Congenital Heart Disease in the Adult

Presenting for Noncardiac Surgery

Ilka D. Theruvath, MD, PhD and
Scott T. Reeves, MD, MBA, FACC
Department of Anesthesia and Perioperative Medicine
Medical University of South Carolina
Charleston, South Carolina

has led to a large and growing population of adults

Learning Objectives:
currently living with ACHD. The exact number is not
As a result of completing this activity, the participant
entirely clear, but estimates suggest that it is most likely
will be able to
more than one million today.2 That means that for the first
 Describe the most common congenital heart
time in history, adults with congenital heart disease
lesions encountered in patients who reach adult-
outnumber children with congenital heart defects. As the
hood
life expectancy of individuals with ACHD increases, rising
 Discuss the anesthetic implications of long-term
numbers of these patients will present with noncardiac
altered physiology in adult congenital heart disease
disease or traumatic injury requiring noncardiac surgery.
 Describe major difficulties in anesthetic manage-
Consensus guidelines and expert opinions suggest that
ment of adult congenital heart disease patients
adults with ACHD undergoing noncardiac surgery can
undergoing noncardiac surgery
experience increased perioperative morbidity and mortal-
Author Disclosure Information: ity, and the recent results of a large retrospective study
Dr. Theruvath and Dr. Reeves have disclosed that emphasize how vulnerable this population is in the
they have no financial interests in or significant perioperative period.3
relationship with any commercial companies per-
taining to this educational activity. Consensus guidelines and expert opinions
suggest that adults with ACHD undergoing
he prevalence of adult congenital heart disease

T (ACHD) in the United States has increased dramati-

cally over the last 30 years. Improvements in medical
as well as surgical management have resulted in the
survival to adulthood of almost 90% of U.S. children born
with congenital heart defects.1 This triumph of survival
Supplemental Digital Content is available for this article. Direct URL
citations appear in the printed text and are available in both the HTML
and PDF versions of this article. Links to the digital files are provided in
the HTML and PDF text of this article on the Journal’s Web site
(www.asa-refresher.com).
112
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
noncardiac surgery can experience increased
perioperative morbidity and mortality.
In this chapter, the anatomy and implications of ACHD
are reviewed for three illustrative cyanotic congenital heart
defects: tetralogy of Fallot (TOF), transposition of the
great arteries (TGA), and univentricular heart/single ven-
tricle. These three lesions allow us to follow a historical
timeline from the first surgical palliative procedure—the
Blalock–Taussig (BT) shunt for a TOF patient in the 1940s,
soon followed by primary surgical repair of TOF in the
Congenital Heart Disease in the Adult 113

The Potts and Waterston shunts were introduced as pos-

sible alternatives to the classic BT shunt. Potts connected the
descending aorta to the left PA, and Waterston, the ascending
aorta to the right PA. These shunts do not involve vessels as
small as the subclavian artery, thus resulting in a lesser like-
lihood of occlusion and thrombosis, but both techniques
were later abandoned for several reasons: preferential blood
flow to one lung, kinking and distortion of the pulmonary
arteries, but most of all, the high incidence of excessive
pulmonary blood flow and subsequent pulmonary hyper-
Figure 1. Historical timeline of surgical palliation/repair for tetralogy of tension. The presence of pulmonary hypertension, therefore,
Fallot (TOF), transposition of the great arteries (TGA), and single-ventricle needs to be assessed in every adult TOF patient with a current
lesions.
or previous Potts or Waterston shunt.
The subsequently modified BT shunt, which was in-
1950s—to the palliative atrial transposition procedures for
troduced in 1962, involves a synthetic graft to create a side-
TGA (Senning and Mustard) that were eventually replaced
to-side anastomosis between the subclavian artery and the
by the arterial switch (Jatene) operation in the early 1980s,
corresponding right or left PA. Advantages of the modified
up to the evolution of surgical palliation for single-ven-
BT shunt include preservation of blood flow to the ipsilateral
tricle lesions such as the Fontan operation from the 1970s
arm, less distortion of the pulmonary arteries, and more
until the current era (Figure 1). The implication of this
controlled blood flow in the pulmonary circulation accord-
timeline is that there are TOF patients now presenting for
ing to the size chosen for the graft. Although overall numbers
surgery in their 70s, TGA patients who had a Senning or
are declining with increasing surgical achievements in early
Mustard operation are in their fifth or sixth decade, and
primary repairs, it is still one of the most commonly used
patients with arterial switch operation are found surviving
shunt procedures in the United States today,4 followed by the
to their 30s, whereas adults with single-ventricle lesions
RV to PA (Sano) shunt, which has gained popularity in the
are often not older than their 20s to 30s.
recent past.5 Both shunt procedures are mainly used for pal-
liation before complete repair of a cyanotic lesion, as an ad-
junct to the staged palliation of a single-ventricle lesion, and
TETRALOGY OF FALLOT in patients with inadequate pulmonary blood flow who are
TOF is the most common cyanotic cardiac defect with the otherwise not amenable to repair.
longest congenital cardiac surgical history and the greatest The surgical repair of TOF started to become possible in
documentation of outcomes. It accounts for 10% of all the 1950s and includes patch closure of the ventricular
congenital heart diseases. Its main anatomic abnormalities septal defect, opening of the right ventricular outflow
are narrowing of the right ventricular outflow tract, which tract, and, in the case of a small pulmonary annulus,
is the primary determinant of symptoms and severity, to- placement of a transannular patch. There is a current trend
gether with a ventricular septal defect, right ventricular
(RV) hypertrophy, and an aorta that straddles the ven-
tricular septum (overriding aorta) (Figure 2). This anomaly
encompasses a morphologic spectrum: the right ven-
tricular outflow tract obstruction frequently occurs at
multiple levels, and the pulmonary valve itself can range
from moderately small to completely absent in the extreme
form of TOF with pulmonary atresia.
The first surgical shunt to treat cyanotic TOF was
conceptualized by Blalock and Taussig in 1945. The classic
BT shunt involves ligation and division of the left sub-
clavian artery and an end-to-side anastomosis to the
pulmonary artery (PA). The BT shunt was initially a great
success as it increased pulmonary blood flow, improved
oxygenation, and relieved the cyanosis. Disadvantages of
the technique include frequent shunt thrombosis and oc-
clusion, owing to the small size of the vessels used, and
distortion of the subclavian artery with the potential for
abnormal growth of the affected arm. Pulses can be di- Figure 2. Classic anatomic features of tetralogy of Fallot. From Motta P,
minished or even be absent in the affected extremity, which Miller–Hance WC, Transesophageal Echocardiography for Congenital Heart
Disease in the Adult, A Practical Approach to TEE, 3rd edition. Edited by
has to be considered as a potential problem when placing Perrino AC, Reeves ST. Philadelphia, Wolters Kluwer Health/Lippincott
an arterial line in a patient with a previous BT shunt. Williams & Wilkins, 2014. With permission.

Copyright r 2015 American Society of Anesthesiologists. All rights reserved.

114
toward earlier primary repair in infancy without per-
forming one of the previously mentioned palliative shunts
as a first stage, but most adults with TOF likely had a shunt
procedure first and then the repair later in childhood, or
even adulthood. If a transannular patch is used, it is gen-
erally very effective in relieving the obstruction, but it
distorts the pulmonary valve apparatus, and free pulmo-
nary regurgitation inevitably results, which leads to vol-
ume loading and dilatation of the RV. For this reason, TOF
is a classic example of a lesion that should be considered
‘‘repaired, but not cured’’ (Supplemental Digital Content 1,
http://links.lww.com/ASA/A525). Despite spectacular im-
provement of the initial surgical results, there is growing
awareness that an appreciable percentage of patients with
repaired TOF show significant problems later in life. The
majority of problems relate to abnormal RV loading: both
pressure load, if there is residual obstruction of the right
ventricular outflow tract, and volume load in the case of
free pulmonary regurgitation after relief of the ob-
struction. Both pressure and volume loading of the RV are
poorly tolerated over time. Progressive RV dysfunction
along with development of dysrhythmias increases the risk
for sudden death in these patients.6
The electrophysiological and hemodynamic substrates
of sudden cardiac death, sustained ventricular tachycardia,
and pulmonary regurgitation are the main underlying he-
modynamic lesions. Preservation or restoration of pulmo-
nary valve function may thus be critical for long-term
outcome in these patients (Supplemental Digital Content
2, http://links.lww.com/ASA/A526). Conventional surgi-
cal strategy for pulmonary valve replacement involves
undergoing repeat open-heart surgery. Recent advances
allow for percutaneous pulmonary valve implantation in
select patients in the cardiac catheterization laboratory
using a bovine valve inside a balloon-expandable stent7
(Figure 3). Early evidence indicates that this procedure
Figure 3. Angiogram of a percutaneously placed pulmonary valve within a
stent seated in the right ventricular outflow tract, showing valvular
competence. Courtesy of R. Bandisode, MD, Pediatric Cardiology, Medical
University of South Carolina, Charleston, South Carolina.
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
Theruvath and Reeves
Figure 4. Surgical placement of a stented pulmonary valve into a conduit in
the right ventricular outflow tract. Courtesy of S. Bradley, MD, Pediatric
Cardiac Surgery, Medical University of South Carolina, Charleston, South
Carolina.
offers a promising alternative to the conventional surgical
strategy, but there are still unanswered questions about the
long-term durability of this valve. The stent seen on a pa-
tient’s chest roentgenogram can certainly cause confusion
when such a patient is coming for an urgent operation and
is uncertain about the details of his history. Challenges for
percutaneous placement arise from complicated anatomy
and severe conduit stenosis. In such cases, surgical place-
ment of such a valve has also been successfully performed
(Figure 4).
TRANSPOSITION OF THE GREAT ARTERIES
Simple (or D-) TGA is characterized by the right atrium
connected to the RV and the left atrium entering the left
ventricle (LV), thereby maintaining atrioventricular con-
cordance, but the RV gives rise to the aorta and the LV to
the PA, causing ventriculoarterial discordance (Figure 5).
D-Transposition accounts for 4% of all cases of congenital
heart disease. In D-TGA, the systemic and pulmonary cir-
culations function in parallel rather than in series, leading
to cyanosis in the neonatal period. Mixing of the parallel
circuits at the atrial, ventricular, or great artery level is
essential for survival.
The surgical management of this lesion has evolved
dramatically over the years. From the 1960s to 1980s,
TGA was palliated with atrial transposition operations,
that is, the Mustard or Senning procedure, that redirected
the blood flow via a baffle placed inside the atria so that
deoxygenated blood from the venae cavae is directed into
the LV and then the PA, and oxygenated pulmonary venous
blood is directed to the morphologic RV and then to the
aorta. In the Senning procedure, the atrial baffle is created
from autologous tissue; the alternative Mustard operation
uses synthetic material to create the baffle. Most TGA
patients more than 25 years old will have undergone one of
these atrial switch procedures, which provide an excellent
clinical result mid-term, but in the long term are associated
Congenital Heart Disease in the Adult 115

Figure 5. Transposition of the great arteries (left) and arterial switch (Jatene) procedure (right). In this operation, the aorta and pulmonary arteries are
transected and anastomosed to their appropriate respective ventricular outflows. The coronary arteries are translocated to the systemic outflow tract. From
Motta P, Miller–Hance WC, Transesophageal Echocardiography for Congenital Heart Disease in the Adult, A Practical Approach to TEE, 3rd edition. Edited by
Perrino AC, Reeves ST. Philadelphia, Wolters Kluwer Health/Lippincott Williams & Wilkins, 2014. With permission.

with significant sequelae. Long-term problems arise from
the fact that a morphologic RV must function as the sys-
temic ventricle.8 Although these patients may compensate
well for decades, the RV function eventually deteriorates,
often leading to heart failure with concomitant tricuspid
regurgitation. In addition, the presence of multiple atrial
suture lines aids the development of atrial arrhythmias.9
Sinus node dysfunction is also a problem in adult
Senning/Mustard patients, probably the result of damage
to the sinus node or interruption of sinus node blood flow
at the time of operation. Because these patients are so
particularly vulnerable to sinus node disease, overzealous
use of perioperative beta-blockers may precipitate
more profound conduction disturbances and may cause
complete heart block. Scarring and narrowing or leaks of
the atrial baffle are infrequent late complications, but
should always be investigated. Obstruction of the superior
vena cava (SVC) is more common than that of the inferior
vena cava (IVC) and may produce ‘‘SVC syndrome.’’
Pulmonary hypertension is a serious complication of atrial
switch repairs. The exact cause is not clear, but patients
appear more likely to develop pulmonary vascular disease
when undergoing the operation beyond 2 years of age and
having had a ventricular septal defect before the repair.
Pulmonary hypertension may also be caused by pulmonary
venous baffle obstruction, which should always be ex-
cluded.
Since the mid-1980s, TGA has usually been repaired in
infancy with an arterial switch procedure (Jatene oper-
ation). It involves transection of the great arteries above
the sinuses and detachment of the coronary arteries along
with a ‘‘button’’ from the aortic wall. The great arteries are
then switched into their new position, with the PA brought
forward anterior to the aorta and the coronary buttons
sutured into the ‘‘neoaorta.’’ The atrial septal opening is
closed and the patent ductus arteriosus ligated. This oper-
ation restores the normal anatomic arrangement of the
circulation so that the LV functions as the systemic ven-
tricle (Figure 5). Physiologically, it is a more attractive
long-term option and has steadily become the procedure of
choice. Over the last 25 years, the initially high perioper-
ative mortality has dropped, and the clinical outcome is
good. Long-term morbidity is dominated by distortion and
regurgitation of the neoaortic valve (which is the anatomic
pulmonary valve) and coronary ostial lesions. Late coro-
nary artery stenoses are rare, but have serious potential to
cause perioperative lethal arrhythmias, myocardial in-
farction, and sudden death. Interestingly, the stenoses are
often asymptomatic for the patient in daily life and are
only discovered by coronary angiography.10
UNIVENTRICULAR HEART/SINGLE VENTRICLE
Patients with one of the various forms of single-ventricle
anatomy and physiology are among the most complex
cases of congenital heart disease now seen in significant
numbers in adulthood. They are the early survivors of in-
novative surgical shunt and conduit techniques developed
a generation ago. Long-term survival was not seen until the
introduction of the Fontan operation in 1971, a palliative
approach in which the systemic venous return is rerouted
to bypass the right heart completely. Initially used for pa-
tients with tricuspid atresia, the concept has now been
applied to congenital lesions of all types in which the nor-
mal two-ventricle circulation cannot be restored. The high
neonatal pulmonary vascular resistance (PVR) precludes
definitive repair of single ventricle in the newborn. Thus, a
staged surgical approach has been introduced to transition
the infant to the end-goal of Fontan physiology.

Copyright r 2015 American Society of Anesthesiologists. All rights reserved.

116
Patients with one of the various forms of
single-ventricle anatomy and physiology
are among the most complex cases of
congenital heart disease now seen in
significant numbers in adulthood.
In the newborn period, a lesion-specific initial palliation
is performed, such as the stage 1 Norwood procedure11 for
a hypoplastic LV. Regardless of the initial anatomy, this
first stage will leave the patient with a typical physiological
profile:
(1) Limited, but reliable source of pulmonary blood flow,
for example, modified BT shunt or RV to PA (Sano)
shunt.12
(2) Unobstructed outflow tract to the systemic arterial
circulation, such as the creation of a ‘‘neoaorta’’ that is
reconstructed using the original main PA. After
dividing the main PA at the level of the bifurcation, a
side-to-side amalgamation is created between the main
PA and the hypoplastic aortic arch (with its coronary
arteries). The areas of hypoplasia and coarctation of
the transverse aorta are augmented with a prosthetic
patch. This creates an unobstructed single arterial
trunk that leaves the heart.
(3) Complete mixing of systemic and pulmonary venous
return. To achieve this, the atrial septum is usually
excised.
The second stage of surgical treatment for single-ven-
tricle lesions is the bidirectional Glenn shunt. The bidirec-
tional Glenn shunt consists of an end-to-side anastomosis
between the SVC, which is divided from the right atrium,
and the right PA. The systemic venous blood coming from
the SVC is shunted into the right and left PA. Because ad-
equate flow through the shunt depends on low PVR, this
procedure is typically performed between 3 and 9 months
of life, after PVR falls from relatively high physiological
neonatal levels. A bidirectional Glenn shunt results in half
of the systemic venous return going directly to the lungs.
The other half—deoxygenated IVC flow from the lower
body—still returns to the heart. Because blood from the
IVC continues to mix with pulmonary venous return in the
single ventricle, the patient remains desaturated. However,
oxygenation of the systemic venous return is more efficient
than when mixed arterial blood is delivered to the PA, as is
the case with the first-stage BT shunt. The fraction of
‘‘blue’’ blood in the ventricle is smaller, which results in
higher systemic oxygen saturations after the Glenn shunt
(85 to 90%) than after the initial palliation. Patients can
live with a Glenn shunt for a long time, but eventually a
long-term complication develops, that is, pulmonary arte-
riovenous fistula. This malformation, which results in
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
Theruvath and Reeves
ineffective gas exchange, is thought to result from the lack
of hepatic venous blood perfusing the lungs. Humoral
factors in hepatic venous blood, which are unknown so far,
seem necessary to prevent formation of these fistulous
communications.
The Fontan procedure is the third and final stage of sur-
gery for single ventricle. Since its initial description, at least
10 different variations have been performed, but in all its
forms, the Fontan bypasses the RV completely, leading to
passive, nonpulsatile flow from both IVC and SVC to the PA.
The complete Fontan procedure usually follows the bidirec-
tional Glenn shunt at 2 to 4 years of age. The Glenn shunt is
left intact to direct SVC blood to the lungs, and either a lat-
eral tunnel inside the right atrium or an extracardiac conduit
is added to direct blood from the IVC to the PA. With an
extracardiac conduit Fontan, the IVC is separated from the
right atrium and a prosthetic tube graft is sewn in from the
IVC to the right PA beside the right atrium, rather than
within it. With either procedure, a small opening or fenes-
tration may be created between the Fontan conduit and the
atrium. It acts as a ‘‘pop-off,’’ opening at times of sudden
increases in PVR such as coughing, etc., which during such
an event leads to desaturation, but helps to maintain cardiac
output. As a consequence, though, this opening represents a
communication between the venous and arterial circulations,
and all intravenous lines must be meticulously de-aired to
reduce the chance of systemic air embolism in patients with a
fenestrated Fontan. After a patient has established a steady
Fontan circulation with continuous adequately low pulmo-
nary pressures, this fenestration is often closed, most com-
monly in the cardiac catheterization laboratory.
All Fontan techniques result in routing of the entire
systemic venous return to the pulmonary arteries, but the
flow is passive and laminar through the lungs without the
help of a ventricle pump and creation of pulsatility.13 It is
critical to maintain low resistance in this pathway, which is
driven by the transpulmonary gradient. Optimal Fontan
physiology therefore depends on many factors that an an-
esthesiologist can potentially influence: adequate preload;
low intrathoracic pressures (weighing the effects of pos-
itive-pressure ventilation vs. negative-pressure sponta-
neous respiration); low PVR with avoidance of hypoxia,
hypercapnia, and atelectasis; good ventricular function
with maintenance of sinus rhythm; judicious use of myocar-
dial depressants such as anesthetic agents; and avoidance of
large increases in afterload.14 As all blood flow is supposed
to pass through the lungs, patients with a Fontan should
maintain arterial oxygen saturations above 90%, and sat-
urations below this level should be considered abnormal
and warrant further investigation.
ANESTHESIA CONSIDERATIONS
Preoperative Assessment
Preoperative assessment of ACHD patients is similar to
that of any other surgical patient with special attention
Congenital Heart Disease in the Adult
directed toward cardiorespiratory function and exercise
tolerance (Supplemental Digital Content 3, http://links.
lww.com/ASA/A527). Examination should aim to de-
termine the presence of congestive heart failure, cyanosis,
and the possible presence of pulmonary hypertension.
Signs of RV failure may include jugular venous distention,
hepatomegaly, and peripheral edema. Special attention
should be paid to renal, hepatic, and hematological tests,
particularly the presence of secondary erythrocytosis due
to chronic hypoxia. A 12-lead electrocardiogram is useful
to detect dysrhythmias, which are a common occurrence in
ACHD patients. Patients with previous surgery near the
conduction system may have varying degrees of heart block
and may be dependent on a permanent pacemaker, which
needs to be interrogated and properly programmed pre-
operatively. Preoperative diagnostic evaluation, possibly in-
cluding cardiac catheterization, may be needed to optimize
some ACHD patients before major noncardiac procedures.
Sedative premedication can be useful to blunt sympathetic-
induced stress responses and pulmonary vasoconstric-
tion, but care must be taken to avoid hypercapnia and
hypoxia, which in cases of cyanotic heart disease or pulmo-
nary hypertension can have profound deleterious effects on
PVR, resulting in RV failure.
Intraoperative Management
All standard anesthetic induction agents have been used
safely in ACHD patients. Of greater importance than the
specific choice of agent is the speed and overall dose ad-
ministered (Supplemental Digital Content 4, http://links.
lww.com/ASA/A528). Many of these patients have slow
circulation times, and consequently there is a risk of over-
dose if the induction drug is administered rapidly. Main-
tenance with oxygen–air mixture and an inhalational
agent is acceptable, as is total intravenous anesthesia. It is
rarely necessary or beneficial to ventilate with a very high
fraction of inspired oxygen. The initiation of positive-
pressure ventilation with high intrathoracic pressures im-
peding venous return can be deleterious, particularly in
patients with ventricular dysfunction and increased PVR.
All standard anesthetic induction agents have
been used safely in ACHD patients. Of greater
importance than the specific choice of agent
is the speed and overall dose administered.
Monitoring should include standard electro-
cardiography, oxygen saturation, core temperature, and
ventilator parameters with particular attention to end-tidal
carbon dioxide and inspired oxygen concentrations. Ad-
ditional monitoring is dictated by the extent of the planned
surgery and the existing cardiac abnormality. Most pa-
tients will have invasive arterial pressure monitoring, but
many will not require central venous measurements. The
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
117
risk of central venous puncture must be weighed against
the possible benefit, particularly in patients with single-
ventricle circulation, in whom thrombotic and embolic
events are more common. PA catheters are rarely used
because the interpretation can be misleading, and calcu-
lation of cardiac output by thermodilution may be in-
accurate in the presence of significant shunting. In all
ACHD patients, careful attention must be paid during line
insertion to prevent air emboli, which can be disastrous in
patients with intracardiac defects. Transesophageal echo-
cardiography can be an invaluable additional monitor,
with real-time interpretation allowing rapid responses to
changing hemodynamic conditions.
Every effort should be made to avoid perioperative hy-
pothermia/shivering with the associated increase in myocar-
dial workload and oxygen demand. Glucose control can
significantly improve morbidity and mortality in adult pa-
tients undergoing cardiac surgery for acquired disease, but
controversy persists whether these findings can be ex-
trapolated to ACHD patients and, if so, exactly how tight
the glucose control should be.15 Regional anesthesia has also
been safely performed in certain ACHD patients with special
attention being paid to coagulation status and the use of
commonly prescribed anticoagulant medications.
The overall goal is to choose the anesthetic agents and
techniques that will have the least impact on the patient’s
cardiovascular system, and it is imperative to be prepared
for decompensation so as to intervene promptly.
The overall goal is to choose the anesthetic
agents and techniques that will have the
least impact on the patient’s
cardiovascular system.
Postoperative Considerations
Postoperative ventilation is not without risk because of its
effect on venous return. Therefore, early extubation is de-
sirable, but not at the risk of hypoventilation, hypoxia, or
hypercapnia (Supplemental Digital Content 5, http://links.
lww.com/ASA/A529). Avoiding hypercapnia is especially
crucial in patients with pulmonary hypertension so as to
avoid any increase in PVR. Achieving adequate post-
operative analgesia can be challenging, but is important
because of the negative effects of painful stress responses
on pulmonary vascular tone and overall hemodynamics in
this vulnerable patient population. ACHD patients are at
increased risk for certain serious perioperative complica-
tions (e.g., pulmonary hypertensive crisis, thromboem-
bolic events; Supplemental Digital Content 6, http://
links.lww.com/ASA/A530) that can occur in the days after
surgery, and ongoing careful monitoring may be needed.
Therefore, only a minority of ACHD patients may be
candidates for ambulatory day surgery.
118
SPECIAL CHALLENGES
Pulmonary Hypertension
Pulmonary hypertension is a marker of severity of the as-
sociated ACHD and is associated with higher mortality
rates.16 In patients with known pulmonary hypertension,
preoperative care should be established by a specialized
physician and all targeted therapies should be continued
throughout the perioperative period, when pulmonary
hypertensive crises are more common. Every effort should
be made to avoid iatrogenic rises in PVR, which could
occur because of sympathetic stimulation (light anesthesia,
pain), hypoxia, hypercapnia, acidosis, hypothermia, or
increased intrathoracic pressures/excessive positive end-
expiratory pressure. In severe pulmonary hypertension,
standard ventilator maneuvers may be of limited value and
may worsen the hemodynamic status. A useful ventilation
strategy may be moderate hyperventilation with a shorter
inspiratory and longer expiratory time and relatively high
fractional inspired oxygen concentrations.
Pulmonary hypertensive crisis is a medical emergency
with a very high mortality rate. If suspected, rapid treat-
ment must be initiated to achieve survival. Removal of
possible precipitating factors is essential, and ventilation
with 100% oxygen should be instituted. Adequate depth
of anesthesia and immediate pain control are imperative.
Rapid pharmacological treatment options for reversible
pulmonary hypertension include inhaled prostaglandins or
nitric oxide. RV failure in this setting may require inodila-
tors such as milrinone or dobutamine. The last resort
remains intraaortic balloon counterpulsation with or
without placement of an RV assist device. Despite these
maneuvers, the death rate remains high.
Chronic severe pulmonary hypertension in the context of
shunting lesions may result in Eisenmenger syndrome. The
chronic exposure of the pulmonary vascular bed to increased
blood flow and pressure results in pulmonary obstructive dis-
ease. As the pulmonary vascular bed becomes obliterated, the
PVR increases until it equals or exceeds systemic vascular re-
sistance, which consequently reverses the shunting of blood
from right to left and results in chronic severe hypoxemia.
Morbidity in these individuals relates to problems associated
with chronic cyanosis and erythrocytosis. They may suffer
from thromboembolic events, cerebrovascular insults, and
hyperviscosity syndrome. Patients with Eisenmenger physiol-
ogy are at highest risk for perioperative mortality, and
noncardiac surgery should be performed only if absolutely
essential. Once the pulmonary/systemic resistance ratio ex-
ceeds 0.7, the risk of surgical procedures becomes prohibitive.
Dysrhythmias
Dysrhythmias represent one of the most common reasons for
hospital admission among ACHD patients. Any dysrhyth-
mia may occur, but the most common are supraventricular
tachycardias, especially in patients after atrial surgery or
with atrial distention. The most common form of tachy-
dysrhythmia observed is intraatrial reentrant tachycar-
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
Theruvath and Reeves
dia originating from the right atrium. Loss of sinus rhythm
can cause serious hemodynamic disturbance in patients with
poor cardiac reserve. Often these dysrhythmias are re-
fractory to pharmacological treatment and require urgent
cardioversion under general anesthesia. If transesophageal
echocardiography is to be performed to exclude thrombus,
intubation may be necessary. Profound hemodynamic com-
promise can occur on induction and with the initiation of
positive-pressure ventilation, especially in patients with a
univentricular heart. A unique aspect in patients with com-
plex ACHD, such as Fontan patients, is the likely inability to
float an intravenous pacing device because of the absence of
direct venous access to the ventricles. Therefore, external
pacing must be immediately available.
SUMMARY
As adults with congenital heart disease are surviving longer
than ever before, it is becoming increasingly apparent that
even relatively simple lesions can be associated with long-
term complications. Most of these patients have received at
least one, if not more, palliative procedures with surgical
shunts or conduits. The cardiovascular anatomy and
physiology in ACHD is complex and has a multitude of
anesthetic implications. Understanding the lesion-specific
and repair-specific implications and long-term outcomes is
key to providing optimal perioperative care. Even com-
plete anatomic correction or repair does not equal cure,
and perioperative risk is increased in these patients.
Serious sequelae that substantially increase perioper-
ative risk are:
(1) Heart failure (e.g., failure of the systemic RV in
Mustard/Senning patients or the single ventricle in
Fontan patients).
(2) Dysrhythmias.
(3) Abnormal pulmonary vasoreactivity and pulmonary
hypertension.
Patients with these conditions should probably receive care
in a specialized ACHD center with multidisciplinary
collaboration among anesthesiologists, cardiologists, sur-
geons, and intensive care teams.
REFERENCES
1. Williams RG, Pearson GD, Barst RJ, et al.: National Heart, Lung, and
Blood Institute Working Group on Research in Adult Congenital
Heart Disease: Report of the National Heart, Lung, and Blood
Institute Working Group on research in adult congenital heart
disease. J Am Coll Cardiol 2006; 47:701–7.
2. Warnes CA, Liberthson R, Danielson GK, et al.: Task force 1: The
challenging profile of congenital heart disease in adult life. J Am Coll
Cardiol 2001; 37:1170–5.
3. Maxwell BG, Wong JK, Kin C, Lobato RL: Perioperative outcomes of
major noncardiac surgery in adults with congenital heart disease.
Anesthesiology 2013; 119:762–9.
4. Williams JA, Bansal AK, Kim BJ, et al.: Two thousand Blalock-
Taussig shunts: A six-decade experience. Ann Thorac Surg 2007;
84:2070–5.
Congenital Heart Disease in the Adult
5. Sharma V, Deo SV, Huebner M, Dearani JA, Burkhart HM: In search of
the ideal pulmonary blood source for the Norwood procedure: A meta-
analysis and systematic review. Ann Thorac Surg 2014; 98:142–51.
6. Gatzoulis MA, Balaji S, Webber SA, et al.: Risk factors for arrhythmia
and sudden cardiac death late after repair of tetralogy of Fallot: A
multicentre study. Lancet 2000; 356:975–81.
7. Bonhoeffer P, Boudjemline Y, Saliba Z, et al.: Percutaneous replace-
ment of pulmonary valve in a right-ventricle to pulmonary-artery
prosthetic conduit with valve dysfunction. Lancet 2000; 356:1403–5.
8. Warnes CA: Transposition of the great arteries. Circulation 2006;
114:2699–709.
9. Wilson NJ, Clarkson PM, Barratt-Boyes BG, et al.: Long-term outcome
after the Mustard repair for simple transposition of the great arteries: 28-
year follow-up. J Am Coll Cardiol 1998; 32:758–65.
10. Hutter PA, Kreb DL, Mantel SF, et al.: Twenty-five years’ experience
with the arterial switch operation. J Thorac Cardiovasc Surg 2002;
124:790–7.
Copyright r 2015 American Society of Anesthesiologists. All rights reserved.
119
11. Norwood WI, Lang P, Hansen DD: Physiologic repair of aortic atresia–
hypoplastic left heart syndrome. N Engl J Med 1983; 308:23–6.
12. Ohye RG, Sleeper LA, Mahony L, et al.: Comparison of shunt types
in the Norwood procedure for single-ventricle lesions. N Engl J Med
2010; 362:1980–92.
13. Sommer RJ, Hijazi ZM, Rhodes JF: Pathophysiology of congenital
heart disease in the adult: Part III: Complex congenital heart disease.
Circulation 2008; 117:1340–50.
14. Cannesson M, Earing MG, Collange V, Kersten JR: Anesthesia for
noncardiac surgery in adults with congenital heart disease. Anesthesi-
ology 2009; 111:432–40.
15. Roy N: Critical care management of the adult patient with congenital
heart disease: Focus on postoperative management and outcome.
Curr Treat Options Cardiovasc Med 2015; 17:362.
16. Diller GP, Dimopoulos K, Okonko D, et al.: Exercise intolerance in
adult congenital heart disease: Comparative severity, correlates, and
prognostic implication. Circulation 2005; 112:828–35.