding message
often through an umbilical vein catheter. Several studies have needs to be clear to the general surgeon in this setting: It must be
shown a decrease in morbidity, but there is inconclusive evidence assumed that in patients with previously repaired congenital
that mortality rates are decreased.6,7 cardiac lesions, even without overt cardiac symptoms, the poten-
A growing number of congenital cardiac lesions are known to tial for significant perioperative cardiorespiratory derangement
be associated with specific genetic mutations, many clearly inher- exists. More simply stated, the presence of a surgical scar on
ited and some presumed to be sporadic. A chromosomal analysis the chest of a patient with known CHD does not suggest that the
is frequently performed in individuals found to have major struc- lesion has been cured. With this message firmly in mind, the
tural cardiac abnormalities; this analysis may be performed during general surgeon may find it challenging to determine the best
gestation through amniocentesis or after delivery. The chromo- source for a qualified consultation for such a patient. At the
somal evaluation is beneficial to the family when planning the risk present time, many adult cardiologists are not adequately trained
of such an occurrence in future offspring. For the clinician, knowl- in CHD to provide competent consultation on adult patients
edge of chromosomal abnormalities in their patients, such as with CHD.
DiGeorge sequence, velocardiofacial syndrome, and Marfan syn- Pediatric cardiologists are not educated in adult medicine and
drome, aids in the delivery of acute medical management. cardiology, and many feel uncomfortable providing consultation
In general terms, the timing of surgery for various congenital on adult patients with CHD. The subspecialty of adult CHD is
cardiac conditions depends on the presenting symptoms and currently becoming more formalized, but the number of physi-
expectations for further associated complications. Neonates pre- cians who have been educated specifically to care for these patients
senting with limited pulmonary blood flow or atretic pulmonary is still few. In 2015, the American Board of Internal Medicine
connections typically require surgery during the first few days of offered the first certification examination in Adult Congenital
life and occasionally within hours of delivery. Lesions associated Heart Disease. The Accreditation Council for Graduate Medical
with excessive pulmonary blood flow result in early heart failure, Educationaccredited fellowship is expected to be available in
which may manifest as poor feeding, tachypnea, or respiratory 2019.10 The practicing general surgeon needs to become familiar
failure. These patients are operated on during early infancy to with the specific issues of concern for patients with CHD to
ameliorate their symptoms and prevent the development of pul- ascertain that the patients unique anatomic and physiologic issues
monary vascular disease. have been evaluated properly. A pediatric cardiologist, in coordi-
Preterm and low-birth-weight infants with CHD have been nation with an adult cardiologist, must evaluate adult patients
presenting for surgical consideration with more frequency. This with CHD who present for care in a center without a designated
treatment strategy requires thoughtful planning and coordination qualified specialist. Of equal importance, the anesthesiologists and
among the surgery, anesthesia, cardiology, intensive care, and intensivists caring for an adult patient with CHD must have a
neonatology teams. At our institution, the Texas Childrens Hos- working understanding of the complexities and nuances of the
pital, we successfully operated on an 800-g infant with transposi- patients cardiac condition. The anesthetic management of patients
tion of the great arteries (TGA). with CHD undergoing general surgical procedures is complicated
The specialty of congenital heart surgery is now recognized as and can become disastrous if managed improperly.
a subspecialty of cardiothoracic surgery. Congenital heart sur-
geons were previously certified in cardiothoracic surgery by the ANATOMY, TERMINOLOGY, AND DIAGNOSIS
American Board of Thoracic Surgery and received additional fel-
lowship training in the United States or abroad in congenital heart Anatomy and Terminology
surgery. As of 2009, the American Board of Thoracic Surgery One of the most intimidating aspects for the student of CHD is
offers a formal certification process for subspecialty training in developing a level of comfort with the terminology used for
congenital heart surgery. At the present time, there are 12 con- describing specific lesions. A thorough and sound understanding
genital cardiac surgery residency programs approved by the of normal cardiac anatomy is mandatory. There are several excel-
Accreditation Council for Graduate Medical Education.8 Most lent texts on this subject; in particular, the text edited by Wilcox
pediatric cardiac surgery is performed in large, multispecialty and coworkers11 is especially concise and clear. One difficulty that
childrens hospitals in association with formal programs focused challenges proper understanding of anatomy is the frequent use
on the care of these complex patients. The management team of abbreviations and eponyms for various congenital lesionsfor
includes pediatric cardiac anesthesiologists, perfusionists, and example, congenitally corrected transposition of the great arteries

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CHAPTER 58 Congenital Heart Disease 1621

(ccTGA), ventricular inversion, and L-transposition all describe Thus, the atrial sidedness is described, followed by the sequence
the same heart, but none provides a complete anatomic descrip- of connections to the ventricles and then great vessels. For
tion. Unless otherwise clear to all clinicians involved in the care example, atrial situs solitus (normal) with atrioventricular discor-
of these complicated patients, the anatomic description needs to dance (reversed) and ventriculoarterial discordance (reversed)
be segmental and complete to avoid mistakes and misinterpreta- describes the heart mentioned earlier as corrected transposition,
tions of structure. or S,L,L by the Van Praagh classification (Fig. 58-3).
In describing congenital cardiac lesions, a segmental approach
is used to determine the relationship of the various structural ele- Diagnosis
ments. The situs describes the relationship of sidednesssitus As with all aspects of surgery, a wide variety of highly sophisticated
solitus (normal), situs inversus (reversed), or situs ambiguus (inde- diagnostic tools are available to examine cardiac structure and
terminate). The cardiac elements described include (in sequence) function. Despite the widespread availability and application of
the atria, ventricles, and great vessels. The relationship of the con- these tools, none has replaced or eliminated the necessity of a
nections must be understood; connections are concordant (e.g., the thorough history and physical examination. Most patients who
right atrium connecting to the right ventricle) or discordant (e.g., have a history of CHD become very well informed about the
the right ventricle connecting to the aorta). The chamber sidedness specifics of their cardiac conditions, as do their parents. A detailed
must be clarified (e.g., a morphologic right atrium may be on the review of the patients past medical history is mandatory. This
left side of the patient). The relationship and connections of the review includes, when possible, securing records from all previous
cardiac valves must then be assessed; connections may be normal, diagnostic and procedural reports. An incorrect assumption often
stenotic, atretic, or straddling. Of note to the general surgeon, is made about a patients previous surgical history and anatomy,
abnormal sidedness of the cardiac structures is frequently associated frequently in a setting in which a patients old operative report or
with abnormal relationships of the thoracic and abdominal organs. clinical summary could easily clarify the misunderstanding.
A thorough assessment of the patients anatomy is recommended In adults with CHD, in particular, there are specific points of
before surgery. Commonly used tools in evaluation of anatomy medical history that must be elucidated. A history of palpitations,
include echocardiogram, computed tomography (CT), magnetic syncope, and neurologic deficit must be investigated further. The
resonance imaging (MRI), and cardiac catheterization. incidence of significant dysrhythmias in certain categories of
There are two widely accepted and applied schools of cardiac adults with CHD is high and, in many cases, warrants further
morphologic description. The Van Praagh nomenclature uses investigation, including continuous monitoring (Holter), electro-
abbreviations to describe the relationship of the atria, ventricular physiologic study, or provocative testing.
looping, and position of the aorta sequentially. The first letter
describes the situs of the atrial chambers (and usually the abdomi- Physical Examination
nal organs): S for situs solitus (normal), I for situs inversus A complete physical examination in a patient with previously
(reversed), or A for situs ambiguus (indeterminate). The second repaired CHD often yields critical information for the proper
letter describes the relationship of the embryologic looping of the planning of a general surgical procedure. Patients need to be
ventricles: D for dextro looping or right-handed topology completely undressed and thoroughly examined. In many cya-
(normal) or L (levo) for left-handed topology. The third and last notic patients, color changes may be prominent, particularly in
letter describes the relationship of the aortic valve to the pulmo- the nail beds, lips, and mucous membranes. In other patients,
nary valve: D for right-sided and L for left-sided (Fig. 58-2). cyanosis may be more subtle, giving the patient a gray or even
The Anderson nomenclature is more wordy and longer but is pale appearance. Previous surgical incisions need to be noted and
perhaps simpler to understand. The descriptions are again of the reconciled with the known medical history. Thoracotomy inci-
sequential relationship of the structures. Starting with the atria, sions on either side may indicate a previous BT shunt using the
the connections and relationships are sequentially described. turned-down, divided subclavian artery or with a prosthetic inter-
position graftthe so-called modified BT shunt. In patients
"NORMALS" with a left aortic arch, a left thoracotomy incision is present if a

Isolated ventricular inversion

S S I I
D RV LV L LV RV D RV LV L LV RV
S RA LA S RA LA I LA RA I LA RA Ao
LA
1 2 3 4 PA TV
Situs solitus A Situs inversus
RA RV
R L
LV
P
FIGURE 58-2 Model depicting cardiac morphology for normal hearts MV
that is, hearts with atrioventricular concordance and ventriculoarterial
concordanceusing Van Praagh nomenclature. The vertical line above FIGURE 58-3 Congenitally corrected transposition of the great arter-
the box denotes the position of the ventricular septum. (From Kirklin ies. Atrial situs solitus (normal) with atrioventricular discordance and
JW, Barratt-Boyes BG: General considerations: Anatomy, dimensions, ventriculoarterial discordance using Anderson nomenclature, S,L,L by
and terminology. In Cardiac surgery, ed 2, New York, 1993, Churchill Van Praagh classification. Ao, Aorta; LA, left atrium; LV, left ventricle;
Livingstone.) MV, mitral valve; RA, right atrium; RV, right ventricle; TV, tricuspid valve.

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1622 SECTION XI Chest

previous coarctation repair has been carried out. Median ster-


notomy incisions or anterior thoracotomy incisions may indicate
previous intracardiac or extracardiac surgery.
A complete vascular examination is often overlooked in patients
with CHD. It is important to assess pulses and obtain blood pres-
sure measurements in all four extremities. Patients who have an
existing or have previously had a BT shunt often have diminished
or absent pulses in the upper extremity corresponding to the
previous shunt. Also, patients with previous coarctation repairs
may have diminished or absent pulses in the left upper extremity,
especially if a subclavian flap angioplasty was performed (Wald-
hausen procedure). Furthermore, a history of previous coarctation
repair does not guarantee that the lower extremity pulses and
blood pressures will be normal. Moreover, patients who have
undergone previous cardiac catheterization may have chronically
stenosed or occluded femoral vessels. All these issues may be of
significance for monitoring and vascular access in a patient under-
going a general surgical procedure.
Later in this chapter, the Fontan procedure for single-ventricle
palliation is reviewed. Briefly, this operation results in significant
systemic venous hypertension, often in 12 to 15mmHg. In
patients with a Fontan circulation, physical examination may FIGURE 58-4 Cardiomegaly and increased pulmonary vascular mark-
reveal hepatic congestion, ascites, pedal edema, venous varicosi- ings in a patient with complete atrioventricular canal defect.
ties, and jugular venous distention. In some patients, macronodu-
lar hepatic cirrhosis may be suspected on the basis of a firm
fibrotic liver edge. conditions such as heterotaxy syndrome, may exhibit deranged or
Entire textbooks have been dedicated to the physical examina- absent sinus node activity, giving rise to a predominant junctional
tion of patients with cardiac disease, and a thorough discussion rhythm, which may significantly compromise cardiac output. The
of this issue, particularly the specifics of cardiac auscultation, is QRS duration and axis reveal information concerning conduction
beyond the scope of this chapter. In general, the cardiac examina- delay and abnormal ventricular forces. For example, patients with
tion includes an assessment of the patients rhythm, point of atrioventricular (A-V) canal defects are known to have left axis
maximal impulse, and character of any auscultated murmurs. deviation. Furthermore, in patients undergoing repair of certain
Also, the absence of a significant cardiac murmur does not rule forms of CHD, there may be an early or late predisposition to
out significant cardiac pathology. malignant dysrhythmias. It is particularly important to elucidate
a history of palpitations from a patient with repaired or unre-
Diagnostic Tests paired CHD; such a history may warrant further investigation
Pulse oximetry. Four-extremity pulse oximetry is an essential with 24-hour continuous ECG monitoring (Holter).
part of the clinical assessment of a patient with suspected CHD. Echocardiography. Noninvasive imaging is well established as
Patients with ductal-dependent circulation to the lower body the primary diagnostic modality for structural cardiac disease. For
(severe aortic coarctation or aortic arch interruption) may present most patients, excellent anatomic detail may be obtained using
with differential cyanosis. This presentation indicates the ejection two-dimensional transthoracic imaging. Standard images include
of desaturated systemic venous blood through the patent ductus subcostal, suprasternal, parasternal, and subxiphoid views and are
to the descending aorta contrasted with fully saturated pulmonary oriented in long and short axis directions. Furthermore, signifi-
venous blood ejected to the ascending aorta and the upper extrem- cant hemodynamic information may be inferred using echo
ities. Baseline (room air) saturation must be documented in all Doppler blood flow velocities and interpreted using the modified
patients for whom an operative intervention is anticipated to Bernoulli formula (pressure gradient = 4V2, where V is echocar-
establish their normal range. diographic velocity in m/sec). To assess the patients cardiac lesion
Plain radiography. Standard chest radiography with antero- properly, segmental analysis of the cardiac structures, connections,
posterior and lateral views is still an essential component of the and valves must be performed. A quantitative estimate of ejection
assessment of a patient with CHD. Standard elements to be fraction, shortening fraction, and valvular inflow velocity aids in
examined include a skeletal survey, assessment of the diaphragms assessing cardiac function. For most patients with CHD, adequate
and hepatic shadow, and location of the gastric bubble. The lung diagnostic information is attainable through echocardiography in
fields are assessed for pulmonary plethora (arterial or venous), air the hands of a qualified pediatric cardiologist.
space disease, and the presence of effusions. The cardiac silhouette Magnetic resonance imaging and computed tomography.
may reveal essential information, such as a cardiothoracic ratio Cardiac MRI and CT are adjuncts to echocardiography for non-
indicative of cardiomegaly or pericardial effusion, the presence of invasive structural and functional assessment of the heart. MRI
atrial enlargement, the presence or absence of the pulmonary has been used with increasing frequency to provide anatomic
artery shadow, and arch sidedness (Fig. 58-4). detail in congenitally malformed hearts in which echocardio-
Electrocardiography. The electrocardiogram (ECG) is impor- graphic detail is lacking or unattainable. This modality has proved
tant in assessing patients with CHD. The rate and rhythm must particularly useful for imaging the extracardiac great vessels and
be noted, including the presence or absence of P wave activity and systemic and pulmonary venous connections and for providing
axis. Many patients with CHD, especially patients with complex accurate estimates of cardiac function, especially right ventricular

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CHAPTER 58 Congenital Heart Disease 1623

ejection fraction. MRI has the added benefit of using nonionizing be particularly true for an adult patient with CHD requiring
electromagnetic fields. CT may also be used for such imaging noncardiac surgery.
detail but has the potential detrimental association with signifi- A thorough understanding of normal cardiorespiratory physi-
cant radiation exposure. A CT scan of the chest averages 5 to ology is critical in interpreting data obtained by cardiac catheter-
7mSv, and CT coronary angiography averages 9 to 11mSv.12 ization in a patient with CHD. Specifically, the normal pressure
Cardiac catheterization. Cardiac catheterization was long con- range, pulse waveforms, and oxygen saturations for the various
sidered the gold standard for diagnostic imaging of congenitally cardiac chambers must be compared against data obtained in a
malformed hearts. With the current sophistication of echocar- deranged circulation. The various cardiac chambers have normal
diography, this is no longer the case for most patients. Nonethe- pulse waveforms. In the atria, there are characteristic waveforms
less, there are still circumstances in which diagnostic cardiac a wave corresponding to atrial contraction, c wave corresponding
catheterization is necessary to obtain accurate anatomic detail. to A-V valve closure, and v wave corresponding to atrial filling
One such circumstance may be patients who have poor echocar- from venous return against the closed A-V valve. Typical normal
diographic windows, although even this issue may be overcome right atrial mean pressures range from 1 to 5mmHg, and left
using transesophageal echocardiography. More often, there are atrial pressures range from 2 to 10mmHg. Right ventricular
specifics of anatomic detail that neither echocardiography nor pressure tracings in normal hearts demonstrate a more gradual
MRI can delineate, such as branch pulmonary artery (or segmen- upstroke when compared with the left ventricle. Filling or end-
tal) stenosis, origin and course of aortopulmonary collateral diastolic pressures are between 2 and 10mmHg in normal hearts.
vessels, and fistulous connections and intracardiac communica- The normal right ventricular systolic pressure (and thus pulmo-
tions (septal defects) not clarified by other imaging modalities. nary artery systolic pressure) is 15 to 30mmHg, and the left
Usually, diagnostic cardiac catheterization is performed to ventricular systolic pressure is 90 to 110mmHg.
obtain precise hemodynamic information needed to make an In normal hearts, there is a small, physiologically insignificant
informed assessment of the consequences of the patients cardiac right-to-left shunt, which results from ventilation-perfusion mis-
lesions. Using oximetric measurements, pressure data, and ther- match in the lungs and coronary venous return directly to the left
modilution cardiac output determination, accurate assessment ventricle (thebesian venous return). This physiologic shunt repre-
of the patients hemodynamic profile is obtained. Measured or sents less than 5% of the cardiac output and, in normal circum-
derived data include central venous pressure, atrial pressure, ven- stances, does not produce detectable systemic arterial desaturation.
tricular pressures (including end-diastolic pressure), shunt frac- Thus, significant systemic arterial desaturation represents a patho-
tion (in the case of ASDs or VSDs), pulmonary artery pressures, logic finding, consistent with pulmonary disease, intracardiac
pulmonary capillary wedge pressure, systemic arterial pressure, shunting, or both. As noted, the origin and degree of intracardiac
and segmental oximetry of cardiac structures, including systemic shunting may be assessed by echocardiography. However, in
and pulmonary venous return (Fig. 58-5). Thus, critical informa- certain circumstances, cardiac catheterization is necessary to
tion is obtained about the presence and degree of shunting, measure cardiac oximetry, calculate shunt fraction, and derive
systemic and pulmonary vascular resistance (PVR), and cardio- systemic and PVR. Using a derivation of the Fick principle, the
pulmonary function. In certain clinical settings, these data are ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs)
mandatory to a successful clinical management strategy. This may can be determined as follows:

69%

65%

20/10
m=15
78%

63%
a=6 92%
v=4 a=5
m=4 v=4 96%
m=3
%
78

a=5
v=4
m=3 76%

65/0,6
75%

70/40
m=52 RA ABG: 7.36/ 42/ 50/ 21/ -4
Arrows indicate catheter course 81%
67
Ht ____cm 6.9
Wt____kg 0.34 2 Hgb____
BSA____m 11.8 Hct___% LSVC___No
5.2 L/min/m2 Qs ___
Qp ___ 1:1 PAR___U.m2 Rp:Rs___
5.2 L/min/m2 Qp:Qs___
(Fick) (Fick)
VO2 cons 150
___ ml/min/m2
(assumed)
FIGURE 58-5 Hemodynamic information obtained after cardiac catheterization.

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1624 SECTION XI Chest

Qp Qs = (SaO2 sat (MO2 sat) (PO2 sat) PaO2 sat) correction, continues to be incorrectly presumed to have ongoing
physiologic perturbation.
where SaO2 sat is systemic arterial oxygen saturation, MO2 sat is
mixed venous oxygen saturation, PO2 sat is pulmonary venous Anesthesia Pitfalls
oxygen saturation, and PaO2 sat is pulmonary arterial oxygen Providing physiologic anesthetic management can be challenging
saturation. in patients with CHD, especially in situations such as chronic
Thus, in a patient with MO2 sat of 60%, PO2 sat of 100%, single-ventricle palliation, unrepaired CHD, chronic cyanosis,
SaO2 sat of 100%, and PaO2 sat of 80%, the equation is as follows: and residual intracardiac pathology. Standard anesthetic manage-
ment paradigms may be completely inappropriate and potentially
Qp Qs = (100 60) (100 80) = 40 20 = 2 : 1
disastrous in the setting of complex CHD. A thorough under-
Calculating vascular resistances may also be crucial in deter- standing of the patients anatomy is mandatory, along with knowl-
mining operability in a patient with CHD. In many settings, a edge of the potential for unexpected response to anesthetic agents
precise measure of vascular resistance is unnecessary based on the and ventilator settings. The field of pediatric and congenital
clinical evidence. For example, in a small child with a large VSD cardiac anesthesia has evolved relative to this specific clinical need;
seen on echocardiogram, the clinical findings of tachypnea, car- the text by Andropoulos and colleagues13 is an excellent resource.
diomegaly, and failure to thrive confirm a large left-to-right shunt Several points concerning anesthesia management warrant dis-
and infer acceptable PVR. However, in less clear circumstances, a cussion. The first is vascular access for intraoperative and postop-
precise calculation may be important in clinical decision making. erative management. In patients with complex CHD, especially
The PVR may be calculated from cardiac catheterization data as patients who have undergone previous complex surgical and cath-
follows: eterization procedures, obtaining appropriate vascular access may
Pulmonary vascular resistance (Rp) be challenging. Typically, a large-bore, multilumen central venous
= (mean pulmonary artery pressure [in mm Hg] line is necessary for appropriate resuscitation and monitoring of
mean left atrial pressure [in 2 mm Hg]) right-sided filling pressures. In some patients, the placement of a
(pulmonary blood flow [Qp, in liters/min/m]) thermodilution pulmonary artery catheter (oximetric) must be
considered because one cannot presume that right-sided filling
In general, patients with an elevated PVR are further evaluated pressures correlate well with left heart volume or functional status
with pulmonary vasodilationhyperventilation, hyperoxygen- (e.g., after a Fontan operation). Options for central access include
ation, and inhaled nitric oxideto determine whether the resis- percutaneous internal jugular or subclavian routes, with a second-
tance is responsive. This information may be critical for patients ary option of common femoral access to the inferior vena cava
who are otherwise marginal candidates. (IVC). Access may be difficult in the setting of previous catheter-
Finally, cardiac catheterization has been evolving as the primary ization or venous reconstruction; this situation may be addressed
therapeutic method for many important structural cardiac defects. with the aid of ultrasound-guided catheter placement, which has
In many childrens hospitals, including Texas Childrens Hospital, become a standard in many cardiac operating room. Arterial
most catheterizations now performed are for interventional pro- access for continuous blood pressure monitoring and sampling is
cedures rather than diagnostic procedures. This fact may be par- important for many patients. Percutaneous radial arterial cannula-
ticularly pertinent to a general surgeon faced with treating a tion can be readily achieved in most patients; however, upper
patient with a previous catheter-based correction of a cardiac extremity blood pressure values may be factitiously altered by
defect. For example, the patient may have had an ASD or VSD previous systemic-to-pulmonary artery shunts, previous aortic
closed with an occluder device in the past. This information may arch surgery (especially coarctation), and abnormalities of vascular
have important ramifications for infectious exposure and vascular origin (e.g., aberrant subclavian origin from the descending aorta).
access. Ventilator management in the perioperative setting of CHD
requires special understanding. In settings of large potential left-
to-right shunts (e.g., unrepaired VSDs), hyperventilation and
PERIOPERATIVE CARE hyperoxygenation promote excessive pulmonary blood flow and
potentially diminish systemic cardiac output. Positive pressure
Perioperative management of a patient with unrepaired or palli- ventilation, particularly positive end-expiratory pressure, nega-
ated CHD can be extremely challenging. Standard hemodynamic, tively influences hemodynamics in many patients, especially in
respiratory, and pharmacologic manipulations appropriate for palliated patients with a single ventricle after the Fontan proce-
structurally normal hearts may be entirely inappropriate in set- dure. Early extubation in these patients can be done to limit the
tings of complex CHD; this is especially true in the operating deleterious effects of positive end-expiratory pressure on the
room and intensive care settings. General rules include a thorough Fontan circulation. Early data showed that early extubation
knowledge of the patients intracardiac anatomy and expected improves outcomes for these patients and reduces overall hospital
physiology. It is possible to make significant management errors costs.14 Finally, pharmacologic manipulation of systemic and PVR
based on incorrect physiologic expectations in the setting of and cardiac performance is an important adjunct in the periopera-
incomplete understanding of the patients anatomy. For example, tive management of patients with CHD. In general, a low-dose
in a patient with unrepaired tetralogy of Fallot (TOF) and associ- infusion of epinephrine 0.05mcg/kg/min (0.02-0.05mcg/kg/
ated significant right ventricular outflow tract obstruction min) with the addition of a phosphodiesterase inhibitor is an
(RVOTO), it is expected that the patient will exhibit some degree effective pharmacologic cocktail to promote a cardiac inotropic
of systemic arterial desaturation. However, a patient with repaired state, lower systemic and PVR, and limit tachycardia. Dopamine,
TOF, with no residual intracardiac shunts, needs to be fully satu- vasopressin, sodium nitroprusside, and nitroglycerin are other
rated. This clinical scenario is a frequent one; a patient with a frequently used agents. Appropriate perioperative analgesia and
specific cardiac diagnosis, despite having undergone a successful sedation are also important aspects of the patients management.

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CHAPTER 58 Congenital Heart Disease 1625

Neurologic Outcomes pulmonary overcirculation, left heart volume overload, and con-
With expectations of almost 100% survival after surgery for gestive heart failure (CHF). A large unrestricted PDA is associated
CHD, emphasis has been placed on the long-term neurologic with pulmonary hypertension; if left untreated, this proceeds to
outcomes and quality of life of these patients. The potential for irreversible pulmonary vascular disease (Eisenmenger syndrome),
neurologic insult in children after CHD arises from the nature of ultimately proceeding to pulmonary and right heart failure, treat-
their disease (e.g., cyanotic defects, low cardiac output state, able only by pulmonary transplantation. Even with a small,
genetic syndromes, effects of cardiopulmonary bypass, circulatory pressure-restrictive PDA, there is an ongoing risk for pulmonary
arrest). Evidence also suggests that patients with CHD may be congestion and left heart volume overloading; endocarditis is
genetically predisposed to neurologic insult. Gestational age has always of concern even for small PDAs. Closure is recommended
been found to be an important factor to consider in the optimiza- for all PDAs.
tion of neurologic outcomes.15 The gold standard of therapy for closure of PDA is surgery,
usually accomplished through a left thoracotomy using ductal
LESION OVERVIEW division, ligation, or clipping (Fig. 58-6). Surgery needs to be a
low-risk procedure associated with minimal potential for persis-
Defects Associated With Increased tence. Nonetheless, the invasive nature of this proven method has
Pulmonary Blood Flow led to the development of alternative strategies for ductal occlu-
Persistent Arterial Duct (Patent Ductus Arteriosus) sion. From a surgical perspective, many PDAs are amenable to
A persistent arterial duct, or PDA, is a frequently encountered thoracoscopic clipping through very small port incisions; robot-
congenital cardiac condition. The arterial duct is necessary during assisted PDA occlusion has been performed in many patients,
gestation to shunt right ventricular blood away from the unven- with good results.11 Medical treatment with indomethacin 0.1mg/
tilated pulmonary vasculature; ductal flow is from the pulmonary kg (0.1mg/kg for <1kg, 0.2mg/kg 1kg on day 1, then 0.1mg/
artery to the aorta during gestation. At delivery, after the first kg daily for days 2-7 oral or IV over 1hr) can be attempted in a
breath of the neonate, ductal flow reverses and becomes left to neonate but carries risk of necrotizing enterocolitis, intracranial
right in most individuals. Over the first several hours or days of hemorrhage, and renal toxicity. However, at the present time,
postnatal life, the PDA closes spontaneously and is completely most PDAs are occluded in the cardiac catheterization laboratory
closed in most infants by 2 to 3 weeks of age. using occlusive devices. Even the repair of large defects in small
In the absence of other congenital cardiac lesions, a PDA infants has been successfully addressed. The long-term effects of
becomes pathologic related to its presence and the degree of left- the devices remaining in the vascular tree are not fully understood
to-right shunting. A PDA may be present in association with yet; however, successful device closure appears to be an extremely
other structural cardiac conditions and may sometimes be neces- effective and durable therapy.
sary for systemic or pulmonary blood flow. The amount of shunt- A PDA in an adult patient can be challenging. As noted, a
ing produced relates to the size and geometry of the duct and the long-standing large PDA may be associated with pulmonary vas-
PVR. A PDA may be responsible for a large Qp/Qs and result in cular disease. A right-to-left shunt in a PDA is cause for significant

Left lung Incision

Left phrenic nerve


Left vagus nerve

Left pulmonary artery


Left recurrent laryngeal nerve

Patent ductus Aortic arch


FIGURE 58-6 Anatomic relationships of a patent ductus arteriosus, exposed from a left thoracotomy. (From
Castaneda AR, Jones RA, Mayer JE, Jr, etal: Patent ductus arteriosus. In Cardiac surgery of the neonate
and infant, Philadelphia, 1994, Saunders.)

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1626 SECTION XI Chest

concern and warrants further investigation. In adults with PDAs,


the arterial wall may calcify, making an attempt at ligation or I
division hazardous. In these patients, ductal occlusion may require
resection of the adjacent descending aorta with patch grafting or
short-segment graft replacement (Dacron).

Aortopulmonary Septal Defect (Aortopulmonary Window)


Ao
An aortopulmonary septal defect is a communication between the PA
ascending aorta and, usually, the main pulmonary artery. This is
a rare defect; it relates to the common embryologic origin of the
arterial trunk and failure of complete separation into the aorta
and pulmonary artery. Defects are classified by their location: LCA II
Type I is proximal, just above the aortic sinuses; type II is more A
distal on the ascending aorta and often involves the origin of the
right pulmonary artery; and type III is more distal and associated
with a separate origin of the right pulmonary artery from the aorta
(Fig. 58-7). An aortopulmonary septal defect may occur in isola- Ao
tion or in association with other conditions, including interrupted
PA
aortic arch (IAA) and anomalous origin of a coronary artery.
Defects are typically large and responsible for a large left-to-right
shunt with systemic pulmonary artery pressures. Children with
this problem typically present with CHF, failure to thrive, and
frequent respiratory infections. Echocardiography, MRI, or B
cardiac catheterization may be used to make the diagnosis.
All aortopulmonary septal defects are surgically closed; this III
lesion is not amenable to catheter-based closure, and such an
attempt is hazardous. A small defect may be ligated through a
thoracotomy or median sternotomy approach, but this method is
not recommended because of significant risk for rupture or
incomplete closure. Surgical closure is accomplished with cardio- Ao
pulmonary bypass support. Options for closure include complete
PA
division and separate patch repairs of the great vessel defects or a
sandwich type of closure, using a patch to construct a common
intervening wall; both methods are effective (Fig. 58-8).

Atrial Septal Defect C


An isolated ASD is one of the most common congenital cardiac FIGURE 58-7 Native anatomy and classification of aortopulmonary
lesions. The most frequently encountered ASD relates to a defect septal defect. A, In type I, the communication is between the ascending
in the interatrial wall, as defined by the fossa ovalis. The defect aorta (Ao) and the main pulmonary artery (PA) on the posterior medial
develops as the result of incomplete closure of the embryologic wall of the ascending aorta. The left main coronary artery (LCA) orifice
patent foramen ovale; the defect is a result of incomplete closure may be close to the defect. B, In type II, the defect is more cephalic
of the septum primum. Although the terminology can be confus- on the ascending aorta. C, In type III, the defect is more posterior and
lateral in the aorta. The communication is with the right pulmonary
ing, these defects are typically termed secundum atrial septal defects.
artery, which may be completely separate from the main pulmonary
They manifest in a wide variety of configurations, ranging from artery. (Adapted from Fraser CD: Aortopulmonary septal defects and
single small defects to multiple fenestrations to complete absence patent ductus arteriosus. In Nichols DG, Ungerleider RM, Spevak PJ,
of the septum primum. The confines of the defect may extend etal, editors: Critical heart disease in infants and children, Philadelphia,
from the IVC orifice up to the superior atrial wall adjacent to the 2006, Mosby, pp 664666.)
aortic root (Fig. 58-9).
The primary pathophysiologic derangement in ASDs relates to
a significant left-to-right shunt in the setting of normal PVR. hypertension. A rare form of presentation relates to the potential
However, even in the setting of a normal PVR, patients with of right-to-left shunting at the atrial level; the ever-present risk
ASDs are capable of transient right-to-left shunting, particularly for paradoxical embolus and cerebrovascular accident must be
during times of increased intrathoracic pressure. The effects of considered when recommending ASD closure.
chronic, large left-to-right shunting (in some patients producing Most centers recommend ASD closure in patients before
a Qp/Qs >3:1) include right heart volume overloading and school age. Since the late 1950s, the standard therapy for ASDs
enlargement. Most children are not overtly symptomatic but may has been surgical closure using cardiopulmonary bypass support.
exhibit some degree of exercise intolerance or frequent respiratory The defect is closed using direct suture closure, autologous
tract infection. Symptoms typically become more prevalent in pericardium, or prosthetic patch material (Fig. 58-10). This
adulthood and include dyspnea on exertion, palpitations, and, is an effective method, with a low associated perioperative risk,
ultimately, evidence of right heart failure. Pulmonary vascular including the virtual absence of residual or recurrent defects.16
disease is not a typical finding in secundum ASDs, but one may Minimally invasive techniques for ASD closure have also
demonstrate an ASD in a patient with primary pulmonary gained popularity.

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CHAPTER 58 Congenital Heart Disease 1627

Ostium
secundum

Ostium
AP septal primum
defect

Ao
Ao PA Tricuspid
Sinus
valve
venosus

Coronary
sinus

A FIGURE 58-9 Types of atrial septal defects as viewed through the


right atrium, ostium secundum, ostium primum, and sinus venosus.
(Adapted from Redmond JM, Lodge AJ: Atrial septal defects and ven-
tricular septal defects. In Nichols DG, Ungerleider RM, Spevak PJ, etal,
editors: Critical heart disease in infants and children, Philadelphia, 2006,
Mosby, p 580.)

Sinus venosus ASDs occur as the result of embryologic


malalignment between the superior vena cava (SVC) or IVC.
Patch These defects are not associated with the ovale fossa and are fre-
closure
quently associated with partial anomalous pulmonary venous
return. A superior sinus venosus ASD occurs high in the atrium,
near the orifice of the SVC. This lesion is frequently associated
with anomalous drainage of a portion of the right lung to the
SVC. An inferior sinus venosus ASD is located low in the atrium,
often extending into the IVC orifice. This lesion is typically associ-
ated with anomalous pulmonary venous drainage of the entire
right lung to the IVC (potentially intrahepatic); pulmonary
sequestration and an abnormal systemic artery perfusing the right
B lower lobe, with origin from the abdominal aorta, may also be
FIGURE 58-8 A, Surgical exposure of aortopulmonary (AP) septal present. In patients with total anomalous pulmonary venous
defect includes a transverse incision in the ascending aorta (Ao). B, The return (TAPVR) to the IVC, the anomalous pulmonary vein may
aortopulmonary septal defect is closed by suturing a patch over the be obvious on a plain chest radiograph and has been described as
aortic side of the defect. PA, Pulmonary artery. (Adapted from Fraser appearing like a saber (scimitar syndrome), first described by Neill
CD: Aortopulmonary septal defects and patent ductus arteriosus. and colleagues.21
In Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Critical Surgery for sinus venosus ASDs is recommended for the same
heart disease in infants and children, Philadelphia, 2006, Mosby,
pathophysiologic reasons surgery is recommended for secundum
pp 664666.)
ASDs. The repair is not amenable to catheter techniques, and
surgery is more complicated than for an isolated secundum ASD.
Superior sinus venosus defects with partial anomalous pulmonary
The potential for closing defects using nonsurgical methods venous return to the SVC may be treated with an intracardiac
has led to the development of catheter-based therapies, which are patch baffle; however, in the setting of high drainage of the anom-
now being widely applied to large numbers of patients worldwide alous pulmonary veins, an SVC translocation operation (Warden
for the treatment of ASD. The most commonly used device is the procedure) may be necessary. Surgery for an inferior sinus venosus
Amplatzer septal occluder device (St. Jude Medical, St. Paul, ASD with a scimitar vein can be more complicated, potentially
MN), made of nitinol metal mesh, which is placed percutaneously involving the need for a patch baffle within the intrahepatic IVC,
and delivered with echocardiographic and fluoroscopic guidance. which may require periods of hypothermic circulatory arrest.
Early reports indicated an acceptable procedure-related complica-
tion rate and successful closure rate.17 However, the long-term Ventricular Septal Defect
effects of having such a device in mobile cardiac structures are not A VSD is a pathologic communication involving a defect in the
fully understood. More recent reports have documented an alarm- interventricular septum. Defects are classified in terms of their
ing incidence of device erosion through the atrial wall and into location and surrounding structures. Patients may be entirely
the adjacent ascending aorta as well as disruption of the conduc- asymptomatic, depending on the size and location of the VSD,
tion system.18,19 A case report showing severe endocarditis involv- along with associated lesions and PVR. In the setting of otherwise
ing a previously placed Amplatzer ASD device has highlighted the normal cardiac morphology and appropriate PVR, the net shunt
need for ongoing observation of the long-term consequences of in patients with VSD is left to right; the Qp/Qs depends on the
placing large prosthetic devices into the circulation.20 size of the defect and pulmonary resistance. Large defects result

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1628 SECTION XI Chest

Membranous
septum
Outlet
septum

2 Trabecular
1 septum
4

A 3
4

PA
Ao 4

SVC Inlet
septum
FIGURE 58-11 Location of ventricular septal defects (VSDs) in the
ventricular septum (view of the ventricular septum from the right side).
TV 1, Perimembranous VSD; 2, subarterial VSD; 3, atrioventricular canal
Patch type VSD; 4, muscular VSD. (From Tchervenkov CI, Shum-Tim D: Ven-
Direct closure
closure tricular septal defect. In Baue AE, Geha AS, Hammond GL, editors:
Glenns thoracic and cardiovascular surgery, ed 6, Stamford, CT, 1996,
Appleton & Lange.)
CS

B C Perimembranous Ventricular Septal Defect


A perimembranous VSD occurs as a defect in the membranous
portion of the interventricular septum; its associated margins
include the annulus of the tricuspid valve, the muscular septum,
and potentially the aortic annulus. The defects may be large and
have associated prolapse of the noncoronary or right coronary
aortic valve cusps. Perimembranous VSDs exhibit a potential for
spontaneous closure, particularly small defects manifesting early
in childhood.
Air
from Muscular Ventricular Septal Defect
LA Muscular VSDs occur in all aspects of the muscular interventricu-
lar septum. Margins of these defects are entirely muscle. The
D lesions may be isolated or involve multiple openings in the septum
FIGURE 58-10 Surgical closure for atrial septal defect. A, Right atri- (so-called Swiss cheese septum). Small defects have great potential
otomy. B, Direct suture closure. C, Patch closure. D, Deairing the left for regression or spontaneous closure.
atrium (LA). Ao, Aorta; CS, coronary sinus; PA, pulmonary artery; TV,
tricuspid valve. (Adapted from Redmond JM, Lodge AJ: Atrial septal Subarterial (Supracristal or Outlet) Ventricular Septal Defect
defects and ventricular septal defects. In Nichols DG, Ungerleider RM, Subarterial VSDs occur in association with the annulus of the
Spevak PJ, etal, editors: Critical heart disease in infants and children, aortic valve, pulmonary valve, or both. The defects are almost
Philadelphia, 2006, Mosby, p 583.) always associated with significant prolapse of the adjacent aortic
valve cusp, usually the right coronary cusp, which may lead to
significant cusp distortion, aortic valve insufficiency, and cusp
in large shunts; high right ventricular and pulmonary artery pres- perforation. The only mechanism for spontaneous closure of these
sures; and significant pulmonary overcirculation, CHF, and left defects relates to the cusp prolapse and valve distortion and is
heart volume overload. In these settings, unrestrictive pulmonary generally not complete or a favorable arrangement. All these
blood flow exposes the patient to the risk for pulmonary vascular defects are surgically closed because of the ongoing risk for aortic
disease and Eisenmenger syndrome. valve injury (Fig. 58-11).
The ventricular septum can be best thought of in terms of the The indications for surgery to close VSDs relate to the size of
pathway of blood and associated cardiac anatomy. Thus, the right the VSD, degree of shunting, and associated lesions. Small infants
ventricular aspect of the septum has an inlet portion; midmuscu- presenting with large VSDs, refractory heart failure, and large
lar portion; apical, posterior, anterior, and outlet portions; and shunts undergo surgical closure of the defects in the newborn
subaortic portion. This knowledge aids in the classification of period, regardless of age or size. Other defects are addressed based
VSDs. Furthermore, defects are understood relative to their on the ongoing concerns of left-to-right shunting, aortic valve
embryologic origins and have varying propensities for spontane- cusp distortion, and risk for endocarditis. Asymptomatic patients
ous decreases in size or closure. with evidence of significant shunts and cardiomegaly are proposed

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CHAPTER 58 Congenital Heart Disease 1629

for surgical therapy. Prophylactic closure of small defects in


asymptomatic patients with normal cardiac size and function is RLL
advocated by some surgeons because of the lifelong risk for endo- TV AL PL
carditis and comparatively low risk for surgery. RSL RIL
Percutaneous VSD closure is an acceptable alternative to surgi- Bridging
SL
cal closure of VSDs with very high procedural success.22 The AL LIL portion
LSL
complex relationship of many defects, including close association MV of LIL
with the aortic valve and cardiac conduction tissue, makes the PL LLL
existing technology less than ideal. At the present time, surgery
remains the primary mode of therapy for VSD closure. Defects A Normal valves B Partial A-V canal
are approached with the aid of cardiopulmonary bypass support
Bridging Bridging
and may be closed with various materials, including autologous RLL RLL
portion portion RSL RLL
pericardium (our preference), Dacron, polytetrafluoroethylene,
of of
and homograft material. Surgical closure of VSDs is a low-risk RSL RIL LSL RSL RIL
LSL RIL
procedure with a high expectation of complete closure. Challeng-
ing anatomic situations, such as Swiss cheese septum or multiple LIL
LSL LSL LIL LSL LIL
apical muscular VSDs, may be initially palliated by limiting pul- LIL
monary blood flow with a pulmonary artery band and deferring
corrective surgery to later in life. LLL LLL LLL
Rastelli A Rastelli B Rastelli C
Atrioventricular Septal Defect C Complete A-V canal or common A-V orifice
(Atrioventricular Canal Defect) FIGURE 58-12 Rastelli classification type A, B, or C. The difference in
Atrioventricular septal defects (AVSDs) are a complex constella- valve morphology in a normal (A), partial (B), and complete (C) canal
tion of cardiac lesions involving deficiency of the atrial septum, defect is illustrated. AL, Anterior leaflet; A-V, atrioventricular; MV, mitral
ventricular septum, and A-V valves. This lesion results from an valve; PL, posterior leaflet; RIL, right inferior leaflet; RLL, right lateral
embryologic maldevelopment involving the endocardial cushions; leaflet; RSL, right superior leaflet; TV, tricuspid valve. (From Kirklin JW,
thus, the term endocardial cushion defect is often applied. AVSDs Pacifico AD, Kirklin JK: The surgical treatment of atrioventricular canal
may be partial, involving no ventricular level component; inter- defects. In Arciniegas E, editor: Pediatric cardiac surgery, Chicago,
1985, Year Book Medical Publishers.)
mediate or transitional, involving a small restrictive VSD; or
complete, involving a large nonrestrictive VSD. The A-V valve
tissue is always abnormal in AVSD, although there is great indi-
vidual variability in terms of the severity of the valvular malforma- of atrial level shunting and the presence of A-V valve regurgita-
tion and valve function. Complete AVSDs are frequently seen in tion. AVSD may also manifest in unbalanced forms, with domi-
patients with trisomy 21 but also occur in patients with normal nance of right-sided or left-sided components. In severely affected
chromosomes. The morphology of the septal defects in this condi- individuals, biventricular repair is not feasible, and patients are
tion is different from that previously discussed. The ASD in this managed along a single-ventricle pathway. AVSD may also be
defect is termed a primum ASD and is distinctly separate from the found in association with TOF; this combination is associated
ovale fossa. There is displacement of the A-V node and bundle of with cyanosis, and repair is more challenging than for either
His to the inferior aspect of the primum defect and A-V junction, condition considered in isolation.
a feature of critical importance during surgical repair. Patients Surgery is the primary mode of therapy for patients with
with AVSD have an inlet VSD, which may extend into the sub- AVSD. Operative goals include complete closure of ASDs and
aortic region and have a component of septal malalignment. The VSDs and effective use of available A-V valve tissue to achieve
chordal support of the A-V valves has a variable relationship to valve competence. As noted, the inferiorly displaced conduction
the interventricular septum. The relationship of the chordal tissue must be protected to avoid the complication of surgically
support and superior bridging component of the left A-V valve induced A-V block (Fig. 58-13). Surgical intervention is per-
has been used to classify complete AVSD, as described by Rastelli formed with the use of the cardiopulmonary bypass machine. The
and associates23: type A, with superior leaflet and chordal support atrial and ventricular septal components are closed with a common
committed to the left side of the ventricular septum; type B, with patch (single-patch method) or separate patches (two-patch tech-
straddling and shared chordal support; and type C, with a floating nique). We believe the two-patch method to be superior in pre-
left superior leaflet component and chordal support on the right serving A-V valve tissue (Fig. 58-14).24 The critical component of
side of the ventricular septum (Fig. 58-12). the repair lies in the valve repair; typically, after suspending the
Patients with complete AVSD typically present in infancy with valve tissue to the reconstructed septum, the line of coaptation
large left-to-right shunts, cardiomegaly, and CHF. Without surgi- between the superior and inferior leaflet components (cleft) is
cal treatment, patients exhibit severe failure to thrive, a suscepti- closed; however, care must be exercised to avoid valvular
bility to severe respiratory infections, and potential for early stenosis.
development of pulmonary vascular disease. Surgical repair is Perioperative care is predicated on an accurate and hemody-
recommended in infancy (usually before 6 months of age) but namically favorable repair. Patients with long-standing pulmonary
may be necessary in the newborn period for neonates with refrac- overcirculation may have a potential for early perioperative pul-
tory heart failure, especially in association with aortic arch anoma- monary hypertensive crisis. This condition may require therapy,
lies. Patients with partial or intermediate defects may have the including oxygen, optimization of fluid balance, continuous seda-
surgery deferred until later in childhood, depending on the degree tion, hyperventilation, and, possibly, inhaled nitric oxide.

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1630 SECTION XI Chest

Adult Patients With Atrioventricular Septal Defect recommended to rule out occult coronary artery lesions and to
Numerous patients with partial or transitional AVSD survive well evaluate PVR. Nonetheless, in the absence of obvious surgical
into adulthood without surgery. These patients have variable pre- contraindication, surgery is recommended for adults with unre-
sentations but may exhibit severe exercise intolerance; evidence of paired AVSD to eliminate the chronic left-to-right shunt and
right heart dysfunction; some elevation of PVR; and, possibly, repair the typically insufficient A-V valves.
atrial dysrhythmias, including atrial fibrillation. In patients Other patients present well into adulthood with previously
with late presentation of AVSD, cardiac catheterization is often repaired AVSDs. These patients may have a widely disparate con-
stellation of findings, including atrial and ventricular dysrhyth-
mias, valvular insufficiency or stenosis, and right heart dysfunction.
RBB In many of them, secondary reparative surgery may become neces-
RLL sary. Furthermore, in the setting of a patient with remotely
BB repaired AVSD requiring noncardiac surgery, potential ongoing
RSL hemodynamic concerns that would affect the perioperative course
PA must be expected.
RIL
Crest of Persistent Arterial Trunk (Truncus Arteriosus)
venticular
septum PB Truncus arteriosus or persistent arterial trunk results from failure
of separation of the embryonic arterial trunk and semilunar valves.
LSL A-V node It is almost always associated with a large nonrestrictive VSD, is
Ao CS typically perimembranous, and is associated with varying degrees
LLL LIL of truncal override of the interventricular septum, including
100% association of the trunk with the right ventricle. The condi-
tion is classified by the relationship of the origins of the pulmo-
nary arteries. In type I truncus arteriosus, there is a demonstrable
common main pulmonary artery with subsequent origins of
Edge of common the branch pulmonary arteries; in type II truncus arteriosus, the
Fossa ovalis Edge of atrial A-V orifice branch pulmonary arteries arise closely, but separately, from the
septum
trunk; in type III truncus arteriosus, the branch pulmonary arter-
FIGURE 58-13 Position of the conducting system in complete atrio- ies are widely separated in origin on the ascending aorta; and in
ventricular canal defect. The anatomic relationships and morphology of type IV truncus arteriosus, no pulmonary arterial branch arises
the common atrioventricular (A-V) valve are shown. The view is through
from the common trunk. Type IV truncus arteriosus is now rec-
a right atriotomy. Ao, Aorta; BB, left bundle branch; CS, coronary sinus;
LIL, left inferior leaflet; LLL, left lateral leaflet; LSL, left superior leaflet; ognized as a form of pulmonary atresia with VSD (Fig. 58-15).
PA, pulmonary artery; PB, penetrating bundle; RBB, right bundle branch; In contrast to patients with aortopulmonary septal defects,
RIL, right inferior leaflet; RLL, right lateral leaflet; RSL, right superior patients with truncus arteriosus have a single outlet valve of highly
leaflet. (From Bharati S, Lev M, Kirklin JW: Cardiac surgery and the variable morphology. The valve may have a normal appearance,
conducting system, New York, 1983, Churchill Livingstone.) with three well-formed and distinct cusps similar to those of a

2
1
3 Leaflet
4
sandwiched
between
patches
5

Pericardial
patch

A B
FIGURE 58-14 Two-patch closure of complete atrioventricular canal defect. A, A ventricular septal patch is
placed first, and a separate patch is used to close the atrial septal defect (ASD) component. B, Note the
position of the coronary sinus and conducting system relative to the ASD patch suture line to avoid injury
to the atrioventricular node. (From Kirklin JW, Barratt-Boyes BG: Cardiac surgery, New York, 1986, Churchill
Livingstone.)

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CHAPTER 58 Congenital Heart Disease 1631

Collett and Edwards

RPA
Ao Ao
LPA Ao

RPA LPA
MPA

Type I Type II Type III Type IV


Van Praagh

Ductus

RPA
Ao
Ao RPA Ao
LPA Ao

MPA LPA

Type A-1 Type A-2 Type A-3 Type A-4


FIGURE 58-15 Collett-Edwards and Van Praagh classification systems for persistent truncus arteriosus (see
text for details). Ao, Aorta; LPA, left pulmonary artery; MPA, main pulmonary artery; RPA, right pulmonary
artery. (Adapted from St Louis JD: Persistent truncus arteriosus. In Nichols DG, Ungerleider RM, Spevak PJ,
etal, editors: Critical heart disease in infants and children, Philadelphia, 2006, Mosby, p 690.)

normal aortic valve. In other patients, the truncal valve may be maintain ductal patency until they undergo repair. Given these
severely malformed, with multiple cusps, dysmorphic leaflets, and considerations, it is recommended that most newborn patients
abnormal commissural relationships. The truncal valve morphol- undergo repair in the first several weeks of life.
ogy and function have significant bearing on patient symptoms The surgical repair is performed on cardiopulmonary bypass
and the difficulty of surgery. Patients with truncus arteriosus support. Components of the repair include division of the
frequently have coronary ostial abnormalities, including juxta- common trunk and reconstruction of confluent central branch
commissural origin and intramural course. There is an associated pulmonary arteries. The large VSD is closed with a patch, typically
interruption of the aortic arch in 25% of newborns presenting through a right ventriculotomy. In patients with abnormal, insuf-
with truncus arteriosus. Abnormalities of thymic genesis, T cell ficient truncal valves, a valve repair may be necessary. It is unusual
function, and calcium homeostasis may frequently be seen in this to have to replace the truncal valve at the initial operation; most
group of patients in association with a chromosome 22 deletion valves can be at least partially repaired to provide the patient with
(DiGeorge syndrome). an adequate aortic valve. Right ventriclepulmonary artery con-
Patients with truncus arteriosus present in the newborn period tinuity must then be established. Most surgeons prefer to inter-
with unrestricted pulmonary blood flow and systemic pulmonary pose a valved conduit between the right ventriculotomy and
artery pressure. With the expected postnatal decrease in PVR, pulmonary artery bifurcation (Fig. 58-16).
massive pulmonary overcirculation, and CHF, patients may Conduits are limited and include homografts (pulmonary
exhibit a wide pulse pressure because of diastolic runoff of blood artery or aorta, valved) or heterografts (bovine or porcine). Experi-
into the pulmonary vasculature. This situation is further exacer- ence with a commercially available, glutaraldehyde-preserved,
bated in the setting of significant truncal valve insufficiency, bovine jugular vein valved conduit (Contegra; Medtronic,
resulting in poor systemic perfusion and cardiovascular collapse. Minneapolis, MN) had been encouraging.25 However, there is a
Some infants can be initially managed with medical decongestive concerning increased incidence of endocarditis with Contegra
therapy (e.g., diuretics, angiotensin-converting enzyme inhibitors, valved conduits compared with other conduits, including homo-
and digoxin) and fortified nutritional support (through gastric grafts and heterografts. Successful repair of truncus arteriosus in
intubation); however, this is a precarious arrangement. In the few infants using a direct hooded anastomosis between the pulmonary
individuals who survive infancy, irreversible pulmonary vascular artery bifurcation and right ventriculotomy has also been
disease develops rapidly, and patients become inoperable. In other reported.26 No option available at the present time offers patients
patients, refractory CHF results in poor weight gain, respiratory the lifetime solution of a connection capable of somatic growth
insufficiency, and susceptibility to infection. The profound hemo- along with a competent, durable pulmonary valve. Thus, it is
dynamic compromise places many newborns with unrepaired expected that all infants undergoing successful truncus repair
truncus arteriosus at high risk for necrotizing enterocolitis. will require multiple subsequent cardiac surgeries as they
Patients with truncus arteriosus and IAA have ductal-dependent outgrow their current right ventriclepulmonary artery conduit.
systemic blood flow and are dependent on intravenous PGE1 to Experience with a percutaneously delivered, catheter-mounted

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1632 SECTION XI Chest

Aorta pulmonary function. The abnormal venous connection drains in


several typical patterns.
LPA In supracardiac TAPVR, the pulmonary veins drain to a verti-
cal vein, which courses cephalad to join a systemic vein. In the
RPA most common variation, the vertical vein courses anterior to the
left pulmonary artery to join the left innominate vein. This vein
Ventricu- VSD may course posterior to the left pulmonary artery, resulting in
lotomy compression of the pulmonary venous pathway between the left
pulmonary artery and left mainstem bronchus (so-called pulmo-
Patch nary artery vise). The vertical vein may also join the SVC or azygos
RV vein. In intracardiac TAPVR, the pulmonary veins drain into the
A B coronary sinus and, in most cases in which the coronary sinus is
intact, into the right atrium. This variant is rarely obstructed and
may not be diagnosed until later in life in some patients. In infra-
cardiac TAPVR, the vertical veins descend in a caudal direction
through the diaphragm to join the embryologic ductus venosus
and then through the liver to join the IVC. This variation is
LPA almost always obstructed at some level (Fig. 58-17). In mixed
Aorta Conduit TAPVR, the pulmonary venous pathway drains in several path-
Conduit ways to reach the heart. Frequently, in mixed TAPVR, one or
several pulmonary veins connect to the SVC, with others draining
RV to an infracardiac or supracardiac connection.
Obstructed total anomalous pulmonary venous return.
Obstructed TAPVR is one of the few true surgical emergencies
C VSD D in congenital heart surgery. When the condition is suspected, it
repaired is diagnosed with transthoracic echocardiography. Obstructed
FIGURE 58-16 Surgical repair of truncus arteriosus. A, The origin of TAPVR occurs when one of the drainage patterns noted earlier is
the truncus arteriosus is excised, and the truncal defect is closed with obstructed, resulting in severe pulmonary venous hypertension.
a direct suture. The incision is made high in the right ventricle (RV). Secondary effects include pulmonary edema, pulmonary artery
B, The ventricular septal defect (VSD) is closed with a prosthetic patch. hypertension, and profound hypoxemia. Interstitial pulmonary
C, Placement of a valved conduit into the pulmonary arteries. D, Proxi- emphysema and frank pneumothorax may develop while attempt-
mal end of conduit is anastomosed to the RV. LPA, Left pulmonary ing vigorous ventilatory support in profoundly desaturated chil-
artery; RPA, right pulmonary artery. (From Wallace RB: Truncus arterio- dren. Patients with obstructed TAPVR may present within hours
sus. In Sabiston DC, Jr, Spencer FC, editors: Gibbons surgery of the of birth in extremis and do not respond to resuscitative efforts.
chest, ed 3, Philadelphia, 1976, Saunders.)
The only useful therapy is rapid surgical repair, regardless of the
severity of the patients preoperative status.
For other forms of TAPVR, elective surgical repair is recom-
pulmonary valve has been encouraging as an interim solution for mended after the condition is diagnosed. Occasionally, the diag-
these patients in an effort to limit the number of required cardiac nosis is not made until later in childhood in patients with an
reoperations.27 unobstructed vertical vein and widely patent atrial communica-
A growing number of adults have survived childhood truncus tion. These patients undergo elective repair to relieve cyanosis,
arteriosus repair. All these patients require diligent longitudinal intracardiac mixing, and right heart volume overload.
cardiology surveillance, and many will require reoperation. Issues Surgical repair of TAPVR requires cardiopulmonary bypass
of concern include late ventricular dysrhythmias, often related to support; occasionally, periods of profound hypothermia and cir-
surgical scarring from the previous right ventriculotomy; branch culatory arrest are necessary. The principles of repair include iden-
pulmonary artery stenosis; stenosis or insufficiency of the right tification of the pulmonary venous confluence and individual
ventriclepulmonary artery conduit; truncal valve insufficiency; pulmonary veins. An anastomosis is constructed between the
and right ventricular dysfunction. venous confluence and left atrium using a superolateral approach,
with the heart reflected to the patients right, or an incision
Abnormalities of Venous Drainage directly through the interatrial septum and corresponding region
Total Anomalous Pulmonary Venous Return of the posterior right atrial wall. The ASD and PDA that are typi-
TAPVR results from embryonic failure of connection of the fetal cally present are closed as well (Fig. 58-18).
pulmonary venous sinus to the left atrium. This fatal condition Cor triatriatum. Cor triatriatum is a rare condition in which
has a spectrum of clinical presentations and may be associated the pulmonary veins enter a chamber posterior to the left atrium
with additional complex structural cardiac disease, including a with a small connection to the right or left atrium. These patients
single ventricle. In TAPVR, pulmonary venous return may take exhibit evidence of pulmonary hypertension and variable desatu-
one of several pathways to return eventually to the right heart. ration. Surgical decompression is necessary to relieve the pulmo-
Initial survival is predicated on an unobstructed pathway and nary venous obstruction; this is accomplished by resection of
unrestricted atrial-level communication so that sufficient intracar- the membrane between the pulmonary venous chamber and
diac mixing affords the patient adequate systemic oxygenation. left atrium.
Patients with TAPVR are desaturated to varying degrees, depend- A dreaded consequence of TAPVR occurs when there is a
ing on the adequacy of the anomalous pathway, atrial mixing, and progressive, malignant sclerosing process involving the individual

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CHAPTER 58 Congenital Heart Disease 1633

LI Anomalous
vertical v.

LA
RA

CS CS
LA LA
RA LV
RA
CS RV
LV
LV RV
RV Liver
DV
A B C
FIGURE 58-17 Types of total anomalous pulmonary venous connection. A, Supracardiac type with a vertical
vein joining the left innominate (LI) vein. B, Intracardiac type with connection to the coronary sinus (CS).
C, Infracardiac type with drainage through the diaphragm via an inferior connecting vein. DV, Ductus venosus;
LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. (From Hammon JW, Jr, Bender HW,
Jr: Anomalous venous connections: Pulmonary and systemic. In Baue AE, editor: Glenns thoracic and cardiac
surgery, ed 5, Norwalk, CT, 1991, Appleton & Lange.)

long-term success, with 50% survival at 5 years.28 In the most


severe cases, the only meaningful surgical option is lung
transplantation.

Anomalous Systemic Venous Drainage


Congenital abnormalities of systemic venous drainage may occur
in isolation or in association with other significant structural
cardiac defects. In the setting of an otherwise normal heart, the
anomaly is frequently not of physiologic significance. The most
common example is a persistent left SVC draining to the coronary
sinus. In the absence of an intracardiac communication or unroof-
ing of the coronary sinus, this is of anatomic significance only. In
A many cases, a persistent left SVC occurs, with absence of a com-
B municating innominate vein. This condition becomes important
FIGURE 58-18 A, Repair of supracardiac total anomalous pulmonary in situations of mechanical occlusion, which may be seen with
venous connection (TAPVC) through a superior approach. B, Repair of trauma or chronic venous intubation with thrombosis. A persis-
infracardiac TAPVC. Elevating the apex of the heart to the right side
tent left SVC frequently is incidentally discovered after placement
exposes the left atrium and pulmonary confluence. Anastomosis is
created as shown. (From Lupinetti FM, Kulik TJ, Beekman RH, etal:
of a left internal jugular central line, which is apparently found
Correction of total anomalous pulmonary venous connection in infancy. to track into the heart on plain chest radiography. A persistent
J Thorac Cardiovasc Surg 106:880885, 1993.) left SVC becomes more significant in patients requiring intracar-
diac or extracardiac surgery. If the left SVC drains to an unroofed
coronary sinus in a patient undergoing atrial septation, the patient
pulmonary veins. This process may be initiated by inaccurate will be profoundly desaturated after surgery. This situation requires
surgery resulting in obstruction of the venous confluence and reconstruction of the coronary sinus or some other method to
individual veins, or it may progress independently of surgical reroute the left SVC to the right atrium.
manipulation. It may progress to intrapulmonary pulmonary An interrupted IVC usually occurs in association with other
venous stenoses. A technique to deal with individual pulmonary structural cardiac disease. The IVC drainage in these settings is
venous stenoses uses a pedicled flap of adjacent pericardium to to the azygos (azygos continuation) or hemiazygos vein and
augment the pulmonary venous orifices (sutureless technique), ultimately the SVC. In these patients, the hepatic veins drain
but this method is not applicable to all patients with pulmonary into the atrium as a common confluence or as individual veins.
venous obstruction. Catheter-based dilation and stenting have The physiologic significance of the interrupted IVC relates to the
been attempted in this setting with good acute relief of obstruc- coexisting cardiac lesion and the necessity of appreciating the
tion but have a high rate of reintervention and unknown abnormality of systemic venous drainage in performing corrective

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1634 SECTION XI Chest

FIGURE 58-19 Drs. Alfred Blalock, Helen Taussig, and Vivien Thomas.

surgery. In patients requiring noncardiac surgery or catheter inter- Left pulmonary a.


vention, the presence of an interrupted IVC is noted when an
attempt is being made to pass a venous catheter from the groin
into the heart.

Cyanotic Congenital Heart Disease


Tetralogy of Fallot
TOF is a common form of cyanotic CHD and is probably the
most studied lesion in the era of surgical correction for CHD.
Many believe that The Johns Hopkins Hospital was the birthplace
of cardiac surgery. Blalock performed the first successful palliative
operation for TOF in November 1944, assisted by his laboratory
technician.1 Blalock was encouraged by Taussig, the matriarch of
pediatric cardiology (Fig. 58-19). Until more recently, some
degree of controversy has surrounded the relative degree of con- Esophagus
tribution by these three individuals in bringing this historical
event to fruition. In actuality, all three were significant partici- Left subclavian a. Vagus
pants in this momentous medical advance. While working at
Divided
Vanderbilt Medical School, Blalock had charged his young and ductus
capable laboratory technician, Vivien Thomas, with the develop-
ment of a surgical model of pulmonary hypertension. Thomas and FIGURE 58-20 Blalock-Taussig shunt.
Blalock developed a method of anastomosing the left subclavian
artery to the divided left pulmonary artery in a canine model. surgery was ushered in. (These historic accounts are factual, the
Specifically, Thomas worked out the technical details, including result of personal interviews with many of those in attendance at
crafting the necessary surgical instruments, and mastered the that event, including Thomas, Taussig, J. Alex Haller, and Denton
operation. This work did not produce the desired effect; canine Cooley.)
PVR is almost infinitely low, and the animals did not develop a The historic account of the development of the BT shunt has
hypertensive pulmonary vasculature. Nonetheless, the technique relevance to the practice of congenital heart surgery today. First,
was developed and published approximately 10 years in advance it is important that the facts surrounding this achievement are
of the clinical application in 1944. acknowledged. Second, this remarkably simple concept still
Blalock subsequently became the Chair of Surgery at Johns remains a frequently applied technique for children with inade-
Hopkins. Taussig had by that time established a reputation as a quate pulmonary blood flow. Finally, over almost 75 years of
meticulous diagnostician of complex congenital heart lesions. She treatment of TOF, thousands of patients have been successfully
had a large clinic of critically ill children with disabling cyanosis treated, but most are not cured; many require subsequent reopera-
blue babies. At her suggestion (and probably her insistence), tive cardiac surgery, even after complete repair.
Blalock was convinced to attempt a surgical palliation for TOF The anatomic hallmark of TOF is anterior malalignment of
by constructing in a human the subclavian-to-pulmonary artery the infundibular septum, which leaves a deficiency in the subaor-
anastomosis that had been perfected in the research laboratory tic regiona malalignment VSD. This VSD is usually perimem-
(Fig. 58-20). Blalock performed the operation in conditions and branous, large, and pressure-nonrestrictive. The relative degree of
with instruments that would be considered extremely crude by malalignment influences the relationship of the aorta to the inter-
todays standards. Thomas stood immediately behind Blalock ventricular septum, producing varying degrees of aortic override.
during that operation and many subsequent cases, providing The deviated infundibular septum produces varying degrees of
instruction and encouragement. The clinical success was an earth- RVOTO. The path of pulmonary blood flow may be impeded at
shattering event; hundreds of patients subsequently traveled to numerous levels, including the infundibulum, pulmonary valve
Johns Hopkins for surgical treatment, and the era of cardiac and annulus, and main and branch pulmonary arteries. Secondary

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CHAPTER 58 Congenital Heart Disease 1635

PDA

Ao PA

ASD

RVOTO

Malalignment
VSD
FIGURE 58-21 Anatomy of tetralogy of Fallot. A malalignment ven-
tricular septal defect (VSD), aortic override, right ventricular outflow
tract obstruction (RVOTO), and subsequent right ventricular hypertro-
FIGURE 58-22 Long right ventriculotomy in a classic transventricular
phy. Ao, Aorta; ASD, atrial septal defect; PA, pulmonary artery; PDA,
approach. (From Morales DL, Zafar F, Heinle JS: Right ventricular infun-
patent ductus arteriosus. (Adapted from Davis S: Tetralogy of Fallot
dibulum sparing [RVIs] tetralogy of Fallot repair: A review of over 300
with and without pulmonary atresia. In Nichols DG, Ungerleider RM,
patients. Ann Surg 250:611617, 2009.)
Spevak PJ, etal, editors: Critical heart disease in infants and children,
Philadelphia, 2006, Mosby, p 756.)
incision through the right ventricular outflow tract (RVOT); this
provides an excellent transventricular view of the VSD, which is
right ventricular hypertrophy occurs relative to the degree and closed with a patch. The pulmonary artery, pulmonary valve, and
duration of the obstruction and is progressive, contributing to the annulus are incised if stenotic, and then the RVOT is patched.
propensity for the lesion to worsen over time (Fig. 58-21). This method was used for many years but has the complicating
The pathophysiology of TOF relates to shunting of desatu- feature of the long ventriculotomy, with attendant right ventricu-
rated, systemic venous blood through the VSD to mix with the lar dysfunction and often severe pulmonic insufficiency (Fig.
systemic cardiac output. The greater the degree of obstruction to 58-22). An alternative method, the transatrial or transpulmonary
pulmonary blood flow, the larger the right-to-left shunt and the approach, first proposed by Imai, has gained popularity. In this
worse the desaturation. There are several modes of presentation. method, the VSD closure and RVOT resection are accomplished
Newborns with TOF and severe RVOTO may present soon after through a right atriotomy via the tricuspid valve. The main pul-
birth with profound cyanosis; some require PGE1 to maintain monary artery and pulmonary annulus are incised only if stenotic,
ductal patency for adequate oxygenation. At the other end of the but there is no transmural infundibular incision. This method is
spectrum is children with little infundibular obstruction and technically more demanding than the classic method but may
normal pulmonary valve and branch pulmonary arteries. These offer the patient improved long-term right ventricular function
patients may have net left-to-right flow through the VSD, occa- (Figs. 58-23 to 58-25). The approach has been further developed
sionally to the extent that they experience pulmonary overcircula- as a right ventricular infundibulum-sparing strategy that focuses
tion and CHF (so-called pink TOF). Most children present on minimizing the right ventricular incision and preserving the
between these extremes; an initially mild to moderate degree of pulmonary valve. The right ventricular infundibulum-sparing
infundibular stenosis progresses over time to become severe with strategy includes an algorithm for optimal timing of the repair
worsening desaturation. A TOF spell occurs when there is an that considers the individual patients weight, age, and overall
acute change in the cardiac inotropic state, often in the setting of clinical picture (Fig. 58-26). Midterm results with this approach
agitation and dehydration. The infundibular stenosis acutely have demonstrated preserved right ventricular function.29
worsens, and patients become profoundly desaturated; this may The long-term sequelae of TOF repair have been unfolding.
be an extremely serious event, leading to brain damage or death. For most patients, successful childhood repair of TOF does not
Acute treatment modalities include sedation, hydration, systemic translate into a cure. As patients age after TOF repair, long-term
afterload augmentation (-adrenergic agonists), beta blockade to complications may develop. Patients with long RVOT incisions
reduce the inotropic state, and endotracheal intubation with (transannular) by necessity have severe pulmonary insufficiency
supplemental inspired oxygen. and a noncontractile infundibulum. Over time, the effects of
The natural history of untreated TOF is dismal, with most chronic right heart volume overload include right ventricular dila-
children dying of progressive cyanosis before 10 years of age. tion and decreased function, often with progressive tricuspid
Surgery is the mainstay of therapy. Medical and catheter-based insufficiency and elevated central venous pressure. These patients
therapy may be used to temporize, but TOF is a surgical disease. may present with hepatomegaly, peripheral edema, and severe
The principles of surgical correction include patch closure of the exercise intolerance. Dysrhythmias may frequently occur; patients
VSD and relief of all levels of the RVOTO and pulmonary artery with large right ventriculotomies develop endocardial scarring,
stenosis. The classic method of TOF repair uses a longitudinal which may be the substrate for ventricular tachycardia. Chronic

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1636 SECTION XI Chest

A B
FIGURE 58-23 A, Surgeons view through a transatrial incision in the transatrial/transpulmonary approach.
B, Right ventricular outflow tract muscle resection through the right atriotomy. (From Morales DL, Zafar F,
Heinle JS: Right ventricular infundibulum sparing [RVIs] tetralogy of Fallot repair: A review of over 300
patients. Ann Surg 250:611617, 2009.)

FIGURE 58-25 Minitransannular incision in the transatrial-


FIGURE 58-24 Ventricular septal defect patch closure with pledgets transpulmonary approach. (From Morales DL, Zafar F, Heinle JS: Right
around the defect and onto the tricuspid valve annulus to avoid the ventricular infundibulum sparing [RVIs] tetralogy of Fallot repair: A
conduction system. (From Morales DL, Zafar F, Heinle JS: Right ven- review of over 300 patients. Ann Surg 250:611617, 2009.)
tricular infundibulum sparing [RVIs] tetralogy of Fallot repair: A review
of over 300 patients. Ann Surg 250:611617, 2009.)

right atrial dilation may ultimately lead to atrial dysrhythmias, Pulmonary Atresia and Intact Ventricular Septum
including atrial tachycardia and fibrillation. Relative to these and Pulmonary atresia with an intact ventricular septum manifests
other potential issues after TOF repair, patients require careful with profound desaturation and ductal-dependent pulmonary
and lifelong medical follow-up. Many need reintervention; this is blood flow in newborns. The cardiac morphology in this condi-
frequently the case in patients with chronic, severe pulmonary tion varies widely. On the most severe end of the spectrum,
insufficiency, which is indicated when right ventricular dilation patients have very small right ventricles, tiny tricuspid inlets, and
and dysfunction become significant. In these patients, placing a often a right ventricledependent coronary circulation. In these
competent pulmonary valve is necessary to relieve chronic right cases, the right ventricle must remain hypertensive to provide flow
ventricular overload. These issues are of particular importance to to these segments of the coronary circulation. At the other end of
a patient with repaired TOF presenting for noncardiac surgery. A the anatomic spectrum, patients have a relatively normal tricuspid
careful assessment of the patients cardiac anatomy and function valve and right ventricle. Most patients fall in between these
is performed, including echocardiography, Holter monitoring, extremes, with some degree of tricuspid valve and right ventricle
and occasionally cardiac catheterization. underdevelopment.

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CHAPTER 58 Congenital Heart Disease 1637

lung segments into a common trunk through a process termed


Tetralogy of Fallot pulmonary artery unifocalization. Depending on the source and
size of the MAPCAs and native pulmonary arteries, this may be
a challenging surgical procedure, but the goal is constructing a
Asymptomatic Symptomatic pulmonary tree as close to normal as possible so that biventricular
repair is feasible (see earlier).
The long-term issues of repair of pulmonary atresia with VSD
>3 months and <4 kg >4 kg are similar to concerns described earlier for TOF. The addition
>4 kg of a right ventriclepulmonary artery conduit guarantees the
need for reoperation because no currently available conduit
choice offers the potential for somatic growth or an indefinitely
RVIS repair SPS *RVIS repair durable valve.

FIGURE 58-26 Algorithm for the right ventricular infundibulum-sparing Valvular Pulmonic Stenosis
(RVIS) strategy. The goal of this strategy is to minimize the right ven- Patients with isolated valvular pulmonary stenosis are almost
tricular incision and preserve the pulmonary valve. It is an individualized always treated in infancy with a percutaneous balloon pulmonary
approach that considers the patients weight, age, and overall clinical valvotomy. The intermediate-term results of this treatment are
picture. SPS, systemic-to-pulmonary artery shunt. (From Morales DL, good; however, all patients are left with significant pulmonary
Zafar F, Heinle JS: Right ventricular infundibulum sparing [RVIs] tetral- valve insufficiency and eventually require pulmonary valve
ogy of Fallot repair: A review of over 300 patients. Ann Surg 250:611
replacement.
617, 2009.)
Conotruncal Anomalies
Because patients are ductal-dependent at birth, an assessment Transposition of the Great Arteries
must be made as to whether the right heart will be capable of TGA is a common cyanotic congenital cardiac lesion. In this
ultimately supporting a biventricular circulation. If the coronary section, our discussion relates only to TGA in which there are two
circulation is truly right ventricledependent, decompressing the good ventricles identified as being capable of independent func-
right ventricle would result in coronary insufficiency. In these tion as the right and left ventricle. TGA is commonly referred to
situations, a palliative BT shunt is created in anticipation of pro- as D-TGA, in relationship to the typically normal D (dextro)
moting the patient down a single-ventricle pathway. In other ventricular looping that occurs in association with the discordant
patients, the atretic pulmonary valve must be opened with percu- ventriculoarterial connection and normal A-V connection. TGA
taneous balloon dilation or open surgical valvotomy. Over time, occurs in the setting of an intact ventricular septum (TGA-IVS)
the hypertensive, often apparently underdeveloped right ventricle or with associated VSD (TGA-VSD). In TGA-VSD, there may
will improve in size and function and become capable of support- be associated aortic arch hypoplasia and coarctation. On the other
ing all or a significant proportion of the cardiac output. At initial extreme, there may be severe pulmonic and subpulmonic stenosis
presentation, many patients have a large patent foramen ovale or (left ventricular outflow tract obstruction [LVOTO]) or even pul-
ASD; in patients with a restrictive ASD and marginal right heart, monary atresia (TGA-VSD with pulmonary atresia).
an atrial septostomy (balloon) allows for atrial-level right-to-left Patients with TGA-IVS typically present in the early newborn
shunting until the right ventricle improves. Ultimately, if the right period with profound cyanosis associated with normal perinatal
ventricle is adequate, the ASD can be closed. PDA closure. In the absence of a significant ASD, the cyanosis is
severe and progresses to death if left untreated. Administration of
Pulmonary Atresia With Ventricular Septal Defect intravenous PGE1 is almost uniformly successful in reestablishing
Pulmonary atresia with VSD is morphologically similar to TOF, ductal patency to improve the patients arterial saturation by pro-
with the exception of an atretic pulmonary valve. Patients may viding left-to-right shunting and improved pulmonary blood flow.
have confluent, normal-sized pulmonary arteries perfused by a In most patients, a balloon atrial septostomy is performed
PDA. In severe cases, the pulmonary arteries are discontinuous, (percutaneous through the umbilical vein or femoral vein) to
and the lungs are variably perfused by diminutive native branch allow atrial-level mixing (Fig. 58-27). This procedure is usually
pulmonary arteries and muscularized, collateral vessels originating effective in allowing sufficient atrial-level mixing so that the
from the descending aorta and brachiocephalic vessels. These patient is adequately saturated (70% to 80%).
major aortopulmonary collateral arteries (MAPCAs) have a pro- After the procedure, the prostaglandin infusion can be discon-
pensity to develop severe stenoses as they are exposed to systemic tinued. In TGA-VSD, there is often sufficient shunting at the level
arterial pressure. Many of these MAPCAs eventually occlude at of the VSD to promote adequate systemic saturation; in patients
an unpredictable rate during childhood. Because they may provide with large VSDs, the predominant presenting symptom may be
the only blood supply to some lung segments, patients become pulmonary overcirculation and CHF. Patients with TGA with
progressively desaturated. pulmonary atresia have ductal-dependent pulmonary blood flow.
The goal of surgical therapy for pulmonary atresia with VSD In patients with TGA-VSD and aortic arch hypoplasia or coarcta-
is biventricular repair to achieve normal cardiac workload and tion, PGE1 may be necessary to maintain ductal patency and
systemic arterial saturations. In patients with confluent native systemic perfusion. Echocardiography is the primary diagnostic
pulmonary arteries of adequate caliber, the VSD is surgically modality for TGA.
closed, and a valved conduit (homograft or heterograft) is inter- The treatment of TGA has evolved significantly during the past
posed between the right ventricle and pulmonary bifurcation. In 60 years of surgical therapy for CHD. Initial success was achieved
patients with pulmonary atresia with VSD and MAPCAs, the by surgical reconstruction to create a physiologic repair. The atrial
pulmonary arteries must be repaired by connecting the various switch operation involves a series of intra-atrial baffles using a

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1638 SECTION XI Chest

A B C

D E
FIGURE 58-28 (A-E) Five basic coronary artery configurations, as
described by Yacoub and Radley-Smith. (Adapted from Mee R: The
arterial switch operation. In Stark J, de Leval M, editors: Surgery for
congenital heart defects, ed 2, Philadelphia, 1994, Saunders, p 484.)

FIGURE 58-27 Angiogram during balloon atrial septostomy. The arrow definition before surgery; all patients undergo the ASO. In most
points to the inflated balloon catheter at the atrial septum. The inter- patients undergoing this procedure, the pulmonary artery bifurca-
ventional cardiologist forcefully pulls the balloon across the patent tion is moved anterior to the reconstructed neoaorta to minimize
foramen ovale to create an open, unobstructed secundum atrial septal the potential for pulmonary artery distortion and compression of
defect. the translocated coronary arteriesthe Lecompte maneuver (Fig.
58-29). Although there are interinstitutional biases in terms of
patch channel (Mustard procedure)30 or infolding of the native nuances of treatment for TGA, the following surgical strategies
atrial wall and interatrial septum (Senning procedure).31 Both are generally agreed on for this group of patients.
procedures achieve the same physiologic result: The systemic Transposition of the great arteriesintact ventricular septum.
venous blood is redirected to the left ventricle (and the pulmonary After balloon atrial septostomy and weaning from PGE1, if pos-
circulation), and the pulmonary venous blood is redirected to the sible, newborns with TGA-IVS undergo semielective ASO in the
right ventricle. After a successful atrial switch, patients are fully first few days to weeks of life. Rarely, patients present with pro-
saturated but are left with their morphologic right ventricle sup- found desaturation refractory to balloon atrial septostomy and
porting the entire systemic cardiac output. In many (perhaps PGE1; in this setting, an emergent ASO is indicated. We have
ultimately all) patients undergoing the atrial switch procedure, the found this to be necessary in one patient during the past decade
right ventricle becomes dysfunctional over time, which is mani- in an experience involving more than 200 ASOs performed in
fested by dilation, decreased ejection fraction, tricuspid insuffi- newborns. For other patients, the ASO needs to be performed in
ciency, and dysrhythmias. The observation of problems with the a timely but nonemergent setting. Even in the presence of ade-
systemic right ventricle in patients after the atrial switch operation quate systemic saturation, the patients morphologic left ventricle
was the primary impetus behind the development and application is functioning in a low-pressure work environmentsupporting
of the arterial switch operation (ASO), which is now established the pulmonary circulation. Thus, left ventricle mass and function
as the surgical treatment of choice for patients with TGA. involute rapidly in the first few weeks of life. By 6 weeks of life,
At the present time, operative survival rates for the ASO the left ventricle may be incapable of supporting the normal
approach 100%.32,33 systemic workload after the ASO. As such, the preferred timing
The ASO provides physiologic and anatomic correction of for the operation is in the first 1 to 2 weeks of life.
TGA by establishing ventriculoarterial concordance. The proce- Transposition of the great arteriesventricular septal defect
dure involves transection and translocation of the malposed great with or without arch hypoplasia. There are several modes of pre-
vessels. The technically challenging requirement of the ASO sentation for patients with TGA-VSD. In patients with small,
relates to the translocation of the coronary arteries to the pulmo- pressure-restrictive VSD, the presenting symptoms are similar to
nary root (the neoaorta). As noted, there are numerous possible those of TGA-IVS. These patients require the ASO early in life,
branching patterns for the coronary arteries in TGAsome are along with VSD closure before left ventricle involution. In patients
easily transferred in the ASO, whereas others are more challenging with TGA and nonrestrictive VSD, there may be adequate mixing
(including single coronary ostium and intramural course) (Fig. to allow reasonable systemic arterial saturation. In this setting,
58-28).34 Nonetheless, precise surgical techniques have been the left ventricle remains pressure-loaded and does not involute;
described and successfully applied to all coronary branching pat- thus, the necessity of early promotion to the ASO is less time-
terns. Given this as well as the known benefit of aligning the compressed. Many newborns with TGA and a large VSD are rela-
morphologic left ventricle with the systemic circulation, the ASO tively asymptomatic soon after birth; they go on to develop CHF
is offered to all patients with TGA regardless of the coronary in the first 1 to 2 months of life as the normal decrease in newborn
branching pattern. There is no need for precise anatomic pulmonary resistance occurs. Our preference for these patients is

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CHAPTER 58 Congenital Heart Disease 1639

hypoplasia or coarctation, early surgery is required. In this setting,


the preferred treatment involves one-stage, complete correction,
Ao
including ASO, VSD closure, and aortic arch repair.
Transposition of the great arteriesventricular septal defect
PA
PA with pulmonary stenosisleft ventricular outflow tract obstruction
(Neoaorta) or pulmonary atresia. The issue of concern in this group of
LCA patients is the degree of LVOTO. In patients with TGA-VSD and
organic LVOTO, with a relatively normal pulmonary valve, the
RCA treatment strategy is as described earlier, with ASO, VSD closure,
and left ventricular outflow tract (LVOT) resection. The situation
RCA becomes more complex in the setting of severe pulmonary stenosis
or pulmonary atresia. These patients may be ductal-dependent as
newborns (pulmonary atresia) and require newborn complete
A correction or a palliative Blalock shunt in the newborn period,
LCA
followed by biventricular repair later in infancy (our preference).
B The goal in these patients is to achieve biventricular repair to
create an unobstructed connection between the morphologic left
ventricle and systemic circulation. Several operations have been
Distal described and successfully used in this setting.
aorta The Rastelli procedure involves an interventricular patch baffle,
which commits the left ventricle to the aorta through the VSD.
Typically, a right ventriclepulmonary artery conduit is then
placed to achieve pulmonary blood flow. Issues of concern include
the potential for LVOTO (at or below the level of the VSD) and
the certain need for future right ventriclepulmonary artery
Neoaorta conduit revision. The REV procedure is designed to minimize the
potential of LVOT obstruction and to use all possible native
tissue-tissue connections to limit the potential need for future
C surgery. This procedure involves resection of the muscular conus
between the aorta and pulmonary roots, interventricular baffle of
the left ventricle to the aorta, and translocation of the native main
pulmonary artery to the right ventricle (by a Lecompte maneuver)
without the use of an intervening conduit. The final option
involves aortic root translocation, which includes resection of
the entire native aortic root and coronary origins, resection of the
intervening muscular conus, and posterior translocation of the
Pericardial aortic root to the surgically enlarged pulmonary root to achieve a
patch direct connection between the left ventricle and aorta. The VSD
repair is then closed, and a conduit is placed or a direct connection is
D Neoaorta created between the right ventricle and pulmonary arteries.
Transposition of the great arteries in adults. The long-term
prognosis of adult patients who have undergone childhood repair
Neopulmonary artery
of TGA is still incompletely understood; however, all these patients
E
require lifelong surveillance and have the potential of developing
FIGURE 58-29 Arterial switch operation. A, The aorta (Ao) and pulmo- significant anatomic and functional cardiac problems. Patients
nary artery (PA) are transected above the sinuses of Valsalva. B, The
who were treated with the atrial switch operation have a morpho-
coronaries are excised from the aorta and anastomosed to the pulmo-
nary artery using a trapdoor technique. C, The distal aorta is brought
logic right ventricle supporting their systemic circulation, which
behind the pulmonary artery (Lecompte maneuver) and anastomosed will predictably fail in many patients. Although fully saturated,
to the neoaorta. D, Separate pericardial patches are sutured to replace these patients may present later in life with signs and symptoms
the excised coronary artery tissue from the aorta. E, Completed repair. of CHF and dysrhythmia. For severely affected individuals,
LCA, Left coronary artery; RCA, right coronary artery. (Adapted from the only realistic treatment option may ultimately be cardiac
Karl TR, Kirshbom PM: Transposition of the great arteries and the arte- transplantation.
rial switch operation. In Nichols DG, Ungerleider RM, Spevak PJ, etal, The long-term issues related to the ASO are less well under-
editors: Critical heart disease in infants and children, Philadelphia, 2006, stood. Despite technical advances in reconstructive methods,
Mosby, p 721.) there is still a troubling incidence of postoperative supravalvular
and branch pulmonic stenosis. The neoaortic root may dilate in
some patients undergoing the ASO, leading to neoaortic insuffi-
to follow them closely for evidence of CHF and perform semielec- ciency and coronary artery distortion. The fate of the surgically
tive ASO and VSD closure in the first 4 to 6 weeks of life. Some translocated coronary ostia is unclear; there is clearly a risk for late
centers prefer to proceed with this surgery sooner; this appears sudden cardiac death related to unsuspected coronary insuffi-
to be a matter of surgeon preference and has not been shown to ciency noted elsewhere in this chapter. For an adult patient under-
affect long-term outcome. In patients with TGA-VSD with arch going noncardiac surgery after previous surgery for complex

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1640 SECTION XI Chest

congenital cardiac disease, including TGA, a high index of suspi- patients with systemic right ventricular dysfunction, one option
cion is warranted. for treatment is a complex reconstruction known as a double
switch (Fig. 58-30). This procedure includes an atrial switch in
Double-Outlet Right Ventricle combination with an arterial switch to align the morphologic left
Double-outlet right ventricle occurs when both great vessels are ventricle with the systemic circulation. In almost all patients with
anatomically committed to the right ventricle. This condition may ccTGA-IVS and right ventricular dysfunction (and in the absence
occur in association with a subaortic VSD, a noncommitted of structural LVOTO), a period of left ventricle retraining is
(remote) VSD, or a subpulmonary VSD (Taussig-Bing anomaly). required before the double-switch procedure. This requirement
As with other complex cardiac conditions, the goal of treatment relates to the fact that the left ventricle will have been functioning
relates to the presenting hemodynamic conditions and patient in the low-pressure pulmonary circulation and will be incapable
symptoms. The ultimate goal is to achieve a biventricular circula- of performing systemic work. Retraining or conditioning the left
tion when possible. Patients may present with severe cyanosis and ventricle requires the surgical creation of pulmonary stenosis by
require corrective or palliative therapy in the newborn period. the placement of a pulmonary artery band. Most surgeons agree
Conversely, they may present with unrestricted pulmonary blood that the left ventricle must work at or very near systemic blood
flow and develop CHF. The challenging issue of constructing a pressure for many months (we favor a minimum of 6 months)
biventricular repair relates to achieving unobstructed outlets from before the double-switch operation. The double switch is a techni-
the right and left ventricles. In patients with double-outlet right cally challenging operation, with significant perioperative risk.
ventricle with subaortic VSD and RVOTO, reconstruction is Because of the small numbers of patients treated worldwide with
similar to that for TOF. More remote VSDs may require enlarge- this complicated surgical strategy, there are only limited data of
ment with interventricular tunnel repair. For the Taussig-Bing the acute and midterm results.35 An issue of concern centers
anomaly, the relationship of the VSD to the pulmonary artery on the long-term ability of the retrained left ventricle to function
makes the ASO the procedure of choice. These patients often have as the systemic ventricle. Nonetheless, patients with ccTGA and
RVOTO and aortic arch hypoplasia, which require attention at depressed right ventricular function have a poor prognosis other-
the time of complete correction. For rare individuals, the relation- wise, and, as such, the complexity and risk of the double-switch
ship of the great vessels and complexity of the VSD preclude a operation appear justified. The only other surgical option for these
biventricular repair, and the patient must be treated as if he or she patients is cardiac transplantation.
has a functional single ventricle. Congenitally corrected transposition of the great arteries with
ventricular septal defect and pulmonic stenosis. Patients in this
Congenitally Corrected Transposition category are often well balanced and have mild cyanosis, with
of the Great Arteries (L-Transposition) minimal symptoms in childhood, whereas others with more severe
ccTGA, or L-TGA, describes a constellation of conditions with the pulmonary stenosis or pulmonary atresia present early in life with
common feature of A-V and ventriculoarterial discordance. ccTGA symptomatic cyanosis. Treatment for an overtly cyanotic infant
may occur in association with VSD, pulmonic and subpulmonic with ccTGA with pulmonary stenosis is initially palliative in the
stenosis, and displaced left A-V valve (Ebsteinoid left A-V valve). form of a Blalock shunt. The ultimate goal for all patients is a
In ccTGA, the morphologic mitral valve is right-sided and associ- biventricular circulation, with normal arterial oxygen saturation.
ated with the morphologic left ventricle; the morphologic tricuspid One option for these patients is to close the VSD surgically and
valve is associated with the morphologic right ventricle. Patients place a conduit between the morphologic left ventricle and pul-
with this condition are physiologically corrected in that in the monary arteries to relieve the pulmonary obstruction. This classic
absence of ventricular-level shunting, they are fully saturated repair benefits the patient by separating the systemic and pulmo-
hence, the term corrected transposition. The age and mode of patient nary circulations and allowing normal oxygen tension. The issue
presentation in this condition depend on the contribution of asso- of concern in patients undergoing this repair is that the morpho-
ciated defects and the function of the morphologic right ventricle, logic right ventricle must act independently as the systemic ven-
which acts as the systemic ventricle. Controversy exists regarding tricle after repair. As noted, the ability of the right ventricle to
the timing and mode of surgical treatment for patients presenting support the systemic circulation may be questionable over the
with various manifestations of ccTGA. long-term in some patients. As such, an alternative strategy in
Congenitally corrected transposition of the great arteries with these patients is to baffle the left ventricular outflow to the aorta
intact ventricular septum. Patients with ccTGA-IVS may be through the VSD, then to perform an atrial switch to reroute the
entirely asymptomatic throughout childhood and early adult- systemic and pulmonary venous return, and finally to place a
hood. Frequently, the diagnosis is made incidentally. In other conduit from the morphologic right ventricle to the pulmonary
patients, the disease manifests with symptoms of CHF in associa- arteries. This option is a modification of the double-switch
tion with right ventricular dysfunction or left A-V valve insuffi- arrangement, affording the patient the benefit of a systemic left
ciency. There is also a high incidence of complete heart block in ventricle. Because the left ventricle has been working at systemic
patients with ccTGA, and the first manifestation may be this pressure before correction, a period of retraining is unnecessary.
dysrhythmia, with associated symptoms. Adult patients with ccTGA, with or without previous surgery,
Treatment for patients presenting with CHF is a challenging warrant careful attention before any noncardiac operation. These
management scenario. For patients with ccTGA and preserved patients may have various complex ongoing cardiac issues, includ-
right ventricular function, left A-V valve repair or replacement ing rhythm disturbance, ventricular dysfunction, and valvular
may be considered. In many of these patients, the valvular insuf- insufficiency.
ficiency may be more a manifestation of declining systemic RV
function, with septal shift and annular dilation, rather than intrin- Left Ventricular Outflow Tract Obstruction
sic valve pathology. In this setting, valve replacement would LVOTO may manifest in isolation or in combination with other
not correct the progression of right ventricular dysfunction. For complex cardiac lesions. The physiologic consequences of severe

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CHAPTER 58 Congenital Heart Disease 1641

Aorta Right ventricle


Foramen Tricuspid
ovale valve Mitral valve

Right Coronary
atrium sinus
Inferior
vena cava

Superior
vena cava

A Pulmonary veins B C
Mitral Right
valve ventricle

Aorta

Tricuspid
valve
Inferior
vena cava

Superior
vena cava

D E Pulmonary veins
FIGURE 58-30 Schematic representation of the Senning procedure for transposition of the great arteries.
A, Two separate incisions, one in the right atrium and the other in the left atrium near the insertion of the
pulmonary veins. B, Location of the incision in the atrial septum. C, The atrial septum sewn down to the
pulmonary veins preparing for oxygenated blood to be directed to the tricuspid valve. The inferior free wall
of the right atrium is sewn along the cut edge of the atrial septum redirecting the deoxygenated blood to
the mitral valve. D, The superior free wall of the right atrium is now sewn to the cut edge of the left atrium,
redirecting the oxygenated blood from the pulmonary veins to the tricuspid valve. E, Schematic representa-
tion of the oxygenated blood and deoxygenated blood pathways. (Reprinted with permission from Texas
Childrens Hospital, 2016.)

LVOTO may be catastrophic, including diminished systemic patients with critical AS with unidentifiable valve commissures
cardiac output and tremendous left ventricular pressure overload. and annular hypoplasia. Infants presenting with critical AS are
Newborns with severe LVOTO may present in shock with dimin- often symptomatic early in the newborn period, presenting with
ished peripheral perfusion, cardiomegaly, and pulmonary conges- shock and profoundly depressed ventricular function. At the
tion. There is a significant risk for necrotizing enterocolitis in these present time, almost all patients are taken to the cardiac catheter-
infants. In older patients, gradual onset of LVOTO may be ini- ization laboratory for balloon aortic valvotomy. This procedure
tially asymptomatic, only to manifest over time as decreasing may be lifesaving in relieving AS and allowing for recovery of
exercise tolerance and declining left ventricular function. Patients ventricular function. However, for most patients, the procedure
with severe LVOTO and cardiomegaly are at high risk for myo- is palliative, with a significant incidence of recurrence of AS or
cardial ischemia and sudden cardiac death. The resting ECG often development of significant aortic insufficiency after the procedure.
demonstrates left ventricular hypertrophy, with a strain pattern. In patients with AS refractory to balloon dilation, an open aortic
If an exercise stress test is performed, it may demonstrate worri- valvotomy may be necessary (Fig. 58-31). A surgical valvotomy,
some ST segment depression and ventricular dysrhythmias. Echo- especially in small infants with adequate annular dimension, can
cardiography is the primary diagnostic tool for patients with be accomplished by an accurate incision down a rudimentary
LVOTO. In rare cases, diagnostic cardiac catheterization may be commissure or raphe to improve cusp mobility.
considered to delineate the level of obstruction. Recurrent AS after previous ballooning may be amenable to
repeat dilation; however, when associated with significant aortic
Valvular Aortic Stenosis insufficiency, the patient requires surgery. Severe aortic insuffi-
Congenital valvular aortic stenosis (AS) is a common cause of ciency after previous balloon dilation is usually related to an
LVOTO. The degree of obstruction may range from mild in avulsed cusp. In these cases, valve repair may be possible, but
patients with a congenitally bicuspid aortic valve to severe in replacement may become necessary. Published series have

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1642 SECTION XI Chest

Ao
PA

A
Pulmonary
autograft
A
FIGURE 58-31 Close-up of the aortic valve demonstrating a surgical
valvectomy. A, The valve is bicuspid, with a prominent raphe in the
Lt
anterior valve leaflet. B, The orifice is enlarged by incising the fused coronary
commissure between the two leaflets. (From Chang AC, Burke RP: Left
ventricular outflow tract obstruction. In Chang AC, Hanley FL, Rt
Wernovsky G, etal, editors: Pediatric cardiac intensive care. Baltimore, coronary
1998, Williams & Wilkins.) Septal
perforator
B
confirmed the usefulness of aortic valve repair procedures, which
is a particularly attractive option for growing children.36-38
The decision to replace the aortic valve in growing children is
clouded by the lack of an ideal aortic valve substitutea valve
capable of lifelong durability, appropriate somatic growth, easily
implantable, and not requiring anticoagulation. Criteria for aortic
valve replacement are beyond the scope of this chapter; however, Ao
severe valvular AS not amenable to catheter or open valvotomy is PA
an appropriate indication. Options for aortic valve replacement Pulmonary
in children include a mechanical prosthesis, heterograft, homo- autograft Cryopreserved
pulmonary
graft, and pulmonary autograft. A mechanical prosthesis may be allograft
considered in childhood; however, the valve size must be sufficient
to provide adequate function as the patient grows. Most surgeons
and cardiologists recommend therapeutic anticoagulation in chil-
dren with a mechanical valve prosthesis, but this can be challeng- C
ing and potentially dangerous in growing children and adolescents.
FIGURE 58-32 Ross procedure. A, The great arteries are transected
Many surgeons believe the risk for such medical treatment out-
above the sinotubular ridge. The coronary arteries are excised using
weighs the potential benefit of a theoretically durable valve. coronary artery buttons. B, The pulmonary autograft is excised from the
Heterograft aortic valve prostheses historically have been asso- right ventricular outflow tract, and the proximal end of the autograft is
ciated with limited durability in children and are not capable of anastomosed to the annulus. C, The coronary artery buttons are anas-
somatic growth. Currently available heterograft prostheses have tomosed to the pulmonary autograft. Ao, Aorta; PA, pulmonary artery.
not undergone sufficient use in children to provide useful data (Adapted from St Louis JD, Jaggers J: Left ventricular outflow tract
concerning improved durability. Human cadaver aortic valves obstruction. In Nichols DG, Ungerleider RM, Spevak PJ, etal, editors:
(aortic homograft) have been used extensively in children and Critical heart disease in infants and children, Philadelphia, 2006, Mosby,
young adults. These valves are usually implanted as a complete p 615.)
aortic root replacement, which requires coronary ostial implanta-
tion. Thus, surgery to place an aortic homograft is considerably
more complex and with potentially higher risk. The positive theoretical advantages of the Ross procedure include the potential
features of an aortic homograft include improved durability for somatic growth, avoidance of anticoagulation, and possibility
compared with heterograft and avoidance of anticoagulation. of extended durability. Enthusiasm for this procedure has been
Nonetheless, these valves eventually fail, necessitating a compli- tempered by the recognition that the need for extension cardiac
cated reoperative aortic root replacement. dissection to harvest the autograft, along with a more complex
Pulmonary autograft aortic root replacement (Ross operation) implantation, is associated with increased operative risk. Further-
involves translocation of the pulmonary valve to the aortic posi- more, the unsupported pulmonary root may dilate in the presence
tion with subsequent replacement of the pulmonary valve with a of systemic arterial pressure, leading to progressive autograft aortic
homograft or heterograft valved conduit (Fig. 58-32). The insufficiency. This observation has led to various modifications of

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CHAPTER 58 Congenital Heart Disease 1643

the implantation technique to support the aortic annulus and the Nonetheless, with careful technique, the risk for these complica-
sinus segment. Given these considerations and the certain need tions is minimized.
for reoperation to replace the right ventricularpulmonary artery
conduit, great caution must be exercised in the application of the Tunnel Subaortic Stenosis
Ross operation.39 Tunnel subaortic stenosis is a more severe form of LVOTO that
is often associated with aortic annular hypoplasia and valvular AS.
Fibromuscular Subaortic Stenosis In severe cases, the LVOTO is not amenable to subaortic resection
This condition is a progressive narrowing of the LVOT related to alone. In this situation, an aortic rootenlarging procedure may
a dense fibrous membrane usually found in association with asym- be necessary to relieve the obstruction (aortoventriculoplasty,
metrical protrusion of the interventricular septum into the outflow or Konno procedure). This complex reconstruction generally is
tract. The membrane is often concentric and becomes densely associated with the necessity of aortic valve replacement using
adherent to the septum and mitral valve. The membrane pro- one of the aforementioned options. Moreover, all degrees of
gresses toward and eventually onto the undersurface of the aortic LVOTO may be seen in association with numerous left heart
valve cusps, which leads to progressive LVOTO, aortic valve cusp obstructive lesions (Shone syndrome41) that may require extensive
retraction, and aortic insufficiency. reconstruction.
Echocardiography is the primary diagnostic tool when assess-
ing the degree of obstruction and progression of subaortic steno- Aortic Arch Anomalies
sis. However, it is not accurate for assessing subtle degrees of cusp Aortic Coarctation
extension.40 Cardiac catheterization is rarely needed to diagnose Coarctation of the aorta is one of the most frequently encountered
this condition; balloon dilation is of no use in treating LVOTO. congenital cardiac lesions. This condition has a wide range of
Surgery is the mainstay of treatment for subaortic stenosis, but presentations, from a severely symptomatic newborn with CHF
there is disagreement about surgical indications. Most surgeons and depressed ventricular function to an adult with proximal
believe that new onset of any degree of aortic insufficiency in hypertension and minimal symptoms. Coarctation is classified
association with a subaortic membrane, regardless of the pressure relative to its association with the ligamentum arteriosus and
gradient, is an indication for surgery. In other patients, an escalat- aortic arch. An infantile or preductal aortic coarctation is seen in
ing LVOT gradient, associated left ventricular hypertrophy, and combination with a large PDA, which may have predominantly
appropriate anatomic substrate are acceptable indications for right-to-left flow to the lower descending aorta. In this setting,
operation. the patient is ductal-dependent for systemic blood flow until the
The surgical procedure for subaortic stenosis involves a trans- coarctation is repaired, and a PGE1 infusion must be maintained
aortic resection of the subaortic membrane, including all attach- to prevent ductal closure. A periductal or juxtaductal coarctation
ments to the mitral valve, septum, and aortic valve cusps. A septal occurs in the region of the ductal insertion and is distal to the
myectomy is performed, along with membrane resection, in most aortic isthmus, which may be normal or hypoplastic (Fig. 58-34).
patients (Fig. 58-33). Complications include membrane recur- Aortic coarctation with or without aortic arch hypoplasia is
rence, injury to the bundle of His, and iatrogenic VSD creation. frequently associated with intracardiac anomalies, including

AV cusp

A B
FIGURE 58-33 A, Excision of discrete subaortic stenosis. The aorta is opened obliquely, and the aortic valve
leaflets are retracted to expose the subaortic membrane. The membrane is excised circumferentially (dotted
line). B, This is usually combined with a muscle resection. (From de Leval M: Surgery of the left ventricular
outflow tract. In Stark J, de Leval M, editors: Surgery for congenital heart defects, ed 2, Philadelphia, 1994,
Saunders.)

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1644 SECTION XI Chest

Ao Coarctation Ao Coarctation Left carotid artery

PDA Innominate a. Left subclavian


artery
PA PA
Left recurrent
Ao
Ligamentum laryngeal nerve
arteriosum Coarctation
A B PA

FIGURE 58-34 Coarctation of the aorta (Ao). A, Infantile or preductal


Intercostal
coarctation. B, Adult coarctation. PA, Pulmonary artery; PDA, patent artery
ductus arteriosus. (From Backer CL, Mavroudis C: Coarctation of LA
the aorta. In Mavroudis C, Backer CL, editors: Pediatric heart surgery.
Philadelphia, 2003, Mosby, p 252.)
A
multiple left heart obstructive lesions (e.g., mitral stenosis, left
ventricular hypoplasia or endocardial fibroelastosis, subaortic ste-
nosis or AS) known as Shone syndrome.41 Patients with large
VSDs may present in infancy with severe aortic coarctation, with
or without subaortic stenosis.
Aortic coarctation may be suspected on clinical examination
by a significant upper extremitylower extremity blood pressure
End-to-end
gradient and diminished or absent femoral and pedal pulses. In anastomosis
older patients with well-developed intercostal collateral arteries, a PDA
continuous murmur may be auscultated over the posterior thorax.
Echocardiography is now the primary diagnostic modality for
aortic coarctation. MRI and CT angiography may also be useful
in some patients. In rare cases, cardiac catheterization is required
to define the anatomy, but this modality is now used more fre-
quently for treatment, including balloon dilation with or without
stenting.
Treatment strategies for aortic coarctation have evolved signifi-
cantly since the first successful surgical treatment almost 70 years
Patch
ago. Newborns presenting with severe aortic coarctation with or augmentation Subclavian
without associated ductal-dependent systemic blood flow are best flap Extended resection
treated by surgery. Initial enthusiasm regarding balloon dilation aortoplasty with primary anastomosis
in these patients has diminished as it has become clear that there
is a high incidence of recurrent coarctation after neonatal dilation.42
Most congenital cardiac surgeons perform isolated coarctation B
repair through a left thoracotomy incision (third or fourth inter-
FIGURE 58-35 Surgical repair for aortic coarctation. A, Surgical inci-
space) using resection of the coarctation and primary anastomosis.
sion and anatomic orientation. B, Four different methods are shown:
For patients with relative hypoplasia of the distal aortic arch, the end-to-end anastomosis, patch augmentation, subclavian flap aorto-
anastomosis can be brought along the lesser curve of the aortic plasty, and extended resection with primary anastomosis. Ao, Aorta;
arch using an extended end-to-end method. For coarctation with LA, left atrium; PA, pulmonary artery. (Adapted from Hastings LA,
a hypoplastic transverse arch, we favor the aortic arch advancement Nichols DG: Coarctation of the aorta and interrupted aortic arch. In
procedure at Texas Childrens Hospital, which uses all native tissue Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Critical heart
repair to allow for growth.43 Other methods include subclavian disease in infants and children, Philadelphia, 2006, Mosby, p 635.)
artery flap aortoplasty (Waldhausen method) and prosthetic patch
aortoplasty. These latter methods are used less frequently than
primary repair (Fig. 58-35). Catheter therapy as a primary treat- Another controversial issue surrounds the concomitant treat-
ment for aortic coarctation is a controversial therapy in the opinion ment of coarctation and significant intracardiac pathology. Several
of most surgeons. Although this methodology has been widely series have demonstrated superior outcomes for simultaneous
applied, its true comparability to surgery requires further prospec- therapy in selected groups of patients, including neonates with
tive study. There are several issues of concern regarding angioplasty large VSDs and coarctation with arch hypoplasia. Our approach
for coarctation. The balloon dilation results in transmural disrup- to this condition has included one-stage complete repair of intra-
tion of the aortic wall in many patients, and there is an acute and cardiac defects along with aortic arch advancement through
ongoing risk for aneurysm formation. To limit this risk and mini- median sternotomy on cardiopulmonary bypass.
mize the potential of recurrence, off-label use of stents has been
done for treatment of coarctation. Obvious issues of concern Interrupted Aortic Arch
include somatic growth and lifetime risk potential of a metal device IAA results from lack of proper fusion and involution of the fetal
in the descending aorta. aortic arches. This is a fatal condition without treatment, and IAA

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CHAPTER 58 Congenital Heart Disease 1645

RCA LCA preserved to afford the patient the best possible long-term
LCA outcome.
LSA The rapid evolution of successful palliation for patients with
RSA LSA
various forms of single-ventricle physiology since the late 1970s
PDA has led to a large and growing population of adults with a single
ventricle. For most of these patients, lifelong cardiac attention is
needed, and the potential for subsequent cardiac reoperation is
high. Patients in this category who present for noncardiac surgery
DAo may be especially difficult to manage because of their challenging
physiology.
AAo MPA An exhaustive discussion of the various forms of single ven-
tricle is well beyond the scope of this chapter. This discussion is
Type A Type B
limited to common forms of single right and left ventricles to
provide examples of the surgical management strategies for a
single ventricle.
LCA LSA
Tricuspid Atresia
Tricuspid atresia is the template of a single-ventricle lesion for
which most current palliative strategies were developed. Patients
with tricuspid atresia have a single morphologic left ventricle and
may have normally related or transposed great vessels (Fig. 58-38).
DAo They may present with excessive pulmonary blood flow and
require pulmonary artery banding early in infancy to relieve pul-
monary overcirculation and CHF. Conversely, patients may have
pulmonary stenosis or pulmonary atresia and require creation of
Type C a Blalock shunt to provide adequate pulmonary blood flow and
FIGURE 58-36 Classification of interrupted aortic arch. AAo, Ascend- systemic oxygenation.
ing aorta; DAo, descending aorta; LCA, left common carotid artery; LSA, As noted, the initial palliative goals in patients with tricuspid
left subclavian artery; MPA, main pulmonary artery; RCA, right common atresia include adequate systemic oxygenation, protection of ven-
carotid artery; RSA, right subclavian artery. (Adapted from Monro JL: tricular function, and adequate pulmonary arterial growth.
Interruption of aortic arch. In Stark J, de Leval M, editors: Surgery for
Patients with ductal-dependent pulmonary blood flow require a
congenital heart defects, ed 2, Philadelphia, 1994, Saunders, p 299.)
Blalock shunt in the newborn period. We prefer to construct the
shunt to the morphologic right pulmonary artery through a right
is frequently associated with serious intracardiac pathology. IAA thoracotomy. This construction allows shunt flow to be governed
is classified based on the level of the interruption. Type A is distal by the size of the subclavian artery. Furthermore, the right pul-
to the left subclavian artery, type B occurs between left subclavian monary artery is typically longer and runs in a more horizontal
and common carotid arteries, and type C occurs proximal to the plane compared with the left pulmonary artery; this facilitates
left subclavian artery (Fig. 58-36). There is a frequent finding of avoiding distortion of a lobar branch. The goal of the shunt is to
an aberrant right subclavian artery (retroesophageal) from the protect the pulmonary arteries, promote adequate pulmonary
descending aorta. Survival for patients with IAA is initially predi- artery development, and support systemic arterial oxygenation for
cated on ductal patency; thus, a PGE1 infusion is required to the first 4 to 6 months of life until the next planned stage of pal-
stabilize the patient. Diagnosis is confirmed by echocardiography; liation (see later discussion of Glenn and Fontan operations). The
other methods, including cardiac catheterization, are needed shunt is not designed for long-term use; thus, in most patients, a
infrequently. small interposition graft (expanded polytetrafluoroethylene, 3.0
IAA requires surgical treatment in the newborn period, which to 4.0mm) is selected. In the early era of single-ventricle pallia-
typically involves simultaneous repair of intracardiac lesions (Fig. tion, less well-controlled shunts were constructed, including
58-37). Repair may be accomplished with the aid of an aortic arch classic Blalock (divided native subclavian artery-to-branch pulmo-
augmentation patch, although researchers at Texas Childrens nary artery), Potts (side-to-side left pulmonary artery to descend-
Hospital reported a series confirming that a primary tissue-tissue ing aorta), and Waterston (side-to-side right pulmonary artery to
repair can be performed in most patients and minimizes the ascending aorta) shunts (Fig. 58-39). These native tissue-tissue
potential for recurrent aortic arch obstruction.44 connections are capable of somatic growth but have the con-
founding risks for pulmonary overcirculation, pulmonary artery
hypertension (potentially irreversible), and branch pulmonary
artery distortion with hypoplasia. During the early stages of devel-
SINGLE VENTRICLE opment of single-ventricle palliation, many patients were treated
Single-ventricle physiology is a frequently encountered form of with these poorly controlled shunts. Thus, significant numbers of
CHD. Patients may present as newborns with inadequate pulmo- adult patients present with complications of these palliations,
nary blood flow, excessive pulmonary blood flow, or balanced including chronic cardiac volume overload and decreased ven-
circulations. The single ventricle may be of right, left, or indeter- tricular function, severe pulmonary artery distortion or isolation,
minate morphology. Surgical treatment is required to provide pulmonary vascular disease, and profound cyanosis. These patients
adequate systemic oxygen delivery, while protecting the pulmo- may present for surgery for noncardiac illness and are extremely
nary vasculature. The function of the single ventricle must be difficult to manage.

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1646 SECTION XI Chest

A B

C D
FIGURE 58-37 A, Type B interrupted aortic arch. B, Cannulation and site of incision for repair. The descend-
ing thoracic aorta is brought upward into the mediastinum (C) and then anastomosed to the ascending aorta
in an end-to-side fashion (D). (From Hirooka K, Fraser CD: One-stage neonatal repair of complex aortic arch
obstruction or interruption. Tex Heart Inst J 24:317321, 1997.)

Ao Ao Ao
PA PA PA
LA LA LA
RA RA RA

RV LV RV LV RV LV

CoA
Ao Ao Ao
PA PA PA
LA LA LA
RA RA RA

RV LV RV LV RV LV

FIGURE 58-38 Anatomy of the various types of tricuspid atresia. Top, Normally related great vessels.
Bottom, D-Transposition of the great vessels. Ao, Aorta; CoA, coarctation of the aorta; LA, left atrium; LV,
left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle. (Adapted from Lok JM, Spevak PJ,
Nichols DG: Tricuspid atresia. In Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Critical heart disease
in infants and children, Philadelphia, 2006, Mosby, pp 800801.)

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CHAPTER 58 Congenital Heart Disease 1647

CLASSIC RIGHT BLALOCK-TAUSSIG

RSA

Potts

RPA LPA
Waterston
MPA
Ao

RIGHT MODIFIED BLALOCK-TAUSSIG

RIA FIGURE 58-40 Anatomy of hypoplastic left heart syndrome. The tiny
ascending aorta is seen arising from a markedly hypoplastic left ven-
tricle. The ductus arteriosus is large, providing forward flow to the
systemic circuit. The right ventricle is hypertrophied, and the pulmonary
artery is enlarged. (From Wernovsky G, Bove EL: Single ventricle
Ao
RPA lesions. In Chang AC, Hanley FL, Wernovsky G, etal, editors: Pediatric
LPA cardiac intensive care, Baltimore, 1998, Williams & Wilkins.)

MPA
Central
After delivery, medical treatment is directed at maintaining
ductal patency and balancing systemic and pulmonary blood flow.
FIGURE 58-39 Systemic-to-pulmonary artery shunts. Ao, Aorta; LPA, Balancing the circulations becomes increasingly challenging with
left pulmonary artery; MPA, main pulmonary artery; RIA, right innomi- the normal decline in neonatal PVR, resulting in massive pulmo-
nate artery; RPA, right pulmonary artery; RSA, right subclavian artery. nary overcirculation. As the overcirculation progresses, infants
(Adapted from Marino BS, Wernovsky G, Greeley WJ: Single-ventricle become tachypneic and may exhibit decreased systemic perfusion.
lesions. In Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Criti- Necrotizing enterocolitis is a significant risk in these children, and
cal heart disease in infants and children, Philadelphia, 2006, Mosby, if there is any question of visceral malperfusion, many centers
p 793.) avoid enteral nutrition in an effort to minimize this potential.
Other medical maneuvers include deliberate hypoventilation, low
inspired oxygen concentration, and additional carbon dioxide in
an attempt to increase PVR and limit pulmonary flow. These
Hypoplastic Left Heart Syndrome options are of limited use in newborns with HLHS; over days to
Hypoplastic left heart syndrome (HLHS) is the prototypical weeks, the infants become progressively ill, with pulmonary con-
single right ventricle. Patients with this condition present with gestion and marginal systemic cardiac output. Patients who are
inadequate left heart structures ranging from mitral stenosis and maintained have the potential of developing increased PVR as
AS with left ventricular hypoplasia to almost complete absence of they age, and there is a known association with advanced age (>30
the left heart structures with aortic and mitral atresia. In the case days) and increased operative mortality.
of aortic and mitral atresia, the ascending aorta is typically small Surgery in the newborn period is the only realistic option for
(1 to 2mm) and is perfused through retrograde aortic arch flow long-term survival in infants born with HLHS. Outcomes for
provided by the PDA. In HLHS, ductal closure results in rapid surgical palliation of HLHS have come to be synonymous with
cardiovascular collapse, with profound systemic hypoperfusion the reputation of the treating center and surgeons. As with tricus-
and hypoxia, followed quickly by death. Therefore, in cases of pid atresia, patients with HLHS require a staged palliative
prenatal diagnosis, patients must be born in a facility qualified to approach. In the experiences of all centers, the first stage is the
institute appropriate medical management immediately, includ- most challenging and risk-laden. The various first-stage options
ing the establishment of suitable vascular access (umbilical artery are described in the following sections.
catheter) and institution of intravenous PGE1 to maintain ductal
patency. Patients with HLHS undiagnosed at birth typically have Neonatal Cardiac Transplantation
an early grace period of a few hours, but with the initiation of Transplantation is a theoretically attractive option in infants with
ductal closure, these children become critically ill and require HLHS that replaces the malformed heart with a structurally
aggressive resuscitation for survival. Although most children with normal one. Leonard Bailey has been an influential champion of
HLHS are otherwise normal, without treatment, HLHS is a uni- this approach and was the first to report exciting results with
formly fatal condition (Fig. 58-40). transplantation in newborns with HLHS.45 Furthermore, although

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1648 SECTION XI Chest

there is an ever-present risk for rejection and infection in children the accurate connection of this often-miniscule ascending aorta
with heart transplants, long-term meaningful survival is possible, to the confluence of the arch and main pulmonary artery stump.
and the quality of life of the recipients is good. The option of The risk for torsion and coronary insufficiency is high. The final
cardiac transplantation is limited by the small numbers of suitable element of the classic Norwood reconstruction is the creation of
donor hearts, and most children with HLHS are unable to a controlled source of pulmonary blood flow in the form of a
survive the wait time for a donor graft. This situation has led most modified BT shunt (Fig. 58-41).
centers, including Texas Childrens Hospital, to abandon cardiac
transplantation as the primary mode of therapy for most neonates Sano Modification of the Norwood Operation
with HLHS. Achieving survival after the Norwood operation is challenging,
involving innumerable technical and medical details. At best, after
Norwood Reconstruction a Norwood procedure, the patient is fragile, with a delicate balance
After initial work and success at Boston Childrens Hospital, between systemic and pulmonary blood flow. This fact and the
Norwood and colleagues46 gained international attention at the observation of widely disparate outcomes for the procedure have
Childrens Hospital of Philadelphia for developing and imple- led to many important advances in the treatment of these chil-
menting a reconstructive technique to palliate newborns with dren. One issue relates to the difficulty of balancing the systemic
HLHS; this methodology now carries the widely used eponym of to pulmonary artery shunt, which lowers diastolic blood pressure
the Norwood procedure. This procedure was gradually refined as (and coronary perfusion pressure) and volume loads the heart.
experience accrued. The most common method involves surgical Sano and associates47 from Okayama University in Japan were the
connection of the divided main pulmonary artery to the recon- first to report a series of infants undergoing a successful Norwood
structed aortic arch. In almost all children with HLHS, there is procedure with the modification of a right ventriclepulmonary
associated aortic arch hypoplasia with coarctation. A critical artery conduit rather than a Blalock shunt. The theoretical advan-
feature of the operation is to reconstruct the aortic arch to provide tage of this approach is the increase in diastolic pressure, creating
unrestricted systemic blood flow. Most surgeons use some form a physiology more similar to a banded circulation rather than
of prosthetic material, usually pulmonary artery homograft patch- shunted circulation. Early reports with this method were encour-
ing. Some surgeons have reported accomplishing the arch recon- aging, although patients appeared to become more rapidly desatu-
struction without the necessity of additional material. After rated as they aged compared with the shunted patients. The
reconstructing the aortic arch, the divided main pulmonary artery long-term effects of the right ventriculotomy on cardiac function
is anastomosed to the arch and small ascending aorta to create a are unknown. In one report, patients undergoing the Norwood
neoaortic confluence providing systemic output from the right operation were randomly assigned to receive a right ventricle-to-
ventricle. The challenging feature of the reconstruction involves pulmonary artery shunt or modified Blalock-Taussig shunt.

Aorta
Main
pulmonary
artery

C
A

B
FIGURE 58-41 Norwood procedure for first-stage palliation of hypoplastic left heart syndrome. A, The main
pulmonary artery is divided proximal to the bifurcation, the ductus arteriosus is ligated and divided, and the
aortic arch is opened from the level of the transected main pulmonary artery to a point distal to the ductal
insertion in the descending aorta. B, A segment of homograft is cut to an appropriate size and shape. This
is sutured into place, creating an unobstructed outflow from the right ventricle to the pulmonary artery and
aorta. C, Polytetrafluoroethylene tube graft is placed from the innominate artery to the right pulmonary artery.
The atrial septectomy is done while the patient is under circulatory arrest. (From Castaneda AR, Jonas RA,
Mayer JE, etal: Hypoplastic left heart syndrome. In Cardiac surgery of the neonate and infant, Philadelphia,
1994, Saunders.)

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CHAPTER 58 Congenital Heart Disease 1649

Transplantation-free survival was higher 12 months after random-


ization in the right ventricle-to-pulmonary artery shunt group, as
was the rate of unplanned reinterventions and complications.48
Midterm results are being analyzed and preliminarily show pos-
sible midterm survival benefit.

Hybrid Procedure
The notion of a combined therapy between interventional cardiol-
ogy and surgery for the first-stage palliation of HLHS has achieved
significant attention. The idea is to minimize the risk of the first
operation by banding the branch pulmonary arteries and deliver-
ing a stent into the ductus to maintain patency. This hybrid
arrangement is designed to allow newborn survival so that a more
complete reconstruction may be performed later in infancy in a
larger child. There appears to be a significant learning curve with
this approach, as with any new procedure, and the incidence of
complications warrants further study. Data have shown that the
prevalence of necrotizing enterocolitis after the hybrid procedure
is significant and comparable to reports after the Norwood pro-
cedure.49 In addition, concerning features include the effect of the
banding on long-term pulmonary artery growth, the fact that
cardiac perfusion is still retrograde through the aortic arch, and
the risk profile of the more extensive reconstruction later in life.
The true place for this mode of therapy is unclear at the present FIGURE 58-42 Angiogram of a dilated right atrium in a patient with an
time, but it represents an important direction of advancement to atriopulmonary Fontan connection.
optimize the opportunity of survival for these children.

Fontan Operation appendage was directly anastomosed to the pulmonary artery


The long-term goal of single-ventricle palliation is to optimize bifurcation with surgical closure of the ASD. Many of these
ventricular function and promote systemic oxygen delivery. As patients present as adults with extreme dilation of the right
noted earlier, patients with a single ventricle who are shunted or atrium, with resulting sluggish flow, hepatic congestion, and atrial
banded have ongoing concerns, including systemic desaturation, dysrhythmias (Fig. 58-42). Today, the most widely practiced
continued intracardiac mixing, and chronic cardiac volume over- modification of the Fontan operation is the total cavopulmonary
load. The current strategy for addressing these concerns uses a connection. First described by DeLeval, this operation involves
direct connection between the branch pulmonary arteries and connection of the divided SVC to the superior and inferior aspects
systemic venous return, as initially proposed by Fontan in the of the right pulmonary artery (typically offset), along with the
early 1970s.50 The Fontan operation is now the treatment of creation of a channel to direct the IVC flow into the pulmonary
choice for children born with all varieties of single ventricle and arteries. The channel may be created using a surgically created
provides acceptable long-term palliation in suitable patients. lateral tunnel in the right atrium (Fig. 58-43) or interposing a
However, the Fontan circulation is not normal and even in the conduit between the IVC and pulmonary arteries (extracardiac
best of circumstances results in significant alteration in normal Fontan) (Fig. 58-44).
cardiorespiratory physiology. The change from a volume-loaded circulation in patients with
The Fontan circulation is established by connecting the sys- a single ventricle who are shunted or banded to a Fontan circula-
temic venous return directly into isolated branch pulmonary tion results in acute volume unloading of the systemic ventricle.
arteries without an intervening power source. Thus, blood flow in In the chronic overloaded heart, this acute change may be poorly
the Fontan circuit is passive, being promoted only by the pressure tolerated, with resultant diastolic dysfunction and decreased ven-
differential between the systemic venous system and pulmonary tricular compliance. To deal with this problem, patients with a
venous atrium. An impediment to flow in the systemic-to- single ventricle typically undergo an intervening stage of palliation
pulmonary pathway results in a poor Fontan outcome. Estab- in the form of a bidirectional, superior cavopulmonary anastomo-
lished criteria for creating an effective Fontan circulation include sis (Glenn shunt). The bidirectional Glenn shunt is constructed
the ability to connect the systemic venous return surgically to the by anastomosing the cephalad end of the divided SVC to the
pulmonary arteries in an unobstructed manner, normal pulmo- superior aspect of the right pulmonary artery (Fig. 58-45). Other
nary artery architecture and resistance, normal pulmonary venous sources of pulmonary blood flow are typically eliminated, and the
drainage and low left atrial pressure, absence of significant A-V heart is volume-unloaded; however, systemic cardiac output is
valve regurgitation, good ventricular function (and low ventricular maintained because the IVC return is preserved. After the Glenn
end-diastolic pressure), an unobstructed systemic arterial outlet, shunt, the patients are not fully saturated; typically, patients have
and good aortic valve function. Compromise of any of these ele- saturations of approximately 80%. Over time, the unloaded ven-
ments may compromise the quality of the Fontan circulation. tricle remodels, and the patient is promoted to reoperation and
The Fontan operation has undergone several technical modifi- completion of the Fontan circulation.
cations in the almost 40 years of successful application to patients Perioperative care of a patient after a Fontan procedure can be
with single-ventricle physiology. Many patients underwent an challenging. The acute changes in cardiac volume loading may
atriopulmonary connection in which the open right atrial negatively affect cardiac output. Even in patients with supposedly

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1650 SECTION XI Chest

SVC

Ao
RPA
PA

Ao PA

RPA ASD
Incisions
SVC
RA
Conduit
RAA

A
IVC
A
Graft

PA B
FIGURE 58-44 A, Extracardiac Fontan procedure. B, Creation of a
Ao fenestration in an extracardiac Fontan procedure using a graft between
the extracardiac conduit and right atrial appendage (RAA). Ao, Aorta;
ASD IVC, inferior vena cava; PA, pulmonary artery; RPA, right pulmonary
artery; SVC, superior vena cava. (Adapted from Lok JM, Spevak PJ,
Nichols DG: Tricuspid atresia. In Nichols DG, Ungerleider RM,
SVC
Spevak PJ, etal, editors: Critical heart disease in infants and children,
IVC Philadelphia, 2006, Mosby, p 814.)
Baffle

positive end-expiratory pressure, impedes pulmonary blood flow


B in the Fontan patient. Conversely, early extubation and effective
FIGURE 58-43 A and B, Lateral tunnel Fontan procedure. Ao, Aorta; spontaneous ventilation will improve pulmonary blood flow in
ASD, atrial septal defect; IVC, inferior vena cava; PA, pulmonary artery; the Fontan patient. Data have suggested that early extubation in
RA, right atrium; RPA, right pulmonary artery; SVC, superior vena cava. the operating room for patients after the Fontan procedure
(Adapted from Lok JM, Spevak PJ, Nichols DG: Tricuspid atresia. In improves hemodynamics, decreases the length of stay for patients,
Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Critical heart
and decreases hospital costs.14
disease in infants and children, Philadelphia, 2006, Mosby, p 813.)
The chronic complications of living with a Fontan circulation
are still unfolding and include chronic hepatic congestion and
cirrhosis, protein-losing enteropathy, atrial dysrhythmias, and
ideal Fontan connections, the central venous pressure acutely venous stasis disease. Management of patients with failing Fontan
increases to 12 to 15mmHg. Consequences of this increased circulations is especially challenging. These patients are at high
venous pressure include pleural effusions, hepatic congestion, and risk for severe cardiac compromise while undergoing general anes-
ascites. In marginal Fontan candidates, some surgeons routinely thesia with positive pressure ventilation or any procedure involv-
place an intentional leak, or fenestration; the goal here is to pre- ing large fluid shifts, including abdominal surgery. Patients with
serve systemic ventricular volume loading and decrease systemic chronic hepatic congestion may develop a coagulopathy related
venous congestion at the expense of some degree of desaturation to a decrease in factor production.
caused by the right-to-left shunting. The practice of routine fen-
estration after the Fontan operation has been examined, and some
early data have shown that excellent outcomes can be achieved MISCELLANEOUS ANOMALIES
with highly selective application of a fenestration, which mitigates
the risks associated with such a procedure, including hypoxia and Vascular Rings and Pulmonary Artery Slings
systemic embolism.51 Any impediment to passive pulmonary Vascular Rings
blood flow will inhibit Fontan flow and result in right heart Vascular rings are abnormalities of the aortic arch and its branches,
failure. Positive pressure ventilation, especially elevated levels of compressing the trachea, esophagus, or both. The ring may be

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CHAPTER 58 Congenital Heart Disease 1651

ANTERIOR

SVC Ligamentum
arteriosum LPA MPA
LSA
Az Ao
LCC

RPA
Esophagus

PA
Trachea
Ascending
RSA aorta
RCC

RPA

A
POSTERIOR
IVC
RPA
RA
FIGURE 58-45 Bidirectional Glenn shunt. Ao, Aorta; Az, azygos vein; SVC
IVC, inferior vena cava; PA, pulmonary artery; RPA, right pulmonary RSA
artery; SVC, superior vena cava. (Adapted from Lok JM, Spevak PJ,
Nichols DG: Tricuspid atresia. In Nichols DG, Ungerleider RM, IVC
Spevak PJ, etal, editors: Critical heart disease in infants and children,
Philadelphia, 2006, Mosby, p 809.)

complete or partial. Categorization of the defects is useful for RCC


description: Trachea
Complete vascular rings
Double arch: Equal arches or left or right arch dominant Esophagus
(Fig. 58-46)
Right arch: Left ligamentum arteriosus from anomalous left
subclavian artery LCC
Right arch: Mirror image branching, with left ligamentum LSA Lig. arteriosum
from descending aorta
Partial vascular rings B
Left arch: Aberrant right subclavian artery FIGURE 58-46 Double aortic arch, posterior (A) and superior (B) views.
Left arch: Innominate artery compression IVC, inferior vena cava; LCC, Left common carotid artery; LPA, left
The double aortic arch is the most common form of complete pulmonary artery; LSA, left subclavian artery; MPA, main pulmonary
artery; RA, right atrium; RCC, right common carotid artery; RSA, right
ring. Two arches arise from the ascending aorta, forming a true
subclavian artery; SVC, superior vena cava. (Adapted from Jonas RA:
ring. The left arch is usually smaller. The right archleft ligamen- Comprehensive surgical management of congenital heart disease. New
tum complex is formed from persistence of the right fourth arch York, 2004, Oxford University Press, p 499.)
and regression of the left fourth arch. The anomalously arising left
subclavian artery is often associated with a diverticulum at its base
(Kommerell diverticulum). In partial rings, the most common of the aorta forms a vascular ring around the trachea but not the
form is an aberrant right subclavian artery arising distal to the left esophagus. The trachea may be compressed, the cartilage may be
subclavian artery with a left arch. The right subclavian artery soft, or there may be intrinsic stenosis of the trachea in the form
passes behind the esophagus from left to right. Innominate artery of complete cartilage rings.
compression arises from a more posterior and leftward origin of
the innominate artery from a left arch, leading to anterior com- Diagnosis and Indications for Intervention
pression of the trachea. Symptoms reflect the degree of tracheal and esophageal compres-
sion from complete rings as well as the presence of coexistent
Pulmonary Artery Slings tracheomalacia or stenosis. Upper respiratory symptoms predomi-
A pulmonary artery sling occurs when the left pulmonary artery nate, with a characteristic brassy cough, recurrent respiratory infec-
arises from the right pulmonary artery, passing leftward between tions, failure to thrive, and sometimes esophageal motility
the trachea and the esophagus. The ligamentum arteriosum problems. In children, documentation of a ring is an indication
attachment from the main pulmonary artery to the undersurface for surgery. Older patients are often asymptomatic. Initially, the

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1652 SECTION XI Chest

diagnosis is based on a high index of suspicion, and barium swallow Anatomy and pathophysiology. Developmentally, failure of the
is the first investigation. Echocardiography can document an normal connection of the left coronary artery bud to the aorta
abnormal head and neck vessel branching pattern, excluding intra- results in an abnormal connection to the pulmonary artery. The
cardiac abnormalities. MRI provides complete anatomic detail. abnormal origin can be situated in the main pulmonary artery or
proximal branches. Associated abnormalities are rare but impor-
Surgery tant to recognize because lowering of the pulmonary artery pres-
Most vascular rings are accessible through a left posterolateral sure by PDA ligation or closure of a VSD can be fatal if the
thoracotomy; the exception is a left arch with right-sided ligamen- ALCAPA is not noted. In utero, with equal pulmonary arterial
tum. Division of the ring and, in the case of double arch, preserva- and aortic pressures, satisfactory perfusion of the ALCAPA can
tion of the dominant arch is performed. Preservation of the occur. After birth, the pulmonary artery pressure falls, and left
recurrent laryngeal nerve is important. Initial experience with coronary artery perfusion decreases. Ischemia causes impaired
endoscopic robotically assisted repair of vascular rings has also ventricular function and myocardial infarcts and leads to left
been reported. Pulmonary artery slings are approached through ventricular dilation. Papillary muscle dysfunction causes mitral
the midline; the use of cardiopulmonary bypass facilitates tracheal regurgitation. Early coronary collateral development may prevent
reconstruction and relocation of the right pulmonary artery (Fig. ongoing infarction.
58-47). Repair can be achieved with low risk. Symptoms may take Diagnosis and indications for intervention. ALCAPA is sus-
months to resolve, with slow resolution of the underlying pected in any infant with mitral regurgitation, ventricular dysfunc-
tracheomalacia. tion, or dilated cardiomyopathy. Infants present with low cardiac
output and systemic heart failure. Feeding may also precipitate
Coronary Artery Anomalies sudden death and angina in infants. Sudden death has been
Anomalies occur as a result of anomalous origin, termination, described in older children. The ECG may reflect ischemic changes.
courses, and aneurysm formation. Of these variables, only an The echocardiogram is usually diagnostic. However, because this
anomalous left coronary artery rising from the pulmonary artery diagnosis is often confused with dilated cardiomyopathy, there is
(ALCAPA) and coronary artery fistulas are discussed here. an argument in favor of catheterizing all patients with dilated
cardiomyopathy in whom the coronary artery anatomy cannot be
Anomalous Left Coronary Artery Rising From clearly defined on echocardiography. Secondary findings of dilated
the Pulmonary Artery cardiac chambers and segmental wall motion abnormalities
An ALCAPA is a rare, often lethal lesion in early infancy. together with mitral regurgitation prompt a search for an ALCAPA.
Untreated, the mortality rate approaches 90%. Diagnosis of an ALCAPA is an indication for intervention.

Stenotic portion
excised

A C

Tracheal
anastomosis

FIGURE 58-47 Method for the management of a pulmonary artery sling with associated tracheal stenosis,
using cardiopulmonary bypass. A, Tracheal resection of the involved segment. B, Anterior translocation of
the left pulmonary artery after transection of the trachea. C, Direct anastomosis of the trachea. (From
Castaneda AR, Jonas RA, Mayer JE, etal: Vascular rings, slings, and tracheal anomalies. In Cardiac surgery
of the neonate and infant, Philadelphia, 1994, Saunders.)

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CHAPTER 58 Congenital Heart Disease 1653

vention. Interventional catheterization is useful for the oblitera-


tion of fistulas and terminal aneurysms.
Surgery. If the lesion is not amenable to transcatheter inter-
B vention, surgery is indicated. Options include suture ligation
without bypass, cardiopulmonary bypass, and aneurysmectomy
with closure of the fistula. Early and late mortality rates are low.
PA Risk factors for death and ventricular dysfunction relate to coro-
nary artery insufficiency and infarction after fistula ligation or
AO C aneurysmectomy.53

Ebstein Anomaly of the Tricuspid Valve


A Ebstein anomaly of the tricuspid valve is a rare defect in which
the tricuspid valve attachments are displaced into the right ventricle
to varying degrees. Ebstein anomaly includes a spectrum of abnor-
malities involving a degree of displacement of the tricuspid valve,
variable right ventricular size, and variable pulmonary outflow
FIGURE 58-48 Direct reimplantation of anomalous left coronary artery obstruction. Associated abnormalities are ASD, pulmonary atresia,
rising from the pulmonary artery (ALCAPA). A, Excision of ALCAPA and ccTGA. The posterior and septal leaflets of the tricuspid valve
from the pulmonary artery (PA). B, Aortic reimplantation of the coronary are variably displaced to the apex of the right ventricle, which
ostium into the aorta. C, Reconstruction of the PA with autologous results in an atrialized portion of the right ventricle. The anterior
pericardium. AO, Aorta. (From Vouhe PR, Tamisier D, Sidi D, etal: leaflet remains large and sail-like. The major hemodynamic issue
Anomalous left coronary artery from the pulmonary artery: Results of
is tricuspid incompetence with decreased pulmonary blood flow
isolated aortic reimplantation. Ann Thorac Surg 54:621626, 1992.)
and, if an ASD is present, right-to-left shunting causing cyanosis.
Long-standing tricuspid incompetence leads to volume overload
of an abnormal right ventricle. Variable pulmonary outflow tract
Surgery. A degree of ventricular dysfunction is usually present. obstruction limits effective pulmonary blood flow. If adequate
Preoperative inotropic support and optimization of hemodynam- pulmonary blood flow requires continued ductal patency, the need
ics may be required before surgical intervention. Severe cardiomy- for neonatal intervention is almost certain.
opathy rarely may necessitate cardiac transplantation. Current
experience indicates that creation of a dual coronary system is safe Diagnosis and Intervention
and reproducible and offers the best opportunity for recovery of The more severe forms of Ebstein anomaly manifest with cyanosis
function.52 Operative considerations include optimal myocardial in infancy. Critically ill neonates tend to have a severe form of the
protection and prevention of left heart distention. Direct reim- disease, with a grossly inefficient right ventricle compounded by
plantation of the ALCAPA into the ascending aorta is the proce- the high pulmonary resistance of the neonate or by pulmonary
dure of choice (Fig. 58-48). Sometimes, limited mobility of the valve atresia. The mortality rate in this group is high. Older
coronary artery precludes reimplantation, and a surgically created patients present in heart failure and may have cyanosis. Supraven-
aortapulmonary arterycoronary artery tunnel is created; this is tricular dysrhythmias and the pre-excitation syndrome (Wolff-
known as the Takeuchi procedure. Ligation of the ALCAPA is not Parkinson-White syndrome) are associated with Ebstein anomaly.
recommended. Echocardiography is diagnostic. Critically ill neonates have poor
Postoperative management is directed toward maintaining survival rates, and surgery is indicated only after stabilization with
adequate coronary perfusion and cardiac output. Mechanical PGE1 and controlled ventilation. In older patients, cyanosis and
support of the heart may be temporarily required. Mitral regur- heart failure are indications to intervene, although earlier inter-
gitation usually improves, and valve replacement is rarely neces- vention in asymptomatic patients, before excessive right ventricu-
sary. Current intervention has a low operative mortality. Risks for lar dilation, is being more actively pursued.
nonsurvival relate to preoperative ventricular dysfunction and
cardiogenic shock. The Takeuchi repair is associated with tunnel Surgery
complications such as obstruction, leak, aortic valve damage, and In critically ill neonates, after stabilization, palliation with a
RVOTO in the long-term. systemic-to-pulmonary artery shunt may be required. The Starnes
operation has allowed salvage in previously hopeless cases. This
Coronary Arteriovenous Fistula and Aneurysms operation consists of patch closure of the tricuspid orifice, atrial
Isolated coronary artery fistula is more rare than ALCAPA. Drain- septectomy, and a systemic-to-pulmonary artery shunt.54 In
age of coronary artery fistula is reported to terminate more com- patients with less severe forms of this disease, tricuspid valve repair
monly in the right side of the heart or pulmonary artery than in or replacement is also an option. Surgical techniques for the treat-
the left side of the heart. A shunt from the high-pressure coronary ment of Ebstein anomaly have been evolving, and outcomes are
artery system into a low-pressure cardiac chamber may result in improving for this challenging group of patients (Fig. 58-49).55
coronary steal and some degree of cardiac volume overload. Coro-
nary artery aneurysms are associated with Kawasaki disease. Mitral Valve Anomalies
Diagnosis and indications for intervention. Presentation Most abnormalities of the mitral valve are associated with other
depends on the amount of functional compromise produced by complex lesions (e.g., Shone complex). More commonly, mitral
the ischemia and volume overload. Echocardiography may be able disease in children is inflammatory in naturethat is, rheumatic
to delineate the anomaly, but coronary angiography is diagnostic. disease or infective endocarditis. It may also be associated with
Details of coronary anatomy are essential for determining inter- collagen vascular disease and Marfan syndrome.

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1654 SECTION XI Chest

Anterior leaflet reader is directed to several excellent texts on CHD for a more
thorough review of each of the lesions reviewed in this chapter.

SELECTED REFERENCES
Bailey LL, Nehlsen-Cannarella SL, Doroshow RW, etal: Cardiac
allotransplantation in newborns as therapy for hypoplastic left
heart syndrome. N Engl J Med 315:949951, 1986.

This classic reference describes the first report of cardiac


transplantation in newborns with hypoplastic left heart syn-
drome (HLHS). Although limited in its applicability because
A Plication of atrialized chamber of limited donor organs, neonatal cardiac transplantation
B has provided children born with HLHS a new option for
survival.
FIGURE 58-49 Repair of Ebstein malformation using the Carpentier
method. A, The anterior and posterior leaflets of the tricuspid valve are
detached from the annulus. B, The atrium is plicated, reducing the
Blalock A, Taussig HB: The surgical treatment of malformations
annular diameter. The detached leaflets are reattached to the annulus. of the heart in which there is pulmonary stenosis or pulmonary
(From Castaneda AR, Jonas RA, Mayer JE, etal: Ebsteins anomaly. atresia. JAMA 128:189202, 1945.
In Cardiac surgery of the neonate and infant, Philadelphia, 1994,
Saunders.) This landmark article describes the surgical procedure that
initiated the era of elective cardiac surgery. The study
reported the initial experience with palliative surgical treat-
Mitral Stenosis ment of patients with pulmonary stenosis or pulmonary
atresia using the Blalock-Taussig shunt.
Mitral stenosis is caused by obstruction at a supravalvular, valvu-
lar, or subvalvular level, singly or in combination. Supravalvular
Fontan F, Baudet E: Surgical repair of tricuspid atresia. Thorax
stenosis is caused by a ring of fibrous tissue above the annulus of
26:240248, 1971.
the mitral valve or attached to the proximal leaflets. Valvular
stenosis involves the leaflets, with commissural fusion occurring
This article represents a milestone in the evolution of surgi-
with or without hypoplasia of the valve ring. Hypoplasia of the
cal management of patients with single-ventricle physiology.
mitral valve is often associated with left ventricular hypoplasia.
It described the first corrective operation for patients with
Frequently, the leaflets and subvalvular apparatus are also dysplas-
tricuspid atresia. Although previous palliative procedures,
tic. Fusion of the leaflets can lead to an accessory orifice and
provided by various systemic-to-pulmonary artery shunts,
produce mitral stenosis at a purely valvular level (so-called double-
improved the clinical condition of patients, systemic blood
orifice mitral valve). Three types of subvalvular stenosis have been
was still a mixture of oxygenated and deoxygenated blood.
recognizedparachute mitral valve, hammock valve, and absence
The Fontan operation redirected superior and inferior vena
of one or both papillary muscles. Mitral regurgitation is a result
cava blood flow to the lungs so that only oxygenated blood
of secondary annular dilation, congenital isolated clefts of the
returned to the heart and subsequently to the systemic
valve, and prolapse of the leaflets from abnormal chordae or papil-
circulation.
lary muscle insertion.
Echocardiography is diagnostic. Intervention includes balloon
Kirklin JW, Dushane JW, Patrick RT, etal: Intracardiac surgery
valvuloplasty, particularly for selected forms of rheumatic mitral
with the aid of a mechanical pump-oxygenator system (gibbon
stenosis, and surgical intervention. Intervention is timed to avoid
type): Report of eight cases. Mayo Clin Proc 30:201206,
irreversible sequelae related to chronic volume overload or pul-
1955.
monary hypertension. Surgical intervention is aimed at preserving
the mitral valve, and valvuloplasty techniques have a valuable
This landmark article demonstrated that open repairs of con-
place in children. Prosthetic valves are the least desirable option.
genital cardiac defects using mechanical pump oxygenator
Bioprosthetic or tissue valves need to be avoided in children.
systems could be performed with minimal risk to patients.
Supra-annular placement of the prosthesis may be necessary.
Repeat placement is ensured.
Mustard W: Successful two-stage correction of transposition of
the great vessels. Surgery 55:469472, 1964.

SUMMARY This classic reference describes one of the initial surgical


approaches to the treatment of transposition of the great
This chapter provides a basic overview of the major congenital
arteries (D-TGA). Although the arterial switch operation is
cardiac lesions and a framework for the diagnosis and treatment
now the surgical treatment of choice for D-TGA, there are
of these conditions. For most patients, the diagnosis of CHD,
many adult patients with congenital heart disease who have
whether surgically treated or not, carries lifelong implications. For
been palliated with the Mustard operation. Understanding
patients with CHD presenting for noncardiac surgery, a thorough
the operation and resulting physiology is critical to general
understanding of the patients unique anatomy and physiology is
surgery management strategies for noncardiac operations.
mandatory when planning a rational management strategy. The

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CHAPTER 58 Congenital Heart Disease 1655

Nichols DG, Ungerleider RM, Spevak PJ, etal, editors: Critical


documented the successful use of cross-circulation to
heart disease in infants and children, ed 2, Philadelphia, 2006,
correct defects such as ventricular septal defect.
Mosby.
Wilcox B, Cook A, Anderson R: Surgical anatomy of the heart,
This text provides a comprehensive and current review of
ed 3, Cambridge, England, 2004, Cambridge University Press.
heart disease in infants and children. It contains numerous
surgical drawings and diagnostic images to supplement the
This text provides an excellent reference manual for under-
didactic material.
standing the complex anatomy of the heart. It contains color
photographs and diagrams and is an invaluable resource for
Norwood WI, Lang P, Casteneda AR, etal: Experience with any student of cardiac surgery.
operations for hypoplastic left heart syndrome. J Thorac Cardio-
vasc Surg 82:511519, 1981.

In this landmark article, Norwood and colleagues reported REFERENCES


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option for survival of patients with HLHS was cardiac trans- pulmonary atresia. JAMA 128:189202, 1945.
plantation. At most centers today, the Norwood operation is 2. Warden HE, Cohen M, Read RC, etal: Controlled cross
the primary mode of therapy for most neonates with HLHS. circulation for open intracardiac surgery: Physiologic studies
and results of creation and closure of ventricular septal
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drome. J Thorac Cardiovasc Surg 126:504509, 2003. 3. Kirklin JW, Dushane JW, Patrick RT, etal: Intracardiac
surgery with the aid of a mechanical pump-oxygenator system
This classic reference describes the right ventricleto (gibbon type): Report of eight cases. Proc Staff Meet Mayo
pulmonary artery conduit used in the Norwood procedure. Clin 30:201206, 1955.
This novel procedure, named after the author, Sano, allowed 4. Marelli A, Gilboa S, Devine O, etal: Estimating the congeni-
for more hemodynamic stability postoperatively from the tal heart disease population in the United States in 2010
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Surgery 45:966980, 1959. lence of congenital heart disease in the general population
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This classic reference describes the first report of a new
(Accessed August 6, 2015).
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11. Wilcox BR, Cook AC, Anderson RH: Surgical anatomy of the
cedure was named after the surgeon, Starnes. This approach
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CHAPTER 58 Congenital Heart Disease 1657

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