Professional Documents
Culture Documents
2
Cardiac Diseases
ALEXANDER MITTNACHT, MD; DAVID L. REICH, MD n
AMANDA J. RHEE, MD; JOEL A. KAPLAN, MD n
28
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Chapter 2 Cardiac Diseases 29
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30 ANESTHESIA AND UNCOMMON DISEASES
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Chapter 2 Cardiac Diseases 31
Percutaneous transluminal alcohol septal ablation is per- volatile anesthetics and nitroglycerin, or p ostural changes.
formed in the catheterization laboratory but requires special Ventricular contractility can be increased by hemodynamic
expertise that is limited to experienced centers.22 Although responses to tracheal intubation or surgical stimulation.
this may be efficacious for subsets of patients with obstructive Transmural distending pressure can be decreased by hypoten-
HCM, the procedural complication rate may exceed that of sion secondary to anesthetic drugs, hypovolemia, or positive-
surgical myectomy.23 Ablation is also associated with the risk pressure ventilation. Additionally, patients with obstructive
of serious adverse events, such as alcohol toxicity and malig- HCM do not tolerate increases in heart rate. Tachycardia
nant tachyarrhythmias.24 A relatively new alternative to induce decreases end-diastolic ventricular volume, resulting in a
septal ablation involves percutaneous transluminal septal coil narrowed LVOT. As noted earlier, the atrial contraction is
embolization, which avoids the problem of alcohol toxicity. extremely important to the hypertrophied ventricle. Nodal
Further experience and outcome data are required before this rhythms should be aggressively treated, using atrial pacing if
new technique is considered a standard treatment modal- necessary.
ity for HCM.25 Although still controversial, evidence suggests Halothane, now a historical drug and no longer available
that atrioventricular sequential (DDD) pacing is beneficial for in the United States, had major hemodynamic advantages for
patients with obstructive HCM.26,27 the anesthetic management of patients with obstructive HCM.
Its advantages were to decrease heart rate and myocardial
ANESTHETIC CONSIDERATIONS contractility. Of the inhalational anesthetics, halothane had
The determinants of the functional severity of the ventricular the least effect on systemic vascular resistance (SVR), which
obstruction in obstructive HCM are (1) the systolic volume of tended to minimize the severity of the obstruction when vol-
the ventricle, (2) the force of ventricular contraction, and (3) ume replacement was adequate. Sevoflurane decreases SVR to
the transmural pressure distending the LVOT. a lesser extent than isoflurane or enflurane and thus may be
Large systolic volumes in the ventricle distend the LVOT preferable. Agents that release histamine, such as morphine,
and reduce the obstruction, whereas small systolic volumes thiopental, and atracurium, are not recommended because
narrow the LVOT and increase the obstruction. When ven- of the resulting venodilation. Agents with sympathomimetic
tricular contractility is high, the LVOT is narrowed, increasing side effects (ketamine, desflurane) are not recommended
the obstruction. When aortic pressure is high, the increased because of the possible tachycardia. High-dose opioid anes-
transmural pressure distends the LVOT. During periods of thesia causes minimal cardiovascular side effects along
decreased afterload and hypotension, however, the LVOT is with bradycardia and thus may be useful in these patients.
narrowed, resulting in greatly impaired cardiac output often Preoperative β-blocker and calcium channel blocker therapy
associated with significant mitral regurgitation. As the ven- should be continued. Intravenous (IV) propranolol, esmolol,
tricle hypertrophies, ventricular compliance decreases, and or verapamil may be administered intraoperatively to improve
passive filling of the ventricle during diastole is impaired. The hemodynamic performance. Table 2-2 summarizes the anes-
ventricle becomes increasingly dependent on the presence of thetic and circulatory management of obstructive HCM.28
atrial contraction to maintain an adequate ventricular end-
diastolic volume. Monitoring should be established that allows
continuous assessment of these parameters, particularly in
patients in whom the obstruction is severe.
In patients with symptomatic obstructive HCM present- TABLE 2-2 n Treatment Principles of Hypertrophic
ing for surgery, an indwelling arterial catheter for beat-to- Obstructive Cardiomyopathy
beat observation of ventricular ejection and continuous blood
Clinical Relatively
pressure (BP) monitoring should be placed before anesthesia Problem Treatment Contraindicated
induction. Transesophageal echocardiography (TEE) provides
↓ Preload Volume Vasodilators
useful data on ventricular function and filling, the severity of
Phenylephrine Spinal/epidural
LVOT obstruction, and the occurrence of SAM and mitral anesthesia
regurgitation. A pulmonary artery catheter (PAC), once more
widely used, has not shown to improve outcome. Its use may ↑ Heart Rate β-Adrenergic Ketamine
blockers β-Adrenergic agonists
be helpful in guiding treatment options, especially in patients Verapamil
with obstructive HCM undergoing major surgery with large
fluid shifts. ↑ Contractility Halothane Positive inotropes
Sevoflurane Light anesthesia
Special consideration should be given to those features of
β-Blockers
the surgical procedure and anesthetic drugs that can p roduce Disopyramide
changes in intravascular volume, ventricular contractility, and
transmural distending pressure of the outflow tract. Decreased ↓ Afterload Phenylephrine Isoflurane
Spinal/epidural
preload, for example, can result from blood loss, sympathec- anesthesia
tomy secondary to spinal or epidural anesthesia, use of potent
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32 ANESTHESIA AND UNCOMMON DISEASES
Anesthesia for management of labor and delivery in the even in patients with a history of successful anesthesia.35,36
parturient with obstructive HCM is quite complex. “Bearing The uncommon nature of the disease makes it difficult to
down” (Valsalva maneuver) during delivery may worsen make specific recommendations for anesthetic management.
LVOT obstruction. Beta-blocker therapy may have been If the condition is known, invasive continuous arterial BP
discontinued during pregnancy because of the association monitoring is prudent intraoperatively. A PAC should prob-
with fetal bradycardia and intrauterine growth retardation. ably be avoided, given the tendency toward arrhythmias.
Oxytocin must be used carefully because of its vasodilating Propofol and etomidate appear to be safe induction agents.34
properties and compensatory tachycardia. Pulmonary edema Neuromuscular blocking agents such as vecuronium, cisa-
has been observed in parturients with HCM, emphasizing the tracurium, and rocuronium are probably safe as well. AICDs
need for careful fluid management.29 Spinal anesthesia is rela- should be managed according to the guidelines published and
tively contraindicated because of the associated vasodilation, referred to throughout this text,37 regardless of the presence of
but epidural anesthesia has been used successfully.30 General ARVC/D.38
anesthesia is preferred by many practitioners. If hypoten-
sion occurs during anesthesia, the use of beta-agonists such
Left Ventricular Noncompaction
as ephedrine may result in worsening outflow tract obstruc-
tion, and alpha-agonists such as phenylephrine, once thought Left ventricular (LV) noncompaction of ventricular myocar-
to result in uterine vasoconstriction, are now preferred.31,32 dium is a genetic disorder with familial and nonfamilial types.
However, careful titration of anesthetic agents and adequate LV noncompaction has a distinctive “spongy” appearance
volume loading (most often guided by invasive monitoring) is to the LV myocardium, with deep intertrabecular recesses
essential to safely conducted anesthesia in this clinical setting. (sinusoids) that communicate with the LV cavity. LV non-
compaction results in LV systolic dysfunction, heart failure,
thromboemboli, arrhythmias, sudden death, and ventricular
Arrhythmogenic Right Ventricular
remodeling.1
Cardiomyopathy/Dysplasia
Arrhythmogenic right ventricular cardiomyopathy/dysplasia ANESTHETIC CONSIDERATIONS
(ARVC/D) is an uncommon (estimated 1:5000 young adults), Anesthetic management in patients with LV noncompaction
newly described, autosomal dominant disease with incomplete depends on the severity of ventricular dysfunction, which should
penetrance. ARVC/D is frequently associated with myocardi- be evaluated preoperatively. In patients with impaired cardiac
tis but is not considered a primary inflammatory cardiomy- function, management should be directed toward preserving
opathy. It involves predominantly the right ventricle initially, contractility and baseline levels of preload and afterload. Up to
progressing to affect the left ventricle in later stages. There is 80% of patients with LV noncompaction have a neuromuscular
a progressive loss of myocytes, with replacement by fatty or disorder, such as Duchenne's or Becker's muscular dystrophy or
fibrofatty tissue, which leads to regional (segmental) or global myotonic dystrophy.39 Thus, depolarizing neuromuscular junc-
pathology. It is three times more common in women. tion blockers should be avoided or used with caution.40 Patients
The clinical presentation of ARVC/D usually includes ven- may be receiving anticoagulation for thromboembolic prophy-
tricular tachyarrhythmias, such as monomorphic ventricular laxis and may therefore have contraindications for using neur-
tachycardia, syncope, or cardiac arrest, with global or segmen- axial techniques. AICDs are often inserted for indications such
tal chamber dilation and regional wall motion abnormalities. as arrhythmias or heart failure, and patients should be managed
It has been recognized as an important cause of sudden death accordingly.41,42
in young athletes.33 Diagnosis involves assessment of multiple Data are limited regarding anesthetic management in
facets of cardiac physiology, including electrical, functional, patients with LV noncompaction syndrome. In a retrospec-
and anatomic pathology. tive study on 60 patients with noncompaction undergoing 220
procedures, only patients undergoing general anesthesia expe-
ANESTHETIC CONSIDERATIONS rienced complications, compared to regional anesthesia or
The main therapeutic options are similar to those for other sedation/analgesia.43 Because the nature of the surgery often
arrhythmia-prone or heart failure patients. Patients often pres- dictates the need for general anesthesia, patients with non-
ent with AICDs, and antiarrhythmic agents such as β-blockers compaction syndrome requiring general anesthetics warrant
or amiodarone may be helpful should arrhythmias occur.34 vigilant monitoring in the perioperative period.
Catheter ablation of diseased areas of myocardium (acting as
arrhythmogenic foci) can be useful in cases of refractory med-
Conduction System Disease
ical therapy. Cardiac transplantation is also an option as a final
alternative. LENÈGRE'S DISEASE
As a rarer heart disease, minimal evidence exists for the Progressive cardiac conduction defect, also known as Lenègre's
optimal anesthetic management of patients with ARVC/D. disease, has an autosomal dominant pattern of inheritance
It is one of the main causes of sudden, unexpected periopera- resulting in ion channelopathies, which manifest as con-
tive death, which can occur in low-risk surgical candidates, duction abnormalities. Lenègre's disease involves primary
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Chapter 2 Cardiac Diseases 33
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34 ANESTHESIA AND UNCOMMON DISEASES
CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR elevations in response to stimuli. Nitrous oxide (N2O) causes
TACHYCARDIA mild sympathetic stimulation and thus should be avoided.
Catecholaminergic polymorphic ventricular tachycardia Medications that can further prolong the QT interval should
(CPVT) has two patterns of inheritance that lead to ventric- probably be avoided, including isoflurane, sevoflurane,77
ular tachycardia triggered by vigorous physical exertion or thiopental, succinylcholine, neostigmine, atropine, glycopyr-
acute emotion, usually in children and adolescents. This can rolate, metoclopramide, 5HT3 receptor antagonists, and
lead to syncope and sudden death. The resting ECG is unre- d
roperidol.78 Ketamine is generally not recommended as an
markable, with the exception of sinus bradycardia and promi- induction agent in patients with congenital long QT s yndrome.
nent U waves in some cases. The most common arrhythmia Despite the QT-prolonging effect, thiopental has been used
seen in CPVT is a bidirectional ventricular tachycardia with without adverse consequences. Propofol has no effect on or
an alternating QRS axis.1,63,71 may actually shorten the QT interval and is theoretically a
good choice of induction agent.79 Anxiolysis with midazolam
SHORT QT SYNDROME has been used successfully.80
Short QT syndrome is characterized by a short QT interval In patients with Brugada's syndrome, sodium channel
(QTc <330 msec) and ECG appearance of tall, peaked T waves. blockers such as procainamide and flecainide are contraindi-
It is associated with polymorphic ventricular tachycardia and cated, and medications such as neostigmine, class 1A antiar-
ventricular fibrillation.1,72,73 rhythmic drugs, and selective α-adrenoreceptor agonists may
increase ST segment elevation and should also be avoided.
IDIOPATHIC VENTRICULAR FIBRILLATION Thiopental, isoflurane, sevoflurane, N2O, morphine, fentanyl,
The literature describes a group of cardiomyopathies desig- ketamine, and succinylcholine have been used successfully.81–83
nated as idiopathic ventricular fibrillation. Data are insuffi- Some report arrhythmias related to propofol administration. In
cient, however, to establish this as a distinct cardiomyopathy. contrast to patients with long QT syndrome, propofol should
It is likely the summation of multiple etiologies that lead to be used with caution in patients with Brugada's syndrome.84,85
arrhythmias, probably caused by ion channel mutations.1,74
ANESTHETIC CONSIDERATIONS
Dilated Cardiomyopathy
Patients with ion channelopathies may present intraoperatively Dilated cardiomyopathy (DCM) has both genetically derived
or in the postanesthesia care unit with sudden arrhythmias that and acquired components, as well as inflammatory and non-
warrant vigilant ECG monitoring. Continuous invasive intra- inflammatory forms.86 It is a relatively common cause of heart
arterial BP monitoring should be considered in patients with failure, with a prevalence of 36 per 100,000 people,87 and is a
a history of frequent arrhythmias. Patients should be treated common indication for heart transplantation. DCM is char-
as any patient prone to arrhythmias; this includes avoiding acterized by ventricular chamber enlargement and systolic
arrhythmogenic medications and immediate availability of a dysfunction with normal left ventricular wall thickness. From
cardioverter-defibrillator device. Few data are available on anes- 20% to 35% of DCM is familial, with predominantly autosomal
thetic recommendations for these cardiomyopathies. Patients dominant inheritance, but also X-linked autosomal recessive
with long QT syndrome will occasionally present for high and mitochondrial patterns.88 The main features of DCM are
left thoracic sympathectomy and left stellate ganglionectomy, left ventricular dilation, systolic dysfunction, myocyte death,
although most patients with these ion channelopathies will most and myocardial fibrosis.89 Evidence indicates genetic simi-
likely present for surgery unrelated to their primary disorder. larities between hypertrophic and dilated cardiomyopathy.90
Patients with long QT syndrome seem to be at increased risk Nonfamilial causes for DCM include infectious agents, par-
of arrhythmia during periods of enhanced sympathetic activ- ticularly viruses that lead to inflammatory myocarditis, and
ity, particularly during emergence from general anesthesia with toxic, degenerative, and infiltrative myocardial processes.91,92
use of potent volatile agents, and when neuromuscular blocker Although there are different systems for classifying DCM, this
reversal drugs were given with ondansetron in children.75 Beta section discusses inflammatory and noninflammatory forms.
blockade has been described as the most successful medical
management of patients with congenital long QT syndrome INFLAMMATORY CARDIOMYOPATHY (MYOCARDITIS)
types I and II, which affect potassium channels. Beta blockade There are a wide variety of toxins and drugs that cause inflam-
is contraindicated in type III, which involves sodium channels.76 matory myocarditis (Table 2-3). Infectious myocarditis typi-
In patients who receive β-blockers, it is reasonable to continue cally evolves through several stages of active infection, through
beta blockade perioperatively. Intraoperatively and particularly healing, and may ultimately culminate in DCM.
during long procedures, supplemental IV doses of a β-blocker Myocarditis presents with the clinical picture of fatigue,
or a continuous infusion of esmolol should be considered. dyspnea, and palpitations, usually in the first weeks of the
The anesthetic technique should be tailored to mini- infection, progressing to overt congestive heart failure
mize sympathetic stimulation. A balanced anesthetic tech- (CHF) with cardiac dilation, tachycardia, pulsus alternans,
nique with adequate opioid administration is appropriate for and pulmonary edema. Between 10% and 33% of patients
this purpose, and is effective at suppressing catecholamine with infectious heart diseases will have ECG evidence of
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Chapter 2 Cardiac Diseases 35
BACTERIAL Arrhythmias
ST–T-wave changes
Diphtherial Endotoxin competitive analog of Conduction system, especially BBB; rare Temporary pacing
cytochrome B valvular endocarditis often required
Syphilis
VIRAL
Continued
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36 ANESTHESIA AND UNCOMMON DISEASES
Rubella
Rubeola
Rabies
Varicella
Lymphocytic
Choriomeningitis
Psittacosis
Viral encephalitis
Cytomegalovirus
Variola
Herpes zoster
Blastomycosis
PROTOZOAL
Balantidiasis
Filariasis
*Inflammatory type usually associated with myofibrillar degeneration, inflammatory cell infiltration, and edema.
BBB, Bundle branch block; DIC, disseminated intravascular coagulation; ECG, electrocardiogram; CHF, congestive heart failure; A-V, atrioventricular.
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Chapter 2 Cardiac Diseases 37
Syphilis, leptospirosis, and Lyme disease represent three exam- NONINFLAMMATORY DILATED CARDIOMYOPATHY
ples of myocardial infection by spirochetes.97 Tertiary syphilis The noninflammatory variety of dilated cardiomyopathy also
is associated with multiple problems, including arrhythmias, presents as myocardial failure, but in this case caused by idio-
conduction disturbances, and CHF. Lyme disease myocarditis pathic, toxic, degenerative, or infiltrative processes in the
usually presents with conduction abnormalities, such as bra- myocardium108,109 (Table 2-4).
dycardia and A-V nodal block.98 As an example of the toxic cardiomyopathy type, alcoholic
Viral infections manifest primarily with ECG abnormali- cardiomyopathy is a typical hypokinetic noninflammatory car-
ties, including PR prolongation, QT prolongation, ST-segment diomyopathy associated with tachycardia and premature ven-
and T-wave abnormalities, and arrhythmias. However, each tricular contractions that progress to left ventricular failure
viral disease produces slightly different ECG changes, with with incompetent mitral and tricuspid valves. This cardiomy-
complete heart block being the most significant. Most of opathy probably results from a direct toxic effect of ethanol
the viral diseases have the potential to progress to CHF if or its metabolite acetaldehyde, which releases and depletes
the viral infection is severe.99 Recent advances in molecular cardiac norepinephrine.110 Alcohol may also affect excitation-
biologic techniques have allowed for more accurate identifi- contraction coupling at the subcellular level.111 In chronic alco-
cation of viruses. Previously, coxsackievirus B was the most holic patients, acute ingestion of ethanol produces decreases
common virus identified as producing severe viral heart dis- in contractility, elevations in ventricular end-diastolic pres-
ease. Currently, the most prevalent viral genomes detected are sure, increases in SVR and systemic hypertension.112–115
enterovirus, adenovirus, and parvovirus B19.100–102 Although Alcoholic cardiomyopathy is classified into three hemody-
the pathogenic role of enterovirus in myocarditis and chronic namic stages. In stage I, cardiac output, ventricular pressures,
DCM is well established, whether parvovirus B19 is inciden- and left ventricular end-diastolic volume (LVEDV) are nor-
tal or pathogenic in viral myocarditis is still unclear. Epstein- mal, but the ejection fraction (EF) is decreased. In stage II, car-
Barr virus (EBV) and human herpesvirus 6 (HHV-6) have also diac output is normal, although filling pressures and LVEDV
been implicated in viral myocarditis. The presence of parvovi- are increased, and EF is decreased. In stage III, cardiac out-
rus B19, EBV, and HHV-6 is associated with a decline in car- put is decreased, filling pressures and LVEDV are increased,
diac function within 6 months. and EF is severely depressed. Most noninflammatory forms of
Subsequently, there may be an autoimmune phase in DCM undergo a similar progression.
which the degree of the cardiac inflammatory response cor- Doxorubicin (Adriamycin) is an antibiotic with broad-
relates with a worse prognosis, which may culminate in spectrum antineoplastic activities. Its clinical effectiveness,
DCM.103 The 2009 H1N1 pandemic influenza strain was however, is limited by its cardiotoxicity. Doxorubicin produces
associated with myocarditis as well. In one study, patients dose-related DCM. Doxorubicin may disrupt myocardial
with H1N1 influenza associated with myocarditis were pre- mitochondrial calcium homeostasis. Patients treated with this
dominantly female, young (mean age 33.2 years), and had drug must undergo serial evaluations of left ventricular systolic
morbidity/mortality of 27%.104,105 function.116,117 Dexrazoxane, a free-radical scavenger, may pro-
Mycotic myocarditis has protean manifestations that tect the heart from doxorubicin-associated damage.118
depend on the extent of mycotic infiltration of the myocar-
dium and may present as CHF, pericarditis, ECG abnormali- PATHOPHYSIOLOGY
ties, or valvular obstruction. The key hemodynamic features of the DCMs are elevated
Of the protozoal forms of myocarditis, Chagas’ disease, or filling pressures, failure of myocardial contractile strength,
trypanosomiasis, is the most significant, and the most com- and a marked inverse relationship between afterload and
mon cause of chronic CHF in South America. ECG changes stroke volume. Both the inherited and the nonfamilial
of right bundle branch block and arrhythmias occur in 80% forms of inflammatory and noninflammatory DCMs pres-
of patients. In addition to the typical inflammatory changes ent a picture identical to that of CHF produced by severe
in the myocardium that produce chronic CHF, a direct neu- coronary artery disease (CAD). In some conditions the pro-
rotoxin from the infecting organism, Trypanosoma cruzi, pro- cess that has produced the cardiomyopathy also involves the
duces degeneration of the conduction system, often causing coronary arteries. The pathophysiologic considerations are
severe ventricular arrhythmias and heart block with syncope. familiar. As the ventricular muscle weakens, the ventricle
The onset of atrial fibrillation in these patients is often an omi- dilates to take advantage of the increased force of contrac-
nous prognostic sign.106,107 tion that results from increasing myocardial fiber length. As
Helminthic myocardial involvement may produce CHF, but the ventricular radius increases, however, ventricular wall
more frequently symptoms are secondary to infestation and tension rises, increasing both the oxygen consumption of
obstruction of the coronary or pulmonary arteries by egg, lar- the myocardium and the total internal work of the muscle.
val, or adult forms of the worm. Trichinosis, for example, pro- As the myocardium deteriorates further, the cardiac output
duces a myocarditis secondary to an inflammatory response falls, with a compensatory increase in sympathetic activity
to larvae in the myocardium, even though the larvae them- to maintain organ perfusion and cardiac output. One fea-
selves disappear from the myocardium after the second week ture of the failing myocardium is the loss of its ability to
of infestation. maintain stroke volume in the face of increased a fterload.
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38 ANESTHESIA AND UNCOMMON DISEASES
NUTRITIONAL DISORDERS
METABOLIC DISORDERS
Hunter's syndrome Same as for Hurler's Similar to but milder than Hurler's
HEMATOLOGIC DISEASES
NEUROLOGIC DISEASE
Duchenne's muscular Muscle fiber degeneration with Conduction defects possibly 50% incidence of cardiac
dystrophy fatty and fibrous replacement secondary to small-vessel CAD involvement
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Chapter 2 Cardiac Diseases 39
Beer drinker's Probably from addition of cobalt Cyanosis Acute onset and rapid course
cardiomyopathy sulfate to beer, with myofibrillar
dystrophy and edema
Rejection cardiomyopathy Lymphocytic infiltration and Arrhythmias and conduction After heart transplantation
general rejection phenomena abnormalities
Rheumatoid arthritis Rheumatoid nodular invasion Mitral and aortic regurgitation; CAD
From coronary arteritis Constrictive pericarditis
Continued
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40 ANESTHESIA AND UNCOMMON DISEASES
Postpartum
cardiomyopathy
NEOPLASTIC DISEASES
*Also called nevus elasticus; Grönblad-Strandberg syndrome (and angioid retinal streaks).
A-V, Atrioventricular; CAD, coronary artery disease; CNS, central nervous system; HOCM, hypertrophic obstructive cardiomyopathy; SVR, systemic vascular resistance.
Normal
of mitral regurgitation is the mechanism of the improvement.
Afterload reduction also decreases left ventricular filling
pressure, which relieves pulmonary congestion and should
Moderate ventricular
dysfunction
preserve coronary perfusion pressure.120
The clinical picture of the DCM falls into the two famil-
iar categories of forward and backward failure. The features
Stroke volume
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Chapter 2 Cardiac Diseases 41
anesthesia and surgery should have central venous access procedures, a regional anesthetic technique is a reasonable
for monitoring and vasoactive drug administration. The use alternative, provided filling pressures are carefully controlled
of a PAC is much more controversial. The American Society and the hemodynamic effects of the anesthetic are monitored.
of Anesthesiologists (ASA) Task Force on Pulmonary Artery A recent study suggests that thoracic epidural used as a ther-
Catheterization has published practice guidelines.121,122 The apeutic strategy in addition to medical therapy in patients
indication for PAC placement depends on a combination of with DCM may improve cardiac function and reduce hospi-
patient-, surgery-, and practice setting–related factors. Patients tal readmission and mortality.134 One problem is that regional
with severely decreased cardiac function from DCM have sig- anesthesia is frequently contraindicated because patients with
nificant cardiovascular disease and are considered at increased cardiomyopathies are frequently treated with anticoagulant and
or high risk. With no evidenced-based medicine to support antiplatelet drugs to prevent embolization of mural thrombi
outcome differences, recommendations for PAC monitoring that develop on hypokinetic ventricular wall segments.
were based on expert opinion at that time. Patients with DCM In planning anesthetic management for the patient with
presenting for surgery who have an overall increased or high- DCM, associated cardiovascular conditions, such as the pres-
risk score should probably have hemodynamic parameters ence of CAD, valvular abnormalities, LVOT obstruction, and
monitored with a PAC. In addition to measuring right- and constrictive pericarditis should also be considered. Patients
left-sided filling pressures, a thermodilution PAC may be used with CHF often require circulatory support intraoperatively
to obtain cardiac output and calculate SVR and pulmonary and postoperatively. Inotropic drugs such as dopamine and
vascular resistance (PVR), which allow for serial evaluation of dobutamine are effective in low output states and produce
the patient's hemodynamic status. PACs with fiberoptic oxim- modest changes in SVR at lower dosages. In severe ventric-
etry, rapid-response thermistor catheters that calculate right ular failure, more potent drugs such as epinephrine may be
ventricular EF, and pacing PAC are available. Pacing PAC and required. Phosphodiesterase-III inhibitors, such as milrinone,
external pacemakers provide distinct advantages in manag- with inotropic and vasodilating properties, may improve
ing the patient with myocarditis and associated heart block. hemodynamic performance. As previously noted, stroke vol-
Recent evidence seems to provide further support for clini- ume is inversely related to afterload in the failing ventricle,
cians who choose not to use PAC monitoring on the basis of and reduction of left ventricular afterload with vasodilating
no outcome differences between high-risk surgical patients drugs such as nicardipine, nitroprusside, and nesiritide are
who were cared for with and without PAC monitoring and also effective in increasing cardiac output.
goal-directed therapy.123–125 In patients with myocarditis, especially of the viral variety,
Transesophageal echocardiography provides useful data transvenous or external pacing may be required should heart
on ventricular filling, ventricular function, severity of mitral block occur. Intra-aortic balloon counterpulsation, left ven-
regurgitation, and response of the impaired ventricle to anes- tricular assist devices, and cardiac transplantation are further
thetic and surgical manipulations. Recent guidelines indicate options to be considered in the case of the severely compro-
that hemodynamic decompensation is a class I indication for mised ventricle. Incidence of supraventricular and ventric-
TEE monitoring.126–128 With the increased availability of equip- ular arrhythmias increases in myocarditis and DCM.135,136
ment and trained anesthesiologists, TEE will become increas- These arrhythmias often require extensive electrophysiologic
ingly important in the perioperative management of patients workup and may be unresponsive to maximal medical ther-
with cardiomyopathies. apy. Frequently, patients with DCM present for AICD implan-
The avoidance of myocardial depression still remains tation or ventricular arrhythmia ablation procedures.137
the goal of anesthetic management for patients with DCM,
although, paradoxically, beta-adrenergic blockade has been
Restrictive Cardiomyopathies
associated with improved hemodynamics and improved sur-
vival in patients with DCM.129–133 (This may result from an Primary restrictive nonhypertrophied cardiomyopathy is a
antiarrhythmic effect.) All the potent volatile anesthetic agents rare form of heart muscle disease and heart failure charac-
are myocardial depressants, and therefore high concentrations terized by biatrial enlargement, normal or decreased volume
of these agents are probably best avoided in these patients. of both ventricles, normal left ventricular wall thickness and
Low doses are usually well tolerated, however, and frequently A-V valves, and impaired ventricular filling with restrictive
used as part of a balanced anesthetic. pathophysiology. Restrictive (or restrictive/obliterative) car-
For the patient with severely compromised myocardial diomyopathies are usually the end stage of myocarditis or an
function, the synthetic piperidine narcotics (fentanyl, infiltrative myocardial process (amyloidosis, hemochromato-
sufentanil, remifentanil) are useful because myocardial con- sis, scleroderma, eosinophilic heart disease) or the result of
tractility is not depressed. Bradycardia associated with high- radiation treatment138 (Table 2-5). New evidence suggests that
dose narcotic anesthesia may be prevented by the use of restrictive cardiomyopathy is genetic in origin, with mutations
pancuronium for muscle relaxation, anticholinergic drugs, or in sarcomeric contractile protein genes.139,140
pacing. Pancuronium, however, should be avoided in patients Restrictive cardiomyopathy may share characteristics with
with impaired renal function, a common problem in cardio- constrictive pericarditis. Cardiac output is maintained in the
myopathy patients. For peripheral or lower abdominal s urgical early stages by elevated filling pressures and an increased
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42 ANESTHESIA AND UNCOMMON DISEASES
Amyloidosis
heart rate. However, in contrast to constrictive pericarditis, an should be established in patients with advanced disease. A PAC
increase in myocardial contractility to maintain cardiac out- offers the advantage of cardiac output measurement and the
put is usually not possible.141 Thromboembolic complications assessment of loading conditions, both of which may be helpful
are common and may be the initial presentation. Advanced in guiding anesthetic management, even though outcome data
states can lead to elevated jugular venous pressure, peripheral has not been established for this particular group of patients.
edema, liver enlargement, ascites, and pulmonary congestion. When inducing anesthesia, it may be prudent to avoid
Also, whereas constrictive pericarditis is usually curable surgi- medications that produce bradycardia, decreased venous
cally, restrictive cardiomyopathy requires medical therapy and return, and myocardial depression. Etomidate can be used
in some patients, valvular repair or cardiac transplantation. for anesthesia induction with little impact on hemodynamics
Imaging techniques such as echocardiographic evaluation and myocardial function.143 Ketamine, even though intrinsic
with speckle-track imaging, velocity vector imaging combined cardiodepressive properties have been described, maintains
with computed tomography (CT), and cardiac magnetic reso- SVR and is frequently used in these patients. Anesthesia can
nance imaging (MRI) can help differentiate constrictive and typically be maintained with a balanced anesthesia technique
restrictive types of cardiomyopathy.138,142 using lower doses of inhaled potent volatile anesthetics, sup-
plemented with an opioid such as fentanyl or sufentanil.144,145
ANESTHETIC CONSIDERATIONS A high-dose opioid technique, as recommended in the past, is
Anesthetic and monitoring considerations in patients with usually reserved for patients with advanced disease who may
restrictive cardiomyopathies are similar to those of constric- not tolerate inhalational anesthetic agents.
tive pericarditis and cardiac tamponade, with the additional
feature of poor ventricular function in later stages of the dis-
Human Immunodeficiency Virus and the Heart
ease. (See Constrictive Pericarditis later for the physiology
and management of restrictive ventricular filling and earlier According to the U.S. Centers for Disease Control and Prevention
Dilated Cardiomyopathy for the management of impaired (CDC), at the end of 2010, more than 1 million people in the
ventricular function.) Anesthetic management depends on United States and more than 34 million worldwide may be
whether restrictive physiology or heart failure is predominant. infected with the human immunodeficiency virus (HIV).146,147
Despite normal ventricular function, diastolic dysfunction HIV affects all organ systems, including the cardiovascular sys-
in patients with restrictive cardiomyopathy leads to a low car- tem. The heart can be affected by the virus directly, by oppor-
diac output state. Monitoring should include at a minimum tunistic infections related to the immunocompromised state, by
invasive intra-arterial BP monitoring, and central venous access malignancies common to the disease, and by drug therapy.
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Chapter 2 Cardiac Diseases 43
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44 ANESTHESIA AND UNCOMMON DISEASES
BENIGN
CARDIAC TUMORS Myxoma 130 29.3
Primary tumors of the heart are unusual. However, the Lipoma 45 10.1
likelihood of encountering a cardiac tumor increases when
Papillary fibroelastoma 42 9.5
metastatic tumors of the heart and pericardium are con-
sidered. For example, breast cancer and lung cancer metas- Rhabdomyoma 36 8.1
tasize frequently to the heart.191 Primary cardiac tumors
Fibroma 17 3.8
may occur in any chamber or in the pericardium and may
arise from any cardiac tissue. Of the benign cardiac tumors, Hemangioma 15 3.4
myxoma is the most common, followed by lipoma, papil- Teratoma 14 3.2
lary fibroelastoma, rhabdomyoma, fibroma, and heman-
gioma192–194 (Table 2-6). The generally favorable prognosis Mesothelioma of A-V node 12 2.7
for patients with benign cardiac tumors is in sharp contrast Granular cell tumor 3 0.7
to the prognosis for those with malignant cardiac tumors.
Neurofibroma 3 0.7
The diagnosis of a malignant primary cardiac tumor is sel-
dom made before extensive local involvement and metas- Lymphangioma 2 0.5
tases have occurred, making curative surgical resection an Subtotal 319 72.0
unlikely event.
MALIGNANT
Rhabdomyosarcoma 26 5.8
Myxomas are most frequently benign tumors. They typically
originate from the region adjacent to the fossa ovalis and project Mesothelioma 19 4.2
into the left atrium. They are usually pedunculated masses that
Fibrosarcoma 14 3.2
resemble organized clot on microscopy and may be gelatinous
or firm. A left atrial myxoma may prolapse into the mitral valve Malignant lymphoma 7 1.6
during diastole. This often results in a ball-valve obstruction to Extraskeletal osteosarcoma 5 1.1
left ventricular inflow that mimics mitral stenosis; it may also
cause valvular damage by a “wrecking-ball” effect. More friable Neurogenic sarcoma 4 0.9
tumors result in systemic or pulmonary embolization, depend- Malignant teratoma 4 0.9
ing on the location and the presence of any intracardiac shunts.
Thymoma 4 0.9
Pulmonary hypertension may result from mitral valve obstruc-
tion or regurgitation caused by a left atrial myxoma, or pulmo- Leiomyosarcoma 1 0.2
nary embolization in the case of a right atrial myxoma. Atrial Liposarcoma 1 0.2
fibrillation may be caused by atrial volume overload. Surgical
therapy requires careful manipulation of the heart before insti- Synovial sarcoma 1 0.2
tution of cardiopulmonary bypass, to avoid embolization, and Subtotal 125 28
resection of the base of the tumor to prevent recurrence, with
444 100
overall very good early and long-term outcomes.195–197 total
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Chapter 2 Cardiac Diseases 45
relieve symptoms caused by mass effects.201 Patients with these become hemodynamically unstable. Adequate preload to
tumors respond poorly to radiotherapy and chemotherapy.202 maximize ventricular filling in the presence of an obstructing
tumor, slow heart rate, and high afterload to maintain perfu-
sion pressure in the setting of a fixed low cardiac output, are all
Metastatic Cardiac Tumors
goals when planning an appropriate anesthetic technique. The
Breast cancer, lung cancer, lymphomas, and leukemia may all use of intraoperative TEE can be invaluable in the management
result in cardiac metastases. About one fifth of patients who of patients with cardiac tumors (Fig. 2-3). In the 2010 prac-
die of cancer have cardiac metastases. Thus, metastatic cardiac tice guideline update, use of TEE is recommended for all open-
tumors are much more common than primary ones. Myocardial heart surgery, including removal of intracardiac tumors.126
involvement results in CHF and may be classified as a restric- Carcinoid tumors demand more challenging manage-
tive cardiomyopathy. Pericardial involvement results in cardiac ment strategies because the hypotension that can result from
compression from tumor mass or tamponade caused by effu- their manipulation may not be responsive to, and may even
sion. Melanoma is particularly prone to cardiac metastasis.203 be provoked by, certain vasoactive drugs, including epineph-
rine, norepinephrine, and dopamine. Castillo et al.209 review
the management of patients undergoing surgery for carci-
Cardiac Manifestations of Extracardiac Tumors
noid heart disease. Usually, general anesthetic management
Carcinoid is a tumor of neural crest origin that secretes sero- includes administration of a preoperative loading dose of the
tonin, bradykinin, and other vasoactive substances.204 Hepatic somatostatin analog octreotide, followed by a continuous infu-
carcinoid metastases result in right-sided valvular lesions, sion. Episodes of hypotension and hypertension are treated
presumably from a secretory product that is metabolized in with additional octreotide boluses and vasoactive drugs.
the pulmonary circulation. Recently, serotonin itself has been Epinephrine should probably be avoided and has been associ-
implicated in the pathogenesis of tricuspid valve dysfunc- ated with higher mortality in a recent study. Weingarten et al.210
tion.205–207 The end result is thickened valve leaflets that may acknowledge, however, that patients receiving epinephrine
be stenotic or incompetent, although regurgitation is more had worse preoperative New York Heart Association (NYHA)
common. functional class symptoms, which could partly explain this
Pheochromocytoma is a catecholamine-secreting tumor finding. The use of vasopressin in the hypotensive patient
also of neural crest origin. Chronic catecholamine excess has with carcinoid is generally considered safe. Most inotropic and
toxic effects on the myocardium that may result in a dilated vasoactive drugs have been administered in these patients in
cardiomyopathy.208 true emergencies and during significant hemodynamic com-
promise when unresponsive to octreotide alone. The periop-
erative administration of octreotide probably decreases the
Anesthetic Considerations
triggering effect of these drugs. Histamine-releasing medi-
The presence of a cardiac tumor requires a careful preoperative cations (e.g., morphine, meperidine, atracurium) should be
assessment of cardiac morphology and function. Transthoracic avoided. Induction medications (e.g., etomidate, propofol)
and transesophageal echocardiography, CT, and MRI are all and benzodiazepines (e.g., midazolam) have all been used suc-
used for diagnosis and assessment of treatment options. For the cessfully in patients with carcinoid disease.
anesthesiologist planning for the appropriate technique, these
imaging results are essential. A right-sided tumor, for exam-
ISCHEMIC HEART DISEASE
ple, is a relative contraindication to PAC insertion because of
the risk of embolization. Functional mitral stenosis caused by The most important aspects of coronary artery disease remain
a large left atrial myxoma may require hemodynamic manage- the same regardless of the etiology of the obstruction in the
ment similar to that of fixed mitral stenosis should the patient coronary arteries. As with that produced by arteriosclerosis,
FIGURE 2-3 A, Transesophageal
echocardiogram of mass on
right cusp of aortic valve. B,
Photograph of resected aortic
valve from same patient, with
the tumor attached to right cusp.
A B
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46 ANESTHESIA AND UNCOMMON DISEASES
the CAD produced by an uncommon disease retains the key Myocardial O2 Balance
clinical features. Physiologic considerations remain essentially
the same, as do treatment and anesthetic management.
The preoperative assessment should determine the symp-
toms produced by the CAD. Symptoms in the patient history
are angina, exercise limitations, and those of myocardial fail-
O2 Supply O2 Demand
ure, such as orthopnea or paroxysmal nocturnal dyspnea. The
physical examination retains its importance, especially when 1. Coronary blood flow 1. Blood pressure (afterload)
2. Oxygen delivery 2. Ventricular volume (preload)
quantitative data regarding cardiac involvement are not avail- a. O2 saturation 3. Heart rate
able. Physical findings such as S3 and S4 heart sounds are b. Hematocrit 4. Contractility
important, as are auscultatory signs of uncommon conditions
such as cardiac bruits, which might occur in a coronary arte-
riovenous fistula. If catheterization, echocardiography, and
other imaging data are available, the specifics of coronary
artery anatomy and ventricular function, such as end-diastolic
pressure, ejection fraction, and presence of wall motion abnor- FIGURE 2-4 Myocardial oxygen supply and demand balance.
malities, are all useful in guiding management.211,212
After ascertaining the extent of CAD, the clinician should
consider special aspects of the disease entity producing the ischemia in carbon monoxide poisoning, where the hemo-
coronary insufficiency. In ankylosing spondylitis, for exam- globin, although quantitatively sufficient, cannot carry oxy-
ple, coronary insufficiency is produced by ostial stenosis, yet gen. Similarly, the partial pressure of oxygen in arterial blood
valvular problems often coexist and even overshadow the (Pao2) is usually not a limiting factor. However, in conditions
CAD.213 In rheumatoid arthritis, however, airway problems where CAD coexists with cor pulmonale, as in schistosomi-
may be the most significant part of the anesthetic challenge. asis or sickle cell disease, the inability to maintain adequate
Hypertension, which frequently coexists with arteriosclerotic oxygenation may limit the myocardial O2 supply. In sickle
CAD, is also a feature of the CAD produced by Fabry's dis- cell disease it may be the key feature; failure to maintain an
ease. Other features to consider are metabolic disturbances, as adequate Pao2, secondary to repeated pulmonary infarctions,
when systemic lupus erythematosus produces both CAD and further increases the tendency of cells containing hemoglo-
renal failure.214 bin S to sickle, compromising myocardial O2 delivery through
“sludging” in the coronary microcirculation.219
The major factors determining myocardial O2 demand
Physiology of Coronary Artery Disease and
include heart rate, ventricular wall tension, and myocar-
Modification by Uncommon Disease
dial contractility. Tachycardia and hypertension after tra-
The key to the physiology of CAD is the balance of myocar- cheal intubation, skin incision, or other noxious stimuli are
dial oxygen (O2) supply and demand (Fig. 2-4). Myocardial common causes of increased myocardial O2 demand dur-
O2 supply depends on many factors, including the heart rate, ing surgery. Additionally, complicating factors of an unusual
patency of the coronary arteries, hemoglobin concentra- disease may also produce increases in demand. Increases in
tion, Pao2, and coronary perfusion pressure. The same fac- rate may occur as a result of tachyarrhythmias secondary to
tors determine supply in uncommon diseases, but the specific sinoatrial (SA) or A-V nodal involvement in amyloidosis or
manner in which an uncommon disease modifies these fac- in Friedreich's ataxia. Increases in wall tension may occur in
tors should be sought. A thorough knowledge of the anatomy severe hypertension associated with systemic lupus erythema-
of the coronary circulation and how the disease process can tosus (SLE), periarteritis nodosa, or Fabry's disease. Outflow
affect arterial patency is a useful starting point; this informa- tract obstruction with increased ventricular work can occur in
tion is usually derived from coronary angiography. In assess- primary xanthomatosis or tertiary syphilis; and diastolic ven-
ing the adequacy of coronary perfusion, the viscosity of the tricular radius can also increase, with greater wall tension, as
blood should be considered because flow is a function both of in aortic regurgitation associated with ankylosing spondylitis.
the dimensions of the conduit and the nature of the fluid in the Modern cardiac anesthesia practice should tailor the
system. In disease processes such as thrombotic thrombocy- anesthetic management to the problems posed by the pecu-
topenic purpura, sickle cell disease, or polycythemia vera, the liarities of the coronary anatomy. For example, knowledge
altered blood viscosity can assume critical importance.215–218 of the presence of a lesion in the left main coronary artery
Oxygen carrying capacity must also be considered in cer- dictates great care during anesthesia to avoid even modest
tain uncommon disease states. Hemoglobin concentration hypotension or tachycardia. Lesions of the right coronary
is usually not a limiting factor in the O2 supply to the myo- artery are known to be associated with an increased inci-
cardium. However, in diseases such as leukemia, anemia dence of atrial arrhythmias and heart block, and steps must
may be a prominent feature, and the myocardial O2 supply be taken either to treat these or to compensate for their car-
may be reduced accordingly. Another example is myocardial diovascular effects.
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Chapter 2 Cardiac Diseases 47
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48 ANESTHESIA AND UNCOMMON DISEASES
insignificant obstruction into a critical lesion. Patients with Systemic Lupus Erythematosus. The pericardium and myo-
coronary artery vasospasm may respond to nitroglycerin and cardium are usually affected in SLE. Patients with SLE, however,
calcium channel blockers. may suffer acute MI in the absence of atherosclerotic CAD.239,240
The hypercoagulable state of SLE together with a predisposition
COCAINE ABUSE to premature coronary atherosclerosis has been implicated.
Cocaine can affect the heart in several ways, and cocaine use In addition, glucocorticoids used for the treatment of SLE may
can result in myocardial ischemia, MI, and sudden death.225–228 also predispose these patients to accelerated atherosclerosis.
Cocaine exerts its effects on the heart mainly by its ability to
Kawasaki's Disease (Mucocutaneous Lymph Node
block (1) sodium channels, resulting in a local anesthetic or
Syndrome). In this disease of childhood, a vasculitis of the
membrane-stabilizing property, and (2) reuptake of norepi-
coronary vasa vasorum leads to weakened walls of the ves-
nephrine, resulting in increased sympathetic activity. Not sur-
sels with subsequent coronary artery aneurysm formation.241
prisingly, therefore, cocaine administered acutely can have
Thrombosis and myocardial ischemia can also occur. Patients
a biphasic effect on LV function, with transient depression
with Kawasaki's disease are prone to sudden death from ven-
followed by a sustained increase in contractility.229 Cocaine
tricular arrhythmias and occasionally from rupture of a coro-
also induces coronary vasospasm and reduced coronary
nary artery aneurysm. Thrombus in the aneurysm may also
blood flow while increasing heart rate and blood pressure.
embolize, causing myocardial ischemia.242
These effects decrease myocardial O2 supply and increase O2
demand. Cocaine and its metabolites can also induce platelet Takayasu's Disease. This disease leads to fibrosis and lumi-
aggregation and release platelet-derived growth factor, which nal narrowing of the aorta and its branches. The coronary
can promote fibrointimal proliferation and accelerated athero- ostia may be involved in this process.243
sclerosis.230,231 Chronic users of cocaine also have an exagger-
ated response to sympathetic stimuli, which may contribute to METABOLIC CAUSES
the LV hypertrophy frequently observed. Homocystinuria
An increased incidence of atherosclerotic disease is reported in
CORONARY ARTERY DISSECTION patients with high levels of homocysteine.244,245 This process may
When there is separation of the intimal layer from the medial involve intimal proliferation of small coronary vessels and an
layer of the coronary artery, there may be obstruction of the increased risk of MI. Nevertheless, meta-analysis and prospective
true coronary artery lumen with subsequent distal myocardial studies have not consistently confirmed these findings.246,247
ischemia. Coronary artery dissection may be primary or sec-
ondary. Primary coronary artery dissection may occur during CONGENITAL ABNORMALITIES OF CORONARY
coronary artery catheterization or angioplasty and in trauma ARTERIAL CIRCULATION
to the heart. Primary coronary artery dissection may also Left Coronary Artery Arising from Pulmonary Artery
occur spontaneously. Spontaneous dissection is usually asso- In Bland-White-Garland syndrome the right coronary artery
ciated with coronary arterial wall eosinophilia, and can also arises from the aorta, but the left coronary arises from the pul-
be seen in the postpartum period232 and with cocaine abuse.233 monary artery. Flow in the left coronary arterial system is ret-
Secondary coronary artery dissection is more common and is rograde, with severe LV hypoperfusion as well as myocardial
usually caused by a dissection in the ascending aorta. ischemia and infarction. As such, most patients with this defect
present in infancy with evidence of heart failure. Untreated
INFLAMMATORY CAUSES patients usually die during infancy. Patients who survive child-
Infectious hood may present with mitral regurgitation from annular
Infectious coronary artery arteritis may be secondary to hema- dilation. The goals of medical therapy are to treat CHF and
togenous spread or direct extension from infectious processes arrhythmias. The defect can be corrected surgically by pri-
of adjacent tissue. The infectious process results in thrombo- mary anastomosis of the left coronary artery to the aorta.248,249
sis of the involved artery with myocardial ischemia. Syphilis In older children, a vein graft or the left internal mammary
is one of the most common infections to affect the coronary artery may be used to establish anterograde flow in the left cor-
arteries. Up to 25% of patients with tertiary syphilis have ostial onary arterial system. Postoperative improvement in LV func-
stenosis of the coronary arteries.234,235 HIV infection has also tion can be expected if this surgery is performed early.250,251
been associated with CAD.236
Coronary Arteriovenous Fistula
Noninfectious There is an anatomic communication between a coronary artery
Polyarteritis Nodosa. This systemic necrotizing vasculitis and a right-sided structure, such as the right atrium, right ven-
involves medium-sized and small vessels. Epicardial coronary tricle, or coronary sinus. The right coronary artery is more fre-
arteries are involved in the majority of cases of polyarteritis quently affected and is usually connected to the coronary sinus.
nodosa. After the initial inflammatory response, the coronary Most patients are asymptomatic. These patients are at risk for
artery may dilate to form small, berrylike aneurysms that may endocarditis, myocardial ischemia, and rupture of the fistulous
rupture, producing fatal pericardial tamponade.237,238 connection.252 These fistulas should be corrected surgically.253
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Chapter 2 Cardiac Diseases 49
Anesthetic Considerations
artery should be checked frequently for signs of arterial insuf-
The anesthetic employed in patients with ischemic heart dis- ficiency. A more central and larger vessel, such as the axillary
ease or CAD should be tailored to the degree of myocardial artery, should be chosen for arterial catheterization. The use
dysfunction.254 In patients with pure coronary insufficiency of a PAC, once routinely deployed in patients with impaired
with good LV function, anesthetic management is aimed at ventricular function and CAD, has not been shown to improve
decreasing O2 demand by decreasing myocardial contractility outcome. In the absence of convincing evidence of outcome
while preserving O2 supply. Continuous monitoring of hemo- benefits associated with pulmonary artery catheterization,
dynamic parameters extending into the postoperative period decisions regarding this type of monitoring should be made on
is probably more important for patient outcome than choice of a case-by-case basis. Central venous access should be consid-
anesthetic technique. In patients with poor ventricular func- ered for administration of vasoactive medications in patients
tion, the anesthetic technique should maintain hemodynamic with significant CAD undergoing major procedures.
stability by avoiding drugs that produce significant degrees
of myocardial depression. A regional technique, if applicable,
may be the preferred anesthetic technique for smaller proce-
PULMONARY HYPERTENSION AND
dures. A neuraxial technique is not contraindicated as long as
COR PULMONALE
the patient's coagulation status, including potent antiplatelet Pulmonary hypertension (PHT) has been defined as mean
medications, is considered, and may actually provide supe- pulmonary artery pressure (PAP) greater than 25 mm Hg at
rior pain control and reduce the stress response to surgery. rest or greater than 30 mm Hg during exercise, or pulmonary
However, caution must be exercised to prevent a sudden drop vascular resistance (PVR) of 3 Wood units or greater, with a
in blood pressure, and thus a spinal technique is relatively con- pulmonary artery occlusion pressure of 15 mm Hg or less.258
traindicated. For major surgery, most practitioners employ a More recently, it has been suggested to simplify the definition
balanced general anesthetic technique. of PHT as a mean PAP greater than 25 mm Hg at rest, without
When coronary vasospasm is considered, it is important taking exercise or PVR into consideration.259 The 2008 revised
to maintain a relatively high coronary perfusion pressure. nomenclature on PHT (Dana Point Classification) lists five
Pharmacologic agents, such as nitroglycerin and calcium main categories: (1) pulmonary arterial hypertension, (2) PHT
channel blockers, may also be used. Patients who are chronic caused by left-sided heart disease, (3) PHT caused by lung dis-
users of cocaine should be considered at high risk for ischemic ease and/or hypoxia, (4) chronic thromboembolic PHT, and
heart disease and arrhythmias. These patients may respond (5) PHT with unclear multifactorial mechanisms1,260 (Box 2-3).
unpredictably to anesthetic agents and other drugs used in the
perioperative period. Ephedrine and other indirect sympatho-
mimetic drugs should be avoided in cocaine users. BOX 2-3 n DIAGNOSTIC CLASSIFICATION OF
In periarteritis nodosa or Fabry's disease, hypertension PULMONARY HYPERTENSION
is often associated with poor LV function. In such patients
1. Pulmonary arterial hypertension (PAH)
a vasodilator such as nicardipine, sodium nitroprusside, or 1.1 Idiopathic PAH
nitroglycerin can be used to control hypertension, rather than 1.2 Heritable PAH
a volatile anesthetic. Milrinone and nesiritide are also options. 1.3 Drug- and toxin-induced
The principles for the management of intraoperative arrhyth- 1.4 PAH associated with
1.5 Persistent PAH of the newborn
mias remain the same as for the treatment of arrhythmias in
1.6 Pulmonary veno-occlusive disease
the patient with atherosclerotic CAD. 2. Pulmonary hypertension caused by left-sided heart disease
The degree of functional impairment of the myocardium 2.1 Systolic dysfunction
and coronary circulation dictates the extent and type of moni- 2.2 Diastolic dysfunction
toring. The selection of ECG leads to monitor depends on the 2.3 Valvular disease
3. Pulmonary hypertension caused by lung disease and/or hypoxia
coronary anatomy involved. Diseases involving left CAD are
3.1 Chronic obstructive pulmonary disease
best monitored using precordial leads, such as the V5 lead. 3.2 Interstitial lung disease
In patients with right CAD, ECG leads used to assess the infe- 3.3 Other pulmonary diseases
rior surface of the heart (leads II, III, or aVF), or the posterior 3.4 Sleep-disordered breathing
surface (esophageal lead), are preferable.255–257 3.5 Alveolar hypoventilation disorders
3.6 Chronic exposure to altitude
Arterial blood pressure should be monitored by indwell-
3.7 Developmental abnormalities
ing catheter in patients with known coronary insufficiency 4. Chronic thromboembolic pulmonary hypertension
undergoing major procedures. The clinician should be cau- 5. Pulmonary hypertension with unclear multifactorial mechanisms
tious when using peripheral arterial monitoring in patients 5.1 Hematologic disorders
with generalized arteritis and carefully evaluate the adequacy 5.2 Systemic disorders (e.g., sarcoidosis)
5.3 Metabolic disorders
of collateral blood flow before cannulation of the periph-
eral artery. In occlusive diseases (e.g., Raynaud's, Takayasu's Modified from Simonneau G, et al: Updated clinical classification of
arteritis, Buerger's) or in cases of sludging in the microcircula- pulmonary hypertension, J Am Coll Cardiol 54:S43-S54, 2009.
tion (e.g., sickle cell disease), the area distal to the c annulated
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50 ANESTHESIA AND UNCOMMON DISEASES
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Chapter 2 Cardiac Diseases 51
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52 ANESTHESIA AND UNCOMMON DISEASES
Pulse oximetry and ABG sampling are simple ways of and cardiac output, in RV failure the reduction in RV afterload
assessing pulmonary function. Capnography is not an accu- can produce similar effects.
rate method of assessing Paco2 when significant “dead space” Dobutamine or milrinone tend to reduce PAP and PVR
ventilation is present. The use of an indwelling arterial cath- and probably are the inotropic drugs of choice in RV fail-
eter facilitates arterial blood sampling and continuous arterial ure without systemic hypotension. If RV perfusion pressures
BP monitoring. Calculation of intrapulmonary venous admix- need to be maintained, or when RV contractility is severely
ture by using mixed venous blood samples obtained from the impaired, norepinephrine or epinephrine is the preferred
pulmonary artery, however, is a more sensitive indicator of catecholamine, even in patients with PHT.273,274 Furthermore,
pulmonary dysfunction than Pao2 values alone. vasopressin is particularly effective for systemic hypotension
In the anesthetic management of patients with PHT or cor in patients with RV failure.275 Vasodilators found effective in
pulmonale, special consideration must be given to the degree reducing RV afterload include sodium nitroprusside, nitro-
of PHT and the functional state of the right ventricle. Possible glycerin, milrinone, adenosine, nifedipine, amlodipine, and
scenarios include isolated PHT with or without right-sided prostaglandin E1.276,277 Inhaled nitric oxide (NO) selectively
heart failure and acute or chronic cor pulmonale with or with- dilates the pulmonary vasculature and has been used to treat
out right-sided heart failure. The anesthetic management may PHT in various clinical settings.278–280
differ accordingly, ranging from vigilant monitoring to acute Prostacyclin acts via specific prostaglandin receptors and
cardiopulmonary resuscitation (CPR). Some general prin- has also been shown to reduce PHT.281 However, the vasodi-
ciples apply. Hypoxia, hypercarbia, acidosis, and hypother- lation is not selective for the pulmonary vasculature, and sys-
mia should be avoided because they increase PVR.258 The use temic hypotension may ensue. Prostacyclin analogs, such as
of a high inspired fraction of oxygen (Fio2) is often advised, epoprostenol, are given for chronic PHT and may also be use-
but pulmonary vascular reactivity and the underlying eti- ful for intraoperative use. One caveat is that inadvertent dis-
ology will often determine if a patient benefits from pulmo- continuation of chronic IV epoprostenol therapy may lead
nary vasodilator (including O2) administration. For example, if to a fatal pulmonary hypertensive crisis. The administration
PHT is coexistent with hypoxia in a patient with chronic bron- of prostacyclin, nitroglycerin, and milrinone by inhalation
chitis, O2 administration may afford significant relief of the is one strategy to reduce systemic side effects.282,283 Inhaled
PHT. If the PHT is secondary to massive pulmonary fibrotic prostaglandins are replacing inhaled NO in some institutions
changes or acute mechanical obstruction from thromboembo- because of cost considerations. Selective p hosphodiesterase-5
lism, however, little relief of PHT would be expected with O2 inhibitors (e.g., sildenafil) are becoming the mainstay of
administration. chronic PHT therapy. Preoperative optimization of patients
The type of anesthetic and anesthesia technique is proba- with severe PHT using sildenafil has been described.284,285
bly less important to patient outcome than the severity of PHT, Endothelin receptor (ET-1)–A antagonists (e.g., bosen-
associated right-sided heart failure, and surgery with expected tan, sitaxsentan, ambrisentan) are newer drugs that have been
hemodynamic instability. In 156 children with PHT undergoing approved by the U.S. Food and Drug Administration (FDA)
256 procedures, the incidence of complications increased with for the use in patients with PHT.286,287 Table 2-7 summarizes
the severity of PHT, and complications were not associated with the hemodynamic effect on the systemic as well as pulmonary
the type of anesthetic or airway management.269 This was con- circulation of some of the more common drugs used in the
firmed in another retrospective analysis of children with PHT anesthesia setting. Furthermore, medications used for treat-
undergoing general anesthesia.270 The type of agents used for ment of chronic PHT should not be discontinued perioper-
induction or maintenance of anesthesia was not associated with atively because of the expected acute exacerbation of PHT.
periprocedural complications. Major surgery, however, was a Since systemic hypotension is a side effect of some of these
predictor of adverse events. Traditionally, it has been taught that drugs, appropriate monitoring as previously outlined should
nitrous oxide might increase PAP and should be used cautiously be established before anesthesia induction. An anesthetic tech-
in this setting, even though scant data support this.271 nique associated with a sudden drop in arterial blood pressure,
When PHT coexists with cor pulmonale, the anesthetic such as a spinal anesthetic, is relatively contraindicated, except
technique should attempt to preserve RV function. The pri- with careful preparation and monitoring. A balanced general
mary concern is the maintenance of RV function in the face of anesthesia or epidural technique is preferred.
an elevated RV afterload. In this setting, a balanced anesthesia
technique employing opioids, such as fentanyl or sufentanil, in
combination with sedative-hypnotic drugs, such as propofol PERICARDITIS, EFFUSION,
or midazolam, or low doses of a potent inhalational agent will AND TAMPONADE
usually provide cardiovascular stability.272 Inotropic support
Constrictive Pericarditis
is often required in the patient with RV failure with chronic
cor pulmonale. An inotropic agent should be selected only The pericardium is not essential to life, as demonstrated by
after considering its pulmonary effects, and the effects of the the benign effects of pericardiectomy. Nevertheless, the peri-
inotropic intervention should be monitored. As in LV failure, cardium has several important functions. The intrapericardial
where the reduced LV afterload can increase stroke volume pressure reflects intrapleural pressure and is a determinant of
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Chapter 2 Cardiac Diseases 53
b-ANTAGONISTS
Propranolol, 0.5-2 mg — NC to ↑ NC or ↓ NC or ↓ ↓↓ NC or ↑
a-ANTAGONIST
PHOSPHODIESTERASE-III INHIBITOR
OTHER DRUGS
*Data not consistent; either NC or ↑ or ↓; most studies show less increase in PVR with norepinephrine compared with phenylephrine.
†Vasopressin significantly decreases cardiac output.
NC, No change; —, data unavailable; SL ↓, slight decrease.
HR, Heart rate; PAP, Pulmonary artery pressure; PCWP, pulmonary capillary wedge pressure; PVR, peripheral vascular resistance; QP, pulmonary blood flow; SAP,
systemic arterial pressure.
ventricular transmural filling pressure. During spontaneous restrictive cardiomyopathy, however, ventricular relaxation
ventilation, the pericardium serves to transmit negative pleu- is usually preserved in patients with constrictive pericarditis.
ral pressure, which maintains venous return to the heart.288 It restricts ventricular diastolic filling, so normal ventricular
Constrictive pericarditis results from fibrous adhesion of end-diastolic volumes are not obtained, and stroke volume is
the pericardium to the epicardial surface of the heart. Table 2-8 decreased. Compensatory mechanisms include an increase in
lists conditions associated with constrictive pericarditis. With heart rate and contractility, usually secondary to an increase
the key feature of increased resistance to normal ventricular in endogenous catecholamine release. This maintains cardiac
filling, constrictive pericarditis is a chronic condition usually output in the face of the restricted stroke volume until the
well tolerated by the patient until well advanced. Constrictive decrease in ventricular diastolic volume is quite severe. As car-
pericarditis often resembles a restrictive cardiomyopathy diac output falls, there is decreased renal perfusion, resulting in
and occasionally presents a diagnostic dilemma.289–292 Unlike increased levels of aldosterone and thus increased extracellular
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54 ANESTHESIA AND UNCOMMON DISEASES
TABLE 2-8 n Conditions Causing Pericarditis and TABLE 2-8 n Conditions Causing Pericarditis and
Cardiac Tamponade Cardiac Tamponade—Cont'd
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Chapter 2 Cardiac Diseases 55
constrictive pericarditis is usually 15 mm Hg or greater and time. In contrast, acute cardiac tamponade is a syndrome with
usually equals left atrial pressure. Both constrictive pericar- a rapid and dramatic onset of symptoms.293–296 With a more
ditis and cardiac tamponade demonstrate a diastolic “pres- gradual accumulation of fluid, the pericardium stretches, and
sure plateau” or “equalization of pressures,” in which the larger pericardial volumes are tolerated before symptoms
right atrial pressure equals the right ventricular end-diastolic occur. Once symptoms begin, however, they proceed rapidly
pressure, pulmonary artery diastolic pressure, and left atrial because of the sigmoidal relationship between pressure and
pressure. volume in the pericardial sac. As the limit of pericardial disten-
sibility is reached, small increases in volume produce dramatic
ANESTHETIC CONSIDERATIONS increases in intrapericardial pressure. As such, the removal of
Monitoring should assess the compensatory mechanisms in small volumes of pericardial fluid in a situation of severe car-
constrictive pericarditis. The ECG should be observed for diac tamponade can produce dramatic relief of symptoms as a
heart rate and ischemic changes, because the myocardial O2 result of a rapid fall in intrapericardial pressure.297
supply/demand ratio can be altered by the pathologic process The clinical features of cardiac tamponade result from
and therapeutic interventions. An indwelling arterial catheter restriction of diastolic ventricular filling and increased
for continuous BP monitoring should be established before pericardial pressure. The increased pericardial pressure
anesthesia induction. A central venous pressure catheter is is transmitted to the ventricular chamber. This decreases
often indicated for venous access in patients with constrictive the atrioventricular pressure gradient during diastole and
pericarditis undergoing more than minor procedures. The use impedes ventricular filling. Thus, despite an increase in dia-
of a PAC is controversial, however, and has not been shown stolic ventricular pressure, there is a decrease in the end-
to improve outcome. In patients with advanced disease, use diastolic ventricular volume and stroke volume. Similar to
of a PAC may provide useful information about cardiac func- advanced constrictive pericarditis, equilibration of right
tion, loading conditions, and cardiac output, particularly in atrial pressure, right ventricular end-diastolic pressure, pul-
the postoperative period when echocardiography is often not monary artery diastolic pressure, and left atrial pressure can
readily available. The intraoperative use of TEE can be help- be seen. Pulsus paradoxus is another nonspecific sign, also
ful in patients with constrictive pericarditis undergoing non- seen in patients with constrictive pericarditis. It consists of
cardiac surgery, when hemodynamic compromise can be a decline in systolic pressure during inspiration of more than
expected based on the planned surgery, or when circulatory 12 mm Hg. Additionally, increased diastolic ventricular pres-
instability persists despite attempted therapy.126 sure decreases coronary perfusion pressure and results in
The main anesthetic goals are to minimize any effects of early closure of the mitral and tricuspid valves, limiting dia-
anesthetic techniques and drugs on the compensatory mecha- stolic flow and reducing ventricular volume (Fig. 2-5).
nisms that maintain hemodynamic stability in patients with The compensatory mechanisms in cardiac tamponade
constrictive pericarditis. Accordingly, bradycardia and myo- are similar to those in constrictive pericarditis. A decrease
cardial depression must be avoided, and preload and afterload in cardiac output results in an increase in endogenous cate-
need to be maintained in the face of a fixed, low cardiac output. cholamines. The consequent increases in heart rate and con-
It is reasonable to induce general anesthesia using IV ketamine tractility help maintain cardiac output in the face of a decreased
or etomidate titrated to effect. Propofol is relatively contrain- stroke volume. Increased contractility increases the ejection
dicated because it may produce hypotension. Thiopental is fraction, allowing more complete ventricular emptying.
best avoided because of venodilation and cardiac depression.
A high-dose opioid anesthesia will not depress myocardial
contractility, but the associated bradycardia may not be tol-
erated. In patients who rely on sympathetic tone for compen- Pericardial
pressure
sation, even the use of high-dose opioids may cause sudden
hemodynamic compromise during anesthetic induction and
Ventricular Ventricular
must be immediately treated, preferably with epinephrine and pressure volume
norepinephrine. The use of a spinal/epidural technique is not
recommended in symptomatic patients with constrictive peri-
Gradient to Coronary Stroke
carditis because of the sympathectomy. ventricular filling perfusion volume
pressure
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56 ANESTHESIA AND UNCOMMON DISEASES
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Chapter 2 Cardiac Diseases 57
a. Congenital
(1) Valvular
(2) Discrete subvalvular
(3) Supravalvular
c. Degenerative
2. Infectious
3. Infiltrative
4. Miscellaneous
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58 ANESTHESIA AND UNCOMMON DISEASES
the valve increases, and total resistance to flow increases. TRICUSPID STENOSIS
The compensatory mechanisms in mitral stenosis include Isolated tricuspid stenosis is rare, and the etiology is either
(1) dilation and hypertrophy of the left atrium, (2) increases carcinoid or congenital (Table 2-11). The problems in tri-
in atrial filling pressures, and (3) a slow heart rate to allow cuspid stenosis are similar to those in mitral stenosis. There
sufficient time for diastolic flow with minimal turbulence.303 is a large, right atrial–right ventricular diastolic gradi-
Decompensation in rheumatic mitral stenosis usually occurs ent, and flow across the stenotic tricuspid valve is related
when there is atrial fibrillation with a rapid ventricular rate. to valve area.305,306 The compensatory mechanisms in tricus-
This causes a loss of the atrial contraction and decreased pid stenosis are also similar to those in mitral stenosis.
time for ventricular filling, which results in pulmonary vas- An increase in right atrial pressure maintains flow across
cular engorgement. Thus, altered LV function is almost the stenotic valve and is associated with hepatomegaly, jug-
never the limiting factor in the ability of the heart to com- ular venous distention, and peripheral edema. The implica-
pensate for mitral stenosis.304 tions of slow heart rate in tricuspid stenosis are the same as
As in other valvular lesions produced by uncommon dis- in mitral stenosis. Right ventricular contractility is usually
eases, coexistent cardiovascular problems that interfere with well maintained. The onset of atrial fibrillation in tricuspid
compensatory mechanisms are very important. Diseases such stenosis may produce symptoms such as increased periph-
as sarcoidosis or amyloidosis can infiltrate ventricular muscle, eral edema, whereas in mitral stenosis it results in signs of
preventing LV filling by decreasing compliance. Amyloidosis, left-sided failure.307
gout, and sarcoidosis can also affect the conduction system of Diseases can interfere with cardiac compensation for tri-
the heart, resulting in heart block, tachyarrhythmias, or atrial cuspid stenosis in much the same way as for mitral stenosis.
fibrillation (Table 2-10). Further restriction of RV filling may occur in conditions such
1. Inflammatory
a. Rheumatic fever
2. Infiltrative
a. Amyloidosis 1. Heart block Atrial dilation and Dilated and restrictive Malfunctioning of other
2. Infiltration of hypertrophy cardiomyopathy valves
conduction system
c. Gout Infiltration of
conduction system
3. Miscellaneous*
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Chapter 2 Cardiac Diseases 59
1. Inflammatory
2. Fibrotic
a. C
arcinoid Fibrosis evolving to 1. P
ulmonary hypertension Pulmonic stenosis
syndrome hypertrophy and with increased RV afterload
dilation 2. Endocardial fibrosis
b. E
ndocardial Similar to carcinoid
fibroelastosis syndrome
3. Miscellaneous
b. M
yxoma of right Usually normal compen
atrium satory mechanisms
CAD, coronary artery disease; LV, Left ventricular; RV, right ventricular; SLE, systemic lupus erythematosus.
as malignant carcinoid syndrome that produce endocardial low cardiac output. When RV pressure rises, a patent fora-
fibrosis, reducing RV compliance. Tricuspid stenosis frequently men ovale may produce right-to-left interatrial shunting.
coexists with pulmonic stenosis in patients with carcinoid syn- Usually, isolated pulmonic stenosis is well tolerated for long
drome, resulting in severely restricted cardiac output.308 periods until compensatory mechanisms fail. When a second
valvular lesion coexists with pulmonic stenosis, the potential
PULMONIC STENOSIS effects of this lesion on compensatory mechanisms should be
As in aortic stenosis, the valve area is the critical determi- considered.309
nant of transvalvular blood flow. Pulmonic stenosis produces Compensatory mechanisms in pulmonic stenosis can be
symptoms that are similar to the classic clinical features of altered in much the same way as in aortic stenosis. Decreases
aortic stenosis: fatigue, dyspnea, syncope, and angina. The in RV contractility occur in infiltrative diseases of the myo-
compensatory mechanisms in pulmonic stenosis are similar cardium, such as Pompe's disease and sarcoidosis. The loss
to those in aortic stenosis, including RV hypertrophy, and RV of the atrial “kick” and the development of tachyarrhyth-
dilation, with a change from a crescent-shaped chamber best mias have the same implications for cardiac function in pul-
suited to handle volume loads to an ellipsoid chamber best monic stenosis as in aortic stenosis. In subacute bacterial
suited to handle pressure loads. The hypertrophied and dilated endocarditis, or with geometric changes resulting in tricus-
right ventricle also interferes with LV function, and LV fail- pid annular dilation, tricuspid insufficiency may coexist with
ure may supervene. Angina occurs occasionally in pulmonic pulmonic stenosis, impairing pressure development in the
stenosis and should be especially noted. Normally, the right right ventricle, especially when RV failure supervenes. With
ventricle is a thin-walled chamber with low intraventricular the increase in RV mass and the increased requirement for
pressures, resulting in a high transmural perfusion pressure O2 delivery to the right ventricle, RV O2 supply may be com-
and good subendocardial blood flow that limits development promised, as in the coronary artery pathology of Pompe's
of RV ischemia. Concentric hypertrophy increases both RV disease (Table 2-12).
mass and RV pressures, increasing the potential for ischemia Many patients with pulmonic stenosis are candidates for
of the right ventricle, since RV O2 requirements are increased balloon valvuloplasty of the pulmonic valve. A balloon cath-
and coronary perfusion may be decreased. Cyanosis can eter is placed percutaneously and the tip guided across the
occur with severe pulmonic stenosis accompanied by a fixed, pulmonic valve. The balloon is inflated, tearing the fused
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60 ANESTHESIA AND UNCOMMON DISEASES
Disease Atrial Transport and Rhythm Contractility and Hypertrophy Associated Problems
1. Congenital
a. Valvular
b. Infundibular
3. Infiltrative
a. Pompe's Arrhythmias from conduction Dilated cardiomyopathy Aortic stenosis and outflow
system infiltration tract obstruction
4. Infectious
5. Neoplastic
a. Mediastinal tumors
b. Primary tumors
6. Physical: extrinsic
causes
a. Aneurysm of ascen
ding aorta or sinus
of Valsalva
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Chapter 2 Cardiac Diseases 61
leaflets apart.310 Various degrees of pulmonic regurgitation of heart failure develop.311 Nevertheless, the onset of clinical
are invariably produced, but this is typically well tolerated symptoms of aortic insufficiency does not necessarily corre-
for a long time. late with ventricular function status.312
The increase in chamber size and eccentric hypertrophy,
which help maintain cardiac function in aortic insufficiency,
Regurgitant Valvular Lesions
can be compromised in such conditions as ankylosing spondy-
AORTIC INSUFFICIENCY litis in which myocardial fibrosis limits the increase in cham-
The primary problem in aortic insufficiency is a decrease ber size to the degree that this disease produces a restrictive
in net forward blood flow from the left ventricle caused by picture. Cogan's syndrome produces a generalized cardiomy-
diastolic regurgitation of blood back into the LV chamber. opathy with CAD and can alter the compensatory mechanism
Acute aortic insufficiency is poorly tolerated and represents by decreasing both the ability of the left ventricle to hypertro-
an acute volume overload of the LV chamber, resulting in phy and the ability of the coronary arteries to deliver oxygen to
increased wall tension, end-diastolic pressure, acute mitral the ventricle. Increases in LV compliance could be prevented in
regurgitation, pulmonary edema, and cardiogenic shock. In situations such as aortic insufficiency caused by methysergide,
chronic aortic insufficiency, however, a number of compen- which produces an endocardial fibrosis and thus decreased
satory changes apply, and depending on the severity of aortic ventricular compliance. The usual ability of the left ventricle
regurgitation, patients may be asymptomatic for years. The LV to maintain the ejection fraction in aortic insufficiency could
chamber size increases, causing an eccentric hypertrophy. The be compromised by the cardiomyopathy of amyloidosis. The
LV compliance is increased, able to accommodate large dia- aortic insufficiency produced by acute bacterial endocarditis
stolic volumes at relatively low filling pressures. The increase is occasionally associated with complete heart block, result-
in ventricular volume allows full use of the Frank-Starling ing in a slow heart rate with ventricular overdistention and a
mechanism, whereby the strength of ventricular contraction decrease in cardiac output. Aortic insufficiency caused by con-
is increased with increasing fiber length. Ejection fraction ditions (e.g., SLE) associated with increased PVR can increase
is maintained, because both stroke volume and ventricular the regurgitant fraction in the face of the incompetent aortic
end-diastolic volume increase together. Despite these com- valve.313,314 Table 2-13 lists causes of aortic insufficiency and
pensatory mechanisms, however, studies have shown that ven- key features of their pathophysiology that can adversely affect
tricular contractility is not normal, and eventually symptoms cardiac compensatory mechanisms.
1. Infiltrative
2. Infectious
c. Rheumatic fever
Continued
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62 ANESTHESIA AND UNCOMMON DISEASES
4. Degenerative
b. O
steogenesis Normal Normal Cystic medial Mitral regurgitation
imperfecta necrosis
a. R
elapsing
polychondritis
b. S
ystemic lupus Pericarditis and effusion Hypertension Mitral regurgitation
erythematosus secondary to renal
disease
c. Reiter's syndrome
6. Systemic syndromes
a. A
nkylosing Complete heart Aortic dissection
spondylitis block
7. Miscellaneous
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Chapter 2 Cardiac Diseases 63
Compensatory mechanisms in mitral regurgitation include is secondary to PHT. Hypoxemia can increase PVR, as occurs
ventricular dilation, elevations in ventricular filling pressure, with the hypoxemia that results from pulmonary vascular dys-
and the maintenance of low PVR. As in aortic insufficiency, function in carcinoid syndrome. It is unusual for a cardiomy-
ventricular dilation allows maximum advantage to be gained opathy to coexist with isolated pulmonic insufficiency; thus
from the Frank-Starling mechanism. A low PVR maintains the potential for eccentric hypertrophy usually remains intact.
forward flow, whereas increases in PVR increase the degree of However, syphilis could allow a cardiomyopathy to coex-
regurgitant flow through the mitral valve. In mitral regurgita- ist with pulmonic insufficiency, although this would depend
tion, the heart benefits from a relatively rapid heart rate; a slow on the extent of syphilitic involvement of the myocardium
rate is associated with an increased ventricular diastolic diam- (Table 2-16).
eter, which may distort the mitral valve apparatus even further
and result in increased regurgitation.
Anesthetic Considerations
A number of diseases can be cited that interfere with the
compensatory mechanisms in mitral regurgitation. When Perioperative problems will arise from valvular lesions
mitral regurgitation is secondary to amyloid infiltration of when compensatory mechanisms acutely fail. Monitoring
the mitral valve, ventricular dilation is compromised by coin- should be selected to give a continuing assessment of the
cident amyloid infiltration of the ventricular myocardium, status of these compensatory mechanisms. Standard moni-
which restricts ventricular diastolic filling.318 Amyloid infil- toring that includes an ECG is essential for monitoring car-
tration of the conduction system can cause heart block and diac rhythm and ischemic changes.321 In moderate or severe
bradycardia, resulting in increased mitral regurgitation for valvular lesions, blood pressure is best monitored continu-
reasons previously noted (Table 2-14). ously with an indwelling arterial catheter, which also allows
for the monitoring of ABGs. TEE is useful for assessing the
TRICUSPID INSUFFICIENCY changes in preload and contractility that result from anes-
Tricuspid insufficiency is mechanically similar to mitral insuf- thetic and surgical manipulations. Pulsed-wave Doppler
ficiency. The most common cause of tricuspid insufficiency is and color flow mapping are useful for determining the flow
right ventricular failure.319 Carcinoid disease can cause defor- characteristics of valvular lesions and their response to phar-
mation and insufficiency of the tricuspid valve,320 and endocar- macologic or surgical manipulations.322 The insertion of a
ditis affecting the tricuspid valve can be seen in IV drug users. central venous catheter for vasoactive drug administration,
Tricuspid insufficiency represents a volume overload of both as well as for monitoring right-sided filling pressures, is
the right ventricle and the right atrium. Isolated tricuspid regur- indicated in patients with moderate to severe valvular dis-
gitation is often well tolerated, unless RV dysfunction develops ease. The value of a PAC is controversial; it may be contra-
or coexists. In this situation the loss of integrity of the RV cham- indicated in patients with tricuspid stenosis and difficult to
ber from the incompetent tricuspid valve increases regurgitant deploy in patients with severe tricuspid regurgitation. In
flow at the expense of forward flow through the pulmonary cir- patients with significant left-sided valvular disease, accurate
culation, decreasing the volume delivered to the left ventricle, assessment of loading conditions can be difficult or mislead-
with a resulting decrease in cardiac output (Table 2-15). ing because of the underlying valvular disease. Cardiac out-
put determination using a PAC can be inaccurate in patients
PULMONIC INSUFFICIENCY with severe tricuspid regurgitation. The decision to employ
Pulmonic insufficiency usually occurs in the setting of PHT a PAC in patients with valvular disease should be made on
or cor pulmonale but may exist as an isolated lesion, as in an individual basis, considering surgery-related factors and
acute bacterial endocarditis in IV drug users. Pulmonic insuf- practitioner experience as well.
ficiency may also be iatrogenic, frequently a sequela of pul- The anesthetic management in patients with valvular
monary valvuloplasty procedures or tetralogy of Fallot repair lesions varies significantly, depending on the severity of the
involving a transannular patch. It is well tolerated for long valvular disease, the underlying etiology, and the planned pro-
periods. As with aortic insufficiency, pulmonic insufficiency cedure. In patients with uncommon diseases, these are the
represents a volume overload on the ventricular chamber, but primary considerations in the choice of anesthetic drugs and
the crescentic RV geometry is such that volume loading is eas- techniques. With increasing severity of the valvular lesion,
ily handled. The right ventricle is normally a highly compli- patient management depends on the type of valvular lesion
ant chamber, and with its steep filling pressure–stroke volume rather than underlying disease, particularly in stenotic val-
curve, it functions well in the presence of volume increases. vular disease. In patients with fixed, low cardiac output from
Disease states can interfere with the compensatory mecha- a stenotic valvular lesion, afterload should be maintained at
nisms of the right ventricle in several ways. Diseases that pro- all times to allow adequate perfusion of vital organs, includ-
duce pulmonic insufficiency, such as the malignant carcinoid ing coronary perfusion. Therefore, an intrathecal technique is
syndrome, also produce an endocardial fibrosis that decreases often contraindicated in patients with significant stenotic val-
the ability of the RV chamber to dilate in response to volume vular lesions. If a neuraxial technique is chosen, an epidural
loading. Increases in RV afterload increase the regurgitant technique is preferred, with careful monitoring and inter-
fraction. This is especially true when pulmonic insufficiency vention to maintain sympathetic tone. Many practitioners,
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64
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a. Aortic regurgitation Usually in failure at this stage Elevated with low output
b. Chordal rupture
b. Rheumatic fever
e. Amyloidosis Sinoatrial and atrioventricular Restrictive and dilated Coronary artery disease
nodal infiltration cardiomyopathy
1. Annular dilation
a. Ebstein's anomaly
LV Compliance
Disease Contractility Heart Rate Rhythm Vascular Resistance Associated Abnormalities
1. Congenital
a. Isolated
(2) Aplastic
(3) Bicuspid
2. Acquired
4. Physical
a. Traumatic
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66 ANESTHESIA AND UNCOMMON DISEASES
however, choose a balanced general anesthetic technique is hypertension. Cyclosporine can also lower the seizure
with invasive hemodynamic monitoring in patients with sig- threshold. Tacrolimus is nephrotoxic and can lead to diabe-
nificant valvular disease. For anesthesia induction, etomidate tes and high blood pressure. Chronic corticosteroid therapy
is well tolerated by patients with valvular lesions,323 whereas is associated with glucose intolerance and osteoporosis, and
thiopental and propofol must be administered slowly and azathioprine is toxic to the bone marrow. The immunosup-
titrated to effect.324 Neuromuscular blocking agents should be pressive agents should be considered in the anesthetic plan,
selected according to their autonomic properties. In stenotic and organ systems that could be affected must be evaluated
lesions, cisatracurium, rocuronium, and vecuronium will not preoperatively.332,333
result in significant increases in heart rate and are preferred
over pancuronium.325,326 In the past, a high-dose opioid tech-
Anesthetic Considerations
nique was often used to maintain anesthesia in patients with
significant valvular heart disease, providing stable hemody- The physiology and response to pharmacologic agents are dif-
namics. Consequently, prolonged mechanical ventilation was ferent in the denervated heart. The vagal innervation of the
frequently required. In current practice, a balanced anesthe- heart is disrupted, and there is a lack of heart rate variability
sia technique using low doses of a potent inhalational volatile with respiration, vagal maneuvers, and exercise. Cholinesterase
anesthetic supplemented with shorter-acting opioids is pre- inhibitors, such as neostigmine and edrophonium, do not usu-
ferred by most practitioners aiming to achieve immediate or ally produce bradycardia, although case reports have linked
early extubation after surgery. cardiac arrest and bradycardia to neostigmine administration,
even in the transplanted heart.334,335 However, the effects on
other organ systems (e.g., salivary glands) remain, and these
PATIENTS WITH TRANSPLANTED HEART drugs must still be used in combination with anticholinergic
The first human heart transplantation was performed in the agents.336 Similarly, anticholinergic agents, such as atropine, do
late 1960s, but this practice was limited by early experiences not increase the heart rate, so that bradycardia is treated with
with organ rejection and opportunistic infections. Since 1980, direct acting agents, such as isoproterenol, or with pacing.
with the introduction of more effective immunosuppression A paradoxical response with the development of A-V block
and improved survival, the procedure has emerged as a widely or sinus arrest was reported after administration of atropine
acceptable treatment modality for end-stage heart disease.327 in patients with transplanted hearts.337 Drugs with vagolytic
These recipients of heart transplants may present for noncar- side effects, such as pancuronium, do not produce tachycar-
diac surgery, and therefore the physiology of the denervated dia. The denervated sinus and A-V nodes have been shown to
heart and the side effects of the immunosuppressive agents be supersensitive to adenosine and theophylline.338
must be considered. Sympathetic stimulation can originate from two sources:
neuronal or humoral. In the denervated heart the neuronal
input is initially disrupted and only partially restored, but
The Denervated Heart
increases in circulating catecholamines will increase heart
The recipient atrium (which may remain after transplanta- rate. Because of the denervation, indirect-acting cardiovascu-
tion) maintains its innervation, but this has no effect on the lar agents have unpredictable effects. The response of the cor-
transplanted heart. Therefore, the transplanted heart is com- onary circulation may also be affected.339,340
monly referred to as being “denervated.” The efferent (to the The normal, innervated heart responds to an increase in
heart) and afferent (away from the heart) limbs of the para- aerobic demand mainly by an increase in heart rate. However,
sympathetic and sympathetic nervous systems are disrupted the initial response of the denervated heart to an increased
during cardiac transplantation. This has significant impact on demand is through the Frank-Starling mechanism: increasing
the physiology of the transplanted heart and the response to stroke volume through preload augmentation. The increase
common pharmacologic agents in the perioperative period. in heart rate by the humoral pathway or circulating catechol-
Some degree of sympathetic reinnervation of the transplanted amines is delayed. Overall, the response of the denervated heart
heart has been documented, although this reinnervation is to exercise or increased metabolic demand is subnormal.341–343
delayed and incomplete.328,329 Sensory fibers in the heart play an important role in main-
taining systemic vascular resistance. With rapid changes in
SVR, the denervated heart may not respond appropriately,
Immunosuppressive Therapy
and these patients tolerate hypovolemia poorly. Sensory fibers
The main agents used for chronic immunosuppression are in the heart are also important in the manifestations of myo-
calcineurin inhibitors, azathioprine, rapamycins (everolimus/ cardial ischemia. As such, the patient with a denervated heart
sirolimus), tacrolimus, mycophenolate mofetil, corticoste- may not experience angina, although there are reports to the
roids, and azathioprine.330,331 These drugs may interact with contrary.
anesthetic agents and have side effects with anesthetic implica- Another factor that must be considered in the anesthetic
tions. Cyclosporine is nephrotoxic and hepatotoxic. Another management of these patients is that the transplanted heart
important side effect associated with the use of cyclosporine is also predisposed to accelerated coronary artery disease.
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Chapter 2 Cardiac Diseases 67
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