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268. Madani MM, Auger WR, Pretorius V, et al. Pulmonary
endarterectomy: recent changes in a single institution’s
experience of more than 2,700 patients. Ann Thorac Surg.
2012;94:97–103; discussion.
269. de Perrot M, Thenganatt J, McRae K, et al. Pulmonary
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270. Morsolini M, Nicolardi S, Milanesi E, et al. Evolving surgical
techniques for pulmonary endarterectomy according to the
changing features of chronic thromboembolic pulmonary
hypertension patients during 17-year single-center experience. J
Thorac Cardiovasc Surg. 2012;144:100–107.
271. de Perrot M, McRae K, Shargall Y, et al. Pulmonary
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272. Maliyasena VA, Hopkins PM, Thomson BM, et al. An Australian
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chronic thromboembolic pulmonary hypertension. Pulm Circ.
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273. Condliffe R, Kiely DG, Gibbs JS, et al. Improved outcomes in
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275. Suntharalingam J, Treacy CM, Doughty NJ, et al. Long-term use
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276. Jais X, D’Armini AM, Jansa P, et al. Bosentan for treatment of
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the treatment of chronic thromboembolic pulmonary
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278. Simonneau G, D’Armini AM, Ghofrani HA, et al. Riociguat for
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Balloon pulmonary angioplasty in patients with inoperable
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287. Roik M, Wretowski D, Labyk A, et al. Refined balloon
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33
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Hemodynamically Unstable Presentations of
Congenital Heart Disease in Adults
David Gregg IV, Stephanie Gaydos, Rochelle Judd, Elyse Foster
OUTLINE
Anatomic and Pathophysiologic Classification of Congenital
Heart Disease, 343
Arrhythmias, 344
Atrial Tachyarrhythmias, 344
Ventricular Tachyarrhythmias, 345
Ischemic Complications, 345
Heart Failure in the Adult With Congenital Heart
Disease, 346
Etiologies of Pump Failure, 347
Abnormalities of Diastolic Function, 347
Abnormalities of Valve Function, 348
Failed Palliative Procedures, 348
Systemic-Pulmonary Arterial Shunts, 348
Cavopulmonary Connections, 348
Heart Failure Management Considerations, 349
Prosthetic Valve and Prosthetic Material Failure, 349
Other Catastrophic Emergencies, 350
Cerebrovascular Disease, 350
Pulmonary Hemorrhage, 350
Eisenmenger Syndrome, 350
Conclusion, 350
Recent decades have seen great growth in the number of adults
with congenital heart disease (ACHD). Between 1985 and 2000,
the number of adults with congenital heart disease has doubled,
resulting in approximately 1 million adult survivors in the United
States who are increasingly having late complications. This is
accompanied by a similar increase in hospitalizations as more
adult patients develop late complications of disease.
population has emerged, adult caregivers are seeing more
congenital heart disease in practice but may have little training
in providing care for it. The anatomy and nomenclature of
congenital heart disease is often intimidating to a cardiologist
treating ACHD, but the care in most cases is analogous to that
of other adult patients. For example, the care of a young adult
with heart failure from a failing systemic right ventricle (RV) is
modeled after the deep clinical experience caring for patients
with left ventricular (LV) failure. Being aware of congenital
anatomy and the complications that are frequently seen in
common congenital lesions, however, is important to help focus
on the likely diagnosis and optimal treatment plan.
In general, patients with congenital heart disease come to
medical attention as adults because they have one or more of
the conditions presented in Box 33.1.
Most of these lesions never present as cardiac emergencies.
However, unique conditions related to surgical techniques and
long-term physiologic burdens do generate potential for emergent
presentations in this patient group. These conditions include
the history of incisions in the atrium or ventricle affecting the
conduction system or forming fibrous scars as the basis for
2
1
As this
arrhythmias, an anatomic RV functioning as a systemic ventricle
for decades, and residual lesions forming substrate for infective
endocarditis (Table 33.1). Another example is the fall in systemic
vascular resistance in these patients with Eisenmenger syndrome
who become pregnant increases the right-to-left shunt and results
in arterial desaturation.
Most emergent complications seen in congenital heart disease,
however, are similar to the emergency situations seen in adults
with acquired rather than congenital heart disease. For example,
the diagnosis of ventricular tachycardia in a patient with repaired
tetralogy of Fallot is treated in a manner similar to the patient
with coronary disease and ventricular tachycardia—unfortunately,
with the same uncertain efficacy. What is important to remember
is that a patient with repaired tetralogy of Fallot is at risk of
developing ventricular tachycardia and to recognize the importance of investigating complaints of palpitations, presyncope,
and syncope. Substantial analogies to the care of general cardiac
patients exist, but this needs to be combined with knowledge of
what complications to expect with what lesion and management
needs to be tailored to an ACHD patient’s unique anatomy (Table
33.2).
Congenital heart disease can predispose the patient to certain
complications that may be responsible for precipitating a cardiac
emergency. For example, in a patient with L-transposition of
the great vessels and shortness of breath, the RV is acting as the
systemic ventricle and is prone to failure. The diagnosis of heart
failure is not difficult and treatment is similar to the treatment
of congestive heart failure due to other conditions.
342
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Keywords
Adult congenital heart disease
emergencies
tetralogy of Fallot
arrhythmia
CHAPTER 33 Hemodynamically Unstable Presentations 343
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BOX 33.1 Complexity of Congenital Heart
Disease Lesions in Adults
Simple Lesions That May Escape Early Diagnosis (Native
Disease) or Have Had Early Repair
Native Lesions
Congenital aortic stenosis
Isolated mitral valve disease
Isolated patent foramen ovale (PFO) or small atrial septal defect (ASD)
Isolated small ventricular septal defect (VSD)
Mild pulmonary stenosis
Repaired Conditions
Previous ligated or occluded patent ductus arteriosus (PDA)
Repaired isolated ASD
Repaired isolated VSD
Moderately Complex Lesions
Asymptomatic in Childhood and Not Repaired
ASD
PDA
Ebstein disease
Coarctation of the aorta
Underwent Palliative Repair
Tetralogy of Fallot
Coarctation of the aorta
Lesions of Great Complexity
Palliated in Childhood
Single ventricles
D-transposition of the great vessels with atrial or arterial switch
Tricuspid atresia with Fontan operation
Not Amenable to a Surgical Procedure
Severe pulmonary vascular disease (Eisenmenger syndrome)
Patients with very small pulmonary arteries located where focalization or
shunts are not possible
ANATOMIC AND PATHOPHYSIOLOGIC
CLASSIFICATION OF CONGENITAL
HEART DISEASE
During the formation of the heart and cardiovascular system,
there are many opportunities for the development of the lesions
of congenital heart disease. The large variety of lesions of
congenital heart disease can be confusing. It is helpful for the
cardiologist seeing adult patients to think of these lesions in
an organized manner. The following classification is helpful in
that all congenital heart patients fit into one or more of these
categories.
1. Predominant left-to-right shunt. Blood that has gone through
the lungs and recirculates to the right side of the heart. As a
result, the pulmonary blood flow is greater than the systemic
blood flow. The shunt can occur at any level (e.g., at the
venous level—anomalous pulmonary vein to the superior
vena cava; at the atrial level—atrial septal defect [ASD]; at
the ventricular level—ventricular septal defect [VSD]; at the
arterial level—patent ductus arteriosus [PDA]).
2. Predominant right-to-left shunt, or “cyanotic disease.” The blood
is shunted from the right heart to the systemic circulation,
bypassing the lungs. The arterial blood is therefore desaturated.
If the amount of desaturated hemoglobin is 5 g/dL or greater,
cyanosis can be observed. The systemic blood flow is greater
than the pulmonary blood flow. This shunt also can occur
at any level of the cardiovascular system (e.g., at the venous
level, superior vena cava draining into the left atrium; at the
atrial level, tricuspid atresia with an ASD; at the ventricular
level, tetralogy of Fallot; at the arterial level, truncus
arteriosus).
3. Stenotic or atretic valves and hypoplastic or atretic ventricles.
The valves can also be incompetent. Either ventricle may be
hypoplastic or atretic.
TABLE 33.1 Cardiac Emergencies
Arrhythmia AF with bypass tract
Ischemia Ongoing chest pain with ischemic ECG changes Chronic nonischemic chest pain
Ventricular failure New murmur + fever
Cyanosis Loss of continuous murmur in patient with BT or central shunt
Noncardiac Hemoptysis
a
Warrants admission to cardiac intensive care unit.
AF, Atrial fibrillation; BT, Blalock-Taussig shunt; CHB, complete heart block; CHF, congestive heart failure; ECG, electrocardiogram; PVCs,
premature ventricular contractions; VT, ventricular tachyarrhythmia.
Life Threatening
Atrial flutter AF without bypass tract
Atrial arrhythmias in Mustard or Fontan procedures CHB: normal hemodynamics and nodal escape
CHB with hypotension or CHF and/or ventricular escape Isolated PVCs
Ventricular tachyarrhythmias with symptoms Asymptomatic, nonsustained VT
Compromising pleural effusion or ascites
Acute pulmonary infection
Transient ischemic attacks or seizures (new onset)
a
Not Life Threatening
Chronic cyanosis
Gout
Biliary colic
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TABLE 33.2 Complications of Congenital
Heart Disease
Lesion Special Considerations
Tetralogy of
Fallot
Fontan Ventricular dysfunction
D-transposition
of great
arteries
L-transposition of
great arteries
Coarctation Dissection
Left-to-right
shunt
Right-to-left
shunt (cyanotic)
Marfan syndrome Dissection
ASD, Atrial septal defect; AV, atrioventricular; IART, intraatrial
reentrant tachycardia; PDA, patent ductus arteriosus; VSD, ventricular
septal defect; WPW, Wolff-Parkinson-White syndrome.
Ventricular tachycardia
Atrial fibrillation
Right ventricular dysfunction
Pulmonary regurgitation (late complication usually
associated with moderate to severe regurgitation)
Sinus node dysfunction
Atrial flutter/IART
Fontan obstruction or leak
Pulmonary embolus
Atrial flutter/IART
Systemic right ventricular dysfunction
AV block (uncommon late)
Baffle systemic AV valve regurgitation
Sinus node dysfunction with junctional rhythm (usually
asymptomatic)
Systemic ventricular failure
AV block
Systemic AV valve regurgitation
WPW (2%–4%)
Bicuspid valve with endocarditis, regurgitation, or
stenosis
Early coronary artery disease or heart failure
Cerebral aneurysm
Pulmonary hypertension (Eisenmenger syndrome)
Atrial fibrillation
Endocarditis (VSD or PDA, rare with ASD)
Paradoxical embolus
Worsening cyanosis
Brain abscess
Bleeding diathesis
Hyperviscosity syndrome with erythrocytosis
Protein-losing enteropathy
Aortic valve regurgitation
Mitral valve prolapse
Patients with arrhythmias may complain of palpitations,
presyncope, or syncope. With atrial tachyarrhythmias, the
patient usually complains of palpitations. Presyncope or syncope
occurs with pulse rates of 200 beats/min or more. If the LV is
noncompliant with an atrial tachyarrhythmia, especially atrial
fibrillation, stroke volume can fall dramatically and the patient
may develop syncope. The diagnosis of this type of arrhythmia
can be made by electrocardiogram (ECG) if the arrhythmia
is persistent. However, even if the patient having syncope or
presyncope is in sinus rhythm at the time of the examination, it
is most important to consider the possibility that the patient with
congenital heart disease, with or without repair, has developed
a potentially fatal arrhythmia.
The atrial bradyarrhythmias and tachyarrhythmias encountered
in patients with congenital heart disease may result from hemodynamic alterations of the atrium or involve areas of slowed
conduction in the areas of scar associated with prior surgery.
These may include surgery for ASD repair,3 both ostium secundum
and ostium primum defects, Fontan procedures for tricuspid
atresia or single ventricle,
for transposition of the great arteries.
4,5
and the Mustard or Senning procedure
6,7
Lesions affecting the conduction system and causing atrioventricular (AV) block have become less common since surgeons
have learned to avoid the conduction system during surgery.
However, with any VSD repair, immediate injury or later injury as
a result of fibrosis is a possible cause of progressive heart block.
With advances in surgical technique and knowledge of the
path of the conduction system, heart block after repair of VSD
is increasingly rare, with persistent heart block seen in less than
1% of patients.
8
L-transposition, or corrected transposition of the great vessels,
is a lesion in which ventricular inversion has occurred without
inversion of the atria or great arteries. In this condition, the
anatomic RV is the systemic ventricle and the anatomic LV is
the pulmonic ventricle, but the physiologic passage of blood is
normal (i.e., the systemic venous return is pumped to the lungs
and the pulmonary venous return is ejected into the aorta). The
conduction system is also inverted, and the AV node is abnormally
located and often dual with elongation of the bundle of His.9
As a result, these patients have a high rate of AV block, which
can occur at all levels of severity—from first to third degree—and
which increases in incidence with age at a rate of about 1% to
2% per year.
4. Great vessel abnormalities. Transposition of the great vessels,
coarctation of the aorta, PDA, vascular rings, and truncus
arteriosus.
5. Abnormalities of position. Transposition of the great vessels,
L-transposition, dextroposition, and dextrocardia.
Arrhythmias
Arrhythmias are some of the more common emergencies seen
in patients with congenital heart disease. There are two types of
arrhythmias: bradyarrhythmias (e.g., sick sinus syndrome, sinus
arrest, varying degrees of heart block—including complete heart
block) and tachyarrhythmias (e.g., atrial fibrillation, atrial flutter,
ventricular tachycardia, and fibrillation).
Atrial Tachyarrhythmias
The diagnosis and treatment of bradyarrhythmias and tachyarrhythmias are the same in patients with congenital heart disease
as for those with other lesions. When symptomatic bradycardia
and hemodynamic instability (such as hypotension or syncope)
are present, a pacemaker is indicated.
Atrial fibrillation, tachycardia, and flutter, when they occur
in patients with congenital heart disease, are usually not life
threatening.
with ASDs and can recur even after the ASD is repaired, especially
when the repair is performed late in life (after the age of 40
years).3 In some defects, atrial fibrillation or atrial flutter can be
10,11
These arrhythmias occur in about 20% of patients
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very serious and even life threatening. The treatment is similar
to that of atrial fibrillation or atrial flutter due to other causes;
in patients with hemodynamic instability, immediate cardioversion
is indicated. In patients who are hemodynamically stable with
noncontracting atria, anticoagulation therapy for 3 weeks before
cardioverting is indicated. In such cases, the patient should receive
anticoagulation for 3 weeks after cardioversion until mechanical
atrial contraction is well established. If the patient is hemodynamically stable, the ventricular response can be slowed with amiodarone, β-blockers, verapamil, or diltiazem. Transesophageal
echocardiography (TEE) to rule out evidence of atrial thrombus
is preferred if technically feasible, as it reduces the risk of
embolization following cardioversion. With atrial tachycardia,
6 mg adenosine given intravenously usually converts the patient
to sinus rhythm. If this treatment is unsuccessful, another 6 to
12 mg of adenosine can be given.
Atrial flutter or intraatrial reentrant tachycardia occurs frequently in patients who have undergone a Fontan procedure,
with prevalence as high as 50% in adult patients. The presentation
is usually subacute but occasionally hemodynamic instability
and even sudden death may occur, especially in the setting of
1 : 1 conduction.12 The patient should be converted to normal
sinus rhythm either pharmacologically or by cardioversion with
the caution that antidysrhythmics may exacerbate sinus node
dysfunction, AV conduction, or promote 1 : 1 conduction of an
atrial arrhythmia. If atrial flutter recurs, the patient should be
referred to an electrophysiologist to map the pathways of flutter,
if possible. If this can be done, catheter ablation of the pathway
is possible. If atrial flutter or atrial tachycardia recurs incessantly
and ablation attempts fail, ablation of the AV node with placement
of a dual-chamber pacemaker should be considered. If the patient
does not remain in sinus rhythm, then a physiologically responsive
pacemaker is the treatment of choice.
Another atrial arrhythmia that can be fatal is atrial tachycardia
and subsequent atrial fibrillation in a patient with an antegrade
conducting bypass tract. In this condition, the impulse conducts
from the atrium to the ventricle over the bypass tract. Especially
with sympathetic stimulation or increased conductivity induced
by digitalis, the ventricular response can approach 250 to 300
beats/min, and the patient may develop ventricular fibrillation.
In patients with a possible AV bypass tract, digoxin should always
be avoided. Patients with Ebstein anomaly have 25% incidence
of bypass tracts in the posteroseptal location and a bypass tract
may be present in 2% to 4% of patients with L-transposition of
the great arteries.
In patients with atrial fibrillation and an AV bypass tract (i.e.,
Wolff-Parkinson-White syndrome), digoxin should always be
avoided. In these patients, the ventricular response is controlled
with a β-blocker or calcium channel blocker. When present, the
AV bypass tract should be ablated.
Ventricular Tachyarrhythmias
Ventricular tachyarrhythmias can occur because of incisions in
the RV or LV with fibrous scar forming the substrate for reentrant
arrhythmias or, more commonly, with progressive ventricular
enlargement with decreased LV or RV function or hypertrophy
of the LV or RV.
The development of ventricular tachyarrhythmias late after
surgery is not uncommon. This is especially true in patients
with tetralogy of Fallot; sudden death later after surgery is seen
in about 6% of patients in long-term follow-up.13 Late sudden
death and ventricular arrhythmias in tetralogy of Fallot correlate
well with the degree and duration of pulmonary regurgitation,
with arrhythmias increasing as continued pulmonary regurgitation
results in progressive RV enlargement.14 As the RV enlarges, there
is increased fibrosis, QRS prolongation, and QT dispersion that
appears to provide the substrate for ventricular tachycardia.
15,16
Fortunately, it appears that timely pulmonary valve replacement
may decrease the incidence of ventricular arrhythmias.
17
The patient with ventricular arrhythmias may have palpitations,
presyncope, or syncope. The diagnosis of ventricular tachycardia
can be made on ECG, either on presentation or on an ambulatory
ECG. If the arrhythmia is frequent but short lived, an event
recorder, which allows continuous ECG monitoring and activation
of recording when symptoms occur, may be helpful in making
the diagnosis.
Any patient with a history of congenital heart disease presenting with presyncope or syncope must be considered as having a
potentially life-threatening arrhythmia. In such patients, if the
diagnosis cannot be made on an ECG, electrophysiologic testing
should be considered for prognostication and to potentially assist
with therapy. Antidysrhythmic therapy has not been shown to
be of life-sustaining benefit in congenital heart disease, although
it may be important for symptom control. Therefore, the use
of an implantable cardioverter-defibrillator (ICD) should be
considered in patients considered at high risk for sudden death.
Ischemic Complications
In adult patients with congenital heart disease, ischemic complications related to their congenital defect causing cardiac emergencies are rare. Coronary artery abnormalities in adulthood can
generally be categorized as either acquired anomalies resulting
from congenital surgical manipulation or as congenital anomalies
associated with ectopic origins of the coronary arteries. The
potential reasons for the occurrence of myocardial ischemia in
adults as related to congenital heart disease are presented in
Box 33.2.
The most common anomaly of an ectopic coronary artery
origin is anomalous left coronary artery arising from a pulmonary
artery (ALCAPA). This condition is nearly always diagnosed in
infancy, as it typically causes severe ischemia leading to an
extensive anterolateral myocardial infarction (MI). Common
presentations include heart failure related to infarction or “angina”
manifested by poor feeding or crying at times of exertion (such
as feeding). By the age of 1 year, most of these children have
been diagnosed or died. Quite rarely, adult patients present with
undiagnosed ALCAPA.
death, dilated cardiomyopathy with heart failure, and progressive
mitral regurgitation. Extensive collateral flow from a dilated
coronary right artery permits survival into adulthood. Prompt
repair is indicated.
Cardiologists may also see patients who have undergone surgical
correction of ALCAPA in infancy or childhood via a tunnel repair
or, occasionally, with simple ligation of the left coronary artery.
19
Presentations include ischemia, sudden
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