The Adult Patient with Congenital Heart Disease
March 16, 2008 25 minutes read
The Adult Patient with Congenital Heart Disease
Author: Preeti Jois-Bilowich, MD, Clinical Associate, Cleveland Clinic, Department of Emergency Medicine, Cleveland, OH.
Peer Reviewer: Alfred Sacchetti, MD, Chief of Emergency Services, Our Lady of Lourdes Medical Center, Camden, NJ.
Nearly three decades ago, a 40-year-old woman presented to my emergency department short of breath. She was possibly the most cyanotic person I have ever seen alive or dead. She had been diagnosed with a "hole in her heart" at birth, and the family was told there was nothing that could be done. She was sent home to die at the age of 2. Because death was imminent, she was not sent to school, never saw a doctor again, and spent most of her life as an invalid. Now at age 40, she had Eisenmenger's syndrome with right to left shunting, severe pulmonary hypertension, and a hemotocrit of 70. She died shortly after presentation.
This patient illustrates just how far we have come in the treatment of congenital heart disease. The same patient today would undergo a serious operation, but with an operative risk of less than 2%. Today, my patient would have lived a long and likely normal life.
Surgical repair of congenital heart disease dates back to the 1940s and the historic work of Helen Taussig (cardiologist), Alfred Blalock (surgeon), and Vivien Thomas (laboratory technician). Their work led to the Blalock-Taussig shunt, which in essence created a patent ductus arteriosus and shunt for blood into the lungs. This operation allowed many children with tetralogy to survive, many until the next phase of cardiac repair was developed.
However a Blalock-Taussig shunt would not have helped the patient described above. Ventricular septal defect (VSD) was first described in 1879, and Eisenmenger described the subsequent right to left shunt in 1898. Lillehei and associates at the University of Minnesota repaired the first VSD in 1954 using cross circulation from a live adult donor. Cardiopulmonary bypass was developed shortly thereafter. Progress in cardiac surgery and anesthesia has made this procedure safe and effective.
But now we are faced with a new group of patients, those whose hearts were surgically rearranged several decades before. Most often they will have illnesses independent of their previous surgery. However, we are still learning about the long-term consequences of a repair. There simply have never been older adults with repaired congenital disease. The physician must be aware of the special consequences of the previous surgery.
Sandra M. Schneider, MD, FACEP, Editor
Introduction
There has been recent emphasis in cardiology literature highlighting the importance of recognizing a new sub-specialty within the field, aimed at treating adult patients who have survived after having congenital heart disease repair. This new arena is focused on adult patients with congenital heart disease (ACHD), alternatively known as grown-up congenital heart disease (GUCH).1,2 During the 1950s, the medical field witnessed the development of early recognition and treatment of congenital heart disease. As this stage was set, innovations in pediatric cardiac surgery and new research brought about better technique for repair, and increased survival in infants born with structural cardiac congenital abnormalities.
As a result of these developments, physicians now are faced with a new population of cardiac patients: the adult with congenital heart disease (ACHD). Their long-term complications are now emerging and will continue to emerge for the next several decades. With increasing survival into adulthood, patients with repaired congenital heart disease have now become a population with which the emergency physician needs to have a better familiarity. These patients will present to emergency departments with a variety of chief complaints related to their post-surgical conditions, and with clinical presentations ranging from mild exacerbations to serious and often life-threatening states.
Background
The term "survival" in the postoperative congenital heart disease patient used to be associated with surgical complications, infections, and immediate morbidity and mortality. With recent estimated numbers of up to 800,000 adult congenital heart disease patients in the United States, the new focus on "survival" in these repaired congenital heart disease patients is in continuing their medical surveillance.3,4 Many patients who undergo cardiac surgery for congenital heart disease may have a perception of "a medical cure," when in fact the reality is more of a palliative rather than a curative condition. The comprehension of this concept is encouraged so that patients are more likely to understand the nature of their anomalies, the potential outcomes of their surgeries, as well as to continue to seek ongoing medical care and advice post-operatively.5 Unlike adult patients who have acquired heart disease, where there is a gradual onset of symptoms that may be noticed by the patient, adult patients with congenital heart disease may not have prolonged and progressive symptoms, but rather present with acute deteriorations.
The true incidence of congenital heart disease is merely an estimate. In general, the incidence of congenital heart disease ranges from 4-8 per 1000 live births.4 In the United States there are approximately 30,000 new cases every year, compared with close to 2 million new cases per year worldwide. Some estimates suggest that there are more adults than children with congenital heart disease in the United States.6 Nearly 20,000 cardiothoracic surgeries are performed yearly in the United States on infants and patients who have congenital heart defects. It is estimated that more than 85% of these patients will go on to reach adulthood.7
ACHD in the Emergency Department
One method to approach the gamut of clinical presentations that may be seen in adult patients with congenital heart disease is to look at some of the most common reasons for emergency room visits. Arrhythmias are the most common reason for symptomatic ACHD patients to present to the emergency department. This is followed by acute heart failure, and then in no particular descending order, infection, syncope, thromboembolic disease, bleeding, and sudden cardiac death.8 (See Table 1.)
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Another way to classify emergency department visits by ACHD patients is to group them by principle diagnosis of the congenital cardiac defect. If this system is utilized, then patients with repaired tetralogy of Fallot and univentricular heart tend to be the majority of ACHD patients presenting to the emergency department. Other common ACHD cases include discordant connections, such as transposition of the great arteries, valvular abnormalities, septal defects, and outflow obstructions.9
Tetralogy of Fallot
Tetralogy of Fallot (TOF) is the most common cyanotic heart condition presenting in early childhood. It also is the most common ACHD that has been surgically repaired.1,2 The defect consists of pulmonary stenosis (obstruction of the right ventricular outflow tract), ventricular septal defect (VSD), overriding aorta, and right ventricular hypertrophy (RVH). (See Figure 1.) (See Table 2 for a list of abbreviations.) This disease, in many ways, spurred the development of new techniques to treat congenital heart disease. In 1945, Blalock and Taussig described their procedure using a palliative end-to-side subclavian to pulmonary artery shunt.10 Surgical creativity continued to flow, with Brock and Campbell using a closed infundibular resection procedure,11 and Kirklin performing the first successful repair of Tetralogy using a pump oxygenator, in 1955.12 Today, most patients are repaired early after diagnosis. While some symptomatic infants still undergo a modified Blalock-Taussig anastomosis, others may be directly repaired in early childhood. The modified Blalock-Taussig shunt is currently the most commonly used surgical procedure to treat patients with TOF.13 (See Figure 2.)
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While early outcomes are positive in patients who are repaired, later problems with arrhythmias and sudden cardiac death may occur. One multi-centered study evaluated nearly 800 patients and addressed risk factors for arrhythmia or sudden death after repair of TOF.14 In this study the mean age at repair was 8 years, and the patients were followed for roughly 21 years post-repair. Overall, the findings showed that older age at initial repair was associated with higher risk of sudden death and atrial tachyarrhythmia. In those patients who had ventricular tachycardia and sudden death, pulmonary regurgitation and a dilated right ventricle were the main underlying hemodynamic lesions. In patients with atrial flutter or atrial fibrillation, tricuspid regurgitation was usually the inciting lesion.
The incidence of late sudden death following repair of tetralogy of Fallot is reported to be close to 6% of survivors. Although sudden death can be caused by later onset of complete atrioventricular block, it is generally believed that poor outcomes from residual hemodynamics as well as sustained ventricular tachycardia are the relevant predisposing factors of sudden death. In a study conducted at Toronto Hospital, most patients with sustained ventricular tachycardia after congenital repair of tetralogy of Fallot have notable structural abnormalities such as right out flow tract aneurysm or significant pulmonary regurgitation.15
Right ventricular dysfunction is also known to be a cause of late morbidity and mortality after TOF repair. In these patients, chronic degrees of moderate to severe pulmonary regurgitation results in augmented right ventricular volume, which then progresses to right heart failure. These patients present with increasing fatigue and dyspnea and may have an associated serious ventricular arrhythmia.16,17 In patients with repaired TOF who have chronic pulmonary regurgitation, sudden death is often the presenting feature. Patients may not know the symptoms until right ventricular dysfunction is severe. In certain cases there is a phenomenon known as "ventricular-ventricular interaction" that occurs in the setting of right ventricular systolic dysfunction and enlargement, and has associated left ventricular dysfunction. However, the mechanism that links right ventricular dysfunction to left ventricular dysfunction is not completely understood.18
Patients with a repaired tetralogy will often have residual outflow track obstruction and pulmonary regurgitation. They will have a harsh to-fro murmur or, at times, a systolic ejection murmur. The normal ECG will show sinus rhythm, with right axis deviation and right ventricular hypertrophy. Many will have right bundle-branch block as well. Any patient who has had a previous surgical repair of tetralogy of Fallot ought to have a normal heart size on chest radiograph, and any evidence of an increased cardiothoracic ratio should prompt the ED physician to look for a residual hemodynamic lesion. This lesion can be a ventricular septal defect, pulmonary stenosis, or aortic regurgitation. The chest x-ray may also show a right aortic arch. As discussed before, the most common problem post-surgically tends to be pulmonary regurgitation. Patients who have residual pulmonary regurgitation will require surgical repair of the pulmonary valve. Early cardiology consultation is required for symptomatic patients in the ED.
Transposition of the Great Arteries (TGA)
Simple or d-transposition is the most common cyanotic abnormality in newborns.5 In this condition, the right atrium is connected to the right ventricle, out of which arises the aorta. The left atrium enters the left ventricle, which consequently gives rise to the pulmonary artery. (See Figure 3.) Surgical procedures first performed in the 1960s used the Mustard or Senning operations, which redirected blood flow via an atrial inlet so that systemic venous blood was directed into the left ventricle and then to the pulmonary artery. Pulmonary venous blood was directed to the right ventricle and then to the aorta.19,20
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The chest x-ray after repair generally will show cardiomegaly from the dilated right ventricle. The ECG will show right axis deviation and right ventricular hypertrophy.
Patients who underwent a Mustard or Senning procedure can develop right heart failure later in life. The innate morphology of the right ventricle does not lend it to be used chronically as the systemic ventricle. So while the right ventricle may function well for ae time after initial repair, its function eventually deteriorates and usually results in tricuspid regurgitation. Congestive heart failure is fairly common by the fourth and fifth decades of life. Patients presenting with exercise intolerance and hypoxemia have the highest risk of hospitalization and death.21 The initial approach to patients who present with heart failure should include a thorough physical examination, chest x-ray, ECG, and, where available, an echocardiogram. Evidence of an increasing cardiothoracic ratio on chest x-ray should prompt a search for tricuspid valve regurgitation. Patients who present in acute congestive heart failure should be treated in the same manner as other patients with acquired heart disease and heart failure. However, there is little evidence that emergent administration of beta-blockers or angiotensin converting enzyme inhibitors has a beneficial effect for these patients on exercise capacity, ejection fraction, or length of life.22 For patients without a systemic left ventricle, there is no current evidence-based therapy.
About 20 years ago, the Mustard or Senning atrial-switch procedure was replaced with an arterial-switch procedure, allowing the left ventricle to regain its function as the systemic ventricle. In the arterial-switch procedure, the pulmonary artery and the aorta are each transected above the level of the semilunar valves and coronary arteries. The aorta is then reattached to the "neoaortic" valve (the former pulmonary valve), and the pulmonary artery is connected to the "neopulmonic" valve (the former aortic valve).23,24
Patients with both post-operative transposition and with congenitally corrected transposition are vulnerable to sinus node disease. Sinus node dysfunction and atrial arrhythmias are more commonly seen in patients who underwent a Mustard or Senning repair, due to surgical trauma to the sinoatrial (SA) node or the SA artery.25,26 The emergency approach to these patients should be stabilization with oxygen administration, cardiac monitoring, and early cardiology or cardiothoracic surgery consultation.5 Current recommendations call for implantation of a pacing system for patients with congenital heart disease and symptomatic SA node dysfunction.27
Patients who have undergone Mustard or Senning repair are also at increased risk for intra-atrial reentrant tachycardia (IART) or atrial flutter. IART is seen years after operations involving atriotomy or other surgical manipulation of the right atrial tissue.28 It is often seen concomitant with other sinus node dysfunction such as tachy-brady syndrome or in patients who were older at time of heart surgery.29 ED treatment of IART (atrial flutter) is with standard therapy using electrical cardioversion, overdrive pacing, or administration of antiarryhthmic agents.30 These patients will require electrophysiologic studies to evaluate for possible pacemaker implantation or surgical intervention. Patients who underwent an arterial switch procedure for transposition also may have had a repair of anomalous origin of the left coronary artery. In these patients, coronary artery stenosis may occur with time with increased risk of coronary artery disease.
Univentricular Heart
The Fontan procedure was first described in 1971 after its use on three patients with tricuspid atresia. The anatomic defect of tricuspid atresia is the absence of normal tricuspid valve leaflets, which prevents a normal tract from forming from the right atrium to the right ventricle. This creates a hypoplastic right heart with resultant univentricular physiology. The Fontan procedure allows systemic venous blood to bypass the right heart and enter the lungs directly.31 Ideally, the Fontan procedure is performed between 18 months and 6 years of age. As a result of surgical modifications and advancements in postoperative care, the long-term survival of patient undergoing a Fontan procedure is increasing. However, patients with single ventricle physiology still retain a risk of developing ventricular dysfunction, dysrhythmias, worsening hypoxemia, elevated pulmonary vascular resistance, and ultimately "failing Fontan physiology."32 In one study, perioperative survivors of Fontan surgery had eventual mortality related to complications from heart failure, thromboembolic events, or sudden death.33
Up to 50% of patients who undergo Fontan operations will have atrial tachycardia within 10 years of the surgery.34 The ECG will be variable but often atrial or ventricular hypertrophy is seen along with axis deviation and conduction abnormalities. In the older Fontan operations, extensive suture lines and long-term hemodynamic stress can result in abnormal atrial myocardial tissue. An ECG obtained in the ED will show atrial flutter at a rate of 2:1 or 3:1. Once it is recognized, it should be treated with electrical cardioversion, antiarrhythmic medications, or pacing therapy.28,29,35
Patients presenting with evidence of severe left ventricular dysfunction will require inotropic support. Phosphodiesterase (PDE) inhibitors such as milrinone provide inotropic and vasodilatory rescue, which is beneficial to the "failing Fontan" patient.36 Despite a lack of clinically tested data, angiotensin-converting enzyme (ACE) inhibitors are frequently used for patients with failing Fontan circuits. Several studies have shown elevated levels of fluid homeostatic hormones (antidiuretic hormone, renin, angiotensin, aldosterone) in patients with Fontan circuits.37,38 These findings support the theory that ACE inhibition may be effective for patients with failing Fontan physiology. Chronic heart failure also leads to increased sympathetic nervous system activity. Beta-blocking agents prevent this upregulation of sympathetic function. In one study, carvedilol demonstrated improved clinical outcomes in pediatric patients with heart failure; however, only 11% of the patients in that study had undergone a Fontan procedure.39 Currently, there are no large trials that have studied the use of beta-blocking agents in patients with heart failure secondary to failing Fontan procedure.
Adult patients with prior Fontan repair may present to the ED with evidence of hypoxemia or elevated pulmonary vascular resistance, owing to failure of their shunt. Slight hypoxemia, with oxygen saturations in the low 90s, can be seen after successful Fontan procedures. Following the procedure, venovenous collateral blood vessels may generate and decompress the venous return from the upper body into the inferior vena cava. These usually do not lead to hypoxemia. However, if venovenous collaterals drain directly into the left atrium or into the pulmonary vein, they can create mixing and arterial desaturation. Supplemental oxygen therapy is helpful in the ED while awaiting definitive care with specialty consultation.32 If elevated pulmonary vascular resistance is suspected in the patient presenting with excessive hypoxemia or low cardiac output, vasodilator agents such as sildenafil or bosentan may be utilized.40 Since there are currently no clinical studies to support this therapy, it should be used in conjunction with specialty consultation if therapy is started in the ED. Protein losing enteropathy occurs in some patients who have undergone a Fontan repair. The cause is unknown. The patients present with peripheral edema and malabsorption. Patients with severe complications from Fontan repair may be candidates for cardiac transplantation.
Eisenmenger's Syndrome
Eisenmenger's syndrome consists of a large left-to-right shunt which, over time, creates pulmonary vascular disease and pulmonary hypertension. As pulmonary vascular resistance meets or exceeds systemic vascular resistance, it causes a reversal of shunt physiology, from right-to-left. The structural changes of Eisenmenger's syndrome usually begin in early childhood, but patients may be asymptomatic until late childhood or early adolescence.41
When right-to-left shunting develops, patients may become cyanotic. Their symptomatic complaints include exertional dyspnea and palpitations. Due to arterial desaturation, erythrocytosis may result, creating hyperviscosity syndromes including fatigue, headache, dizziness, and visual disturbances. Hemoptysis, secondary to pulmonary infarction, aorticopulmonary vessel collateralization, or dilated pulmonary arteries, may occur.42 Patients with Eisenmenger's syndrome may have syncopal events secondary to arrythmias or inadequate cardiac output. Cerobrovascular accidents can also occur due to embolization, venous thrombosis, or intracranial hemorrhage.
Physical examination is prominent for digital clubbing and cyanosis, low volume arterial pulses, parasternal heave, and loud pulmonary component of the second heart sound (S2). The electrocardiogram shows right ventricular hypertrophy. Atrial arrythmias may also be present. The ED workup of a patient with suspected or known Eisenmenger syndrome should include ECG, chest x-ray, and rapid consultation with cardiology or cardiothoracic surgery. Patients will require Doppler echocardiography, as well as pulmonary function tests. Treatment in the ED should include IV fluids to prevent intravascular volume depletion and supplemental oxygen.41
Rates of survival vary as time from diagnosis elapses from 80% 10 years post-diagnosis to 42% at 25 years post-diagnosis.43 Patients who present with syncope, severe hypoxemia, or clinical signs of low cardiac output have a poor outcome.
Septal Defects
Atrial septal defects (ASD) are seen in about one-third of congenital heart disease cases, and are more commonly found in women. ASD can be found in the form of ostium secundum defects in the fossa ovalis or as ostium primum defects in the lower part of the atrial septum. They may occur with other types of cardiac abnormalities such as mitral valve prolapse (MVP), mitral regurgitation (MR), and anomalous pulmonary venous drainage (usually into the right atrium or the vena cava).44,45
The course of ASD, with shunting between the atria, usually results in substantial physiologic consequences. A sizable defect (greater than 2 cm) may create a large left-to-right shunt, with resultant dilation of the right atrium, right ventricle, and pulmonary arteries. Patients with undetected ASD will often have a wide and fixed split second heart sound. There may also be a systolic murmur and at times a diastolic tricuspid murmur as well. The ECG has a normal to right axis and an rSR pattern may be seen in the right precordial leads. They are generally in a normal sinus rhythm on ECG until physiologic changes develop. This may result in atrial dysrhythmias such as atrial fibrillation or supraventricular tachycardia (SVT). Chest x-ray may show prominence of the pulmonary arteries and pulmonary vascularity.
Young adult patients with previously undiagnosed ASD or patent foramen ovale (PFO) may present with acute onset of stroke-like symptoms, transient ischemic attack (TIA), or recurrent stroke.46,47 Other symptomatic presentations include dyspnea on exertion and fatigue. Patients may also present with a first-time episode of dysrhythmia, either SVT or atrial fibrillation, or with a repeat visit for recurrent pulmonary infection. Initial ED treatment will involve stabilization of the dysrhythmia, if present. SVT can be treated with adenosine for the stable patient, or DC cardioversion if the patient is unstable. Atrial fibrillation in the stable patient should be treated with medications aimed at slowing AV nodal conduction and/or slowing atrial rate. If the patient is unstable, then DC cardioversion with 100 J is recommended. As with other ACHD patients, initial workup will include prompt cardiology consultation. Adult patients with undiagnosed ASD will require further workup including echocardiography to evaluate for possible surgical closure of the defect.
Ventricular septal defect (VSD) is the most common congenital abnormality in infants and children. The majority of VSD are located in the membranous portion of the interventricular septum. Roughly 90% of defects close by age 10.48,49
The size of the VSD and the relative resistance of the pulmonary vasculature determine the long-term physiologic consequences. Initially, when systemic vascular resistance is greater than pulmonary vascular resistance, the shunting is from left-to-right. Over time, especially with a large VSD, pulmonary vascular resistance increases, and left-to-right shunting declines, and may even convert to right-to-left shunting. Patients with small defects may live asymptomatic lives.50
Conversely, patients with large defects will develop pulmonary sequelae and will present to the ED with complaints of dyspnea or with symptoms related to left ventricular failure. Patients with VSD will have a loud, harsh, and often palpable pansystolic murmur at the left lower sternal boarder. The ECG should be normal. Patients with diastolic overload will have prominent Q waves in leads V5, V6, I, and avL. The chest x-ray should be normal after repair. Initial ED treatment is aimed at relieving presenting symptoms, with supplemental oxygen. Adjunctive workup such as ECG and chest x-ray may show evidence of left atrial and ventricular enlargement as well as increased pulmonary vascularity in the symptomatic patient. If pulmonary hypertension is present, a rightward axis will be seen on the ECG, and prominence of pulmonary arteries will manifest on the chest x-ray. Further workup usually requires cardiac catheterization and Doppler echocardiography to evaluate the location and magnitude of the shunt.51
Aortic Stenosis
In patients with documented aortic stenosis, the most common pathologic finding is bicuspid valve. This anomaly is more common in men than women, and is often associated with other cardiac abnormalities such as patent ductus arteriosus (PDA) or aortic coarctation.52 At birth, the abnormal valve does not pose a hemodynamic problem. However, as the valve is exposed to increasing hemodynamic stress, the leaflets become thickened and/or calcified. As the area of stenosis increases to roughly 1.0 cm2 the patient becomes symptomatic.
The common presenting symptoms include chest pain or angina, and syncope or near-syncope. Once symptoms appear, survival is poor, with median survival as low as five years after angina develops. ED workup should include an ECG, chest x-ray, and laboratory panel including complete blood count (CBC) to ascertain for anemia or infection, basic metabolic panel (BMP) to evaluate renal function, and coagulation profile (PTT, PT) to evaluate for possible operative intervention. ECG may reveal left ventricular hypertrophy (LVH), and chest x-ray may show evidence of an enlarged left ventricle. An echocardiogram will facilitate assessment of the aortic valve area. Treatment should be aimed at symptom relief, with supplemental oxygen and IV fluids. It is important to prevent intravascular volume depletion or hypotension, as any decrease in preload can be deleterious for the patient. Administration of nitrates in patients with known or suspected aortic stenosis may result in acute decrease in blood pressure and cardiovascular collapse.53 Symptomatic patients presenting to the ED will require cardiology consultation to further evaluate treatment and surgical options. Patients with aortic stenosis and left ventricular hypertrophy may be at increased risk for cardiac ischemia. Blood flow to the coronaries may be decreased and there may be an increased demand for oxygen due to increased myocardial work.
Aortic Coarctation
The surgical management of coarctation of the aorta was first described in 1945.54,55 Since then, there have been various adaptations and adjustments to the procedure, which is usually performed between ages 2 to 5. The most recent figures show an operative mortality rate of 1% for initial surgical repair of simple aortic coarctation.56
Patients with unrepaired coarctation classically have a difference in blood pressure between the right and left arm or between the arms and legs, depending on the level of the coarctation. After repair the blood pressures should be equal. After repair patients are at risk for hypertension and (see below), because of that, accelerated atherosclerosis. In addition, they have a risk of endocarditis.
With increasing survival into adulthood, the most frequent persistent complication after surgical repair of aortic coarctation is hypertension. This is usually seen in the right arm, and the prevalence of hypertension is related to age at initial repair and length of time after repair at presentation. In one long-term study of post-surgical outcomes, the prevalence of hypertension was nearly 50% at 40 years after surgery.57 Additionally, aneurysms can occur in the descending or ascending aorta. Although these can be seen on magnetic resonance imaging (MRI) or computed tomography (CT) scans, patients with this complication may present in extremis with acute dissection. The goal of ED therapy is rapid heart rate control, blood pressure reduction, and expedient cardiothoracic surgery consultation.
Other Complications in ACHD
Prior to 1992, blood was not routinely screened for hepatitis C virus (HCV). Patients with CHD who had operative procedures requiring blood transfusion prior to 1992 were at increased risk of HCV exposure. A recent study looked at nearly 200 patients in their institution's Adult Congenital Cardiac Program and found roughly 12% of those patients either tested positive for HCV antibody or for HCV ribonucleic acid (RNA).58 This can be a potentially deleterious complication in a population of patients already at increased risk for heart failure. The symptoms of heart failure and liver failure may not be easily distinguished, and so a thorough history and past medical review are especially important in this group of patients.
Due to the invasive nature of corrective cardiac surgery, as well as the complicated pathways that are surgically created, patients with surgically corrected CHD find themselves at increased risk for infective endocarditis (IE). The introduction of antibiotic therapy for IE led to a reduction in mortality from this complication. However, the prevalence of IE is still high in certain populations of CHD patientsspecifically those with chronic hypoxemia, foreign bioprosthetic or synthetic materials used in surgery, repeated interventional maneuvers, and dental work.59
There has been a trend in improved care and longevity of adult patients with repaired CHD. However, for many patients, the real problem is not surgical, but psychosocial. Patients face unique issues such as quality of life, employment factors, exercise participation, and pregnancy. A study in Minnesota evaluated outcomes of pregnancy in women with TOF. These patients tend to have increased risk of fetal loss, and their offspring are more likely to have congenital abnormalities compared with babies in the general population. In addition, pregnancy can be complicated by severe pulmonary hypertension, pulmonic regurgitation with right ventricular dysfunction, or left ventricular dysfunction.60
Many ACHD patients are experts on their own disease. Yet, they want to be healthy, and may often hide symptoms from caregivers because they are afraid of the potential medical consequences.61 It is suggested that patients with more complex disease may have problems with emotional functioning.62 It is important for healthcare providers to recognize these potential psychosocial impact factors, and to provide the appropriate referral to patients.
Summary
Adolescents and adults with CHD already pose an interesting challenge to cardiologists and cardiothoracic surgeons.63 Owing to surgical advances and innovative procedures, surgical correction of congenital cardiac abnormalities creates positive outcomes for patients. As they survive into adulthood, they will become a population that is seen with increasing frequency in emergency departments.
References
1. Shinebourne EA, Gatzoulis MA. Adult congenital heart disease. Curr Paed 2002;12:220-226.
2. Curtis SL, Stuart AG. Outcome in congenital heart disease. Curr Paed 2005;15:549-556.
3. Warnes CA, Liberthson R, Danielson GK, et al. Task force 1: The changing profile of congenital heart disease in adult life. J Am Coll Cardiol 2001;37:1170-1175.
4. Marelli AJ, Mackie AS, Ionescu-Ittu R, et al. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation 2007;115:163-172.
5. Warnes CA. The adult with congenital heart disease: Born to be bad? J Am Coll Cardiol 2005;46:1-8.
6. Spray TL. Congenital heart surgery in adults: Introduction. Thoracic Cardiovasc Surg 2005;17:138.
7. Perloff JK, Warnes CA. Challenges posed by adults with repaired congenital heart disease. Circulation 2001;103: 2637-2643.
8. Capelli H. Grown-up congenital heart disease: The problem of late arrhythmia and ventricular dysfunction. Prog Ped Cardiol 2006;22:165-173.
9. Kaemmerer H, Bauer U, Pensl U, et al. Management of emergencies in adults with congenital heart disease. Am J Cardiol 2007 (accepted for publication Sep 2007).
10. Blalock A, Taussig H. The surgical treatment of malformations of the heart. JAMA 1945;128:189-202.
11. Brock RC, Campbell M. Infundibular resection or dilatation for infundibular stenosis. Br Heart J 1950;12:403-424.
12. Kirklin JW, Payne WS. Surgical treatment for tetralogy of Fallot after previous anastamosis of systemic to pulmonary artery. Surg Gynecol Obstet 1960;110:707-713.
13. Bernstein D. Cyanotic congenital heart lesions: Lesions associated with decreased pulmonary blood flow. In: Behrman RE, et al, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia: Saunders; 2004: 1524-1528.
14. Gatzoulis MA, Balaji S, Webber SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: A multi-center study. Lancet 2000;356: 975-981.
15. Harrison DA, Harris L, Siu SC, et al. Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot. J Am Coll Cardiol 1997;30:1368-1373.
16. Redington AM, Oldershaw PJ, Shinebourne EA, et al. A new technique for assessment of pulmonary regurgitation and its application to the assessment of right ventricular function before and after repair of tetralogy of Fallot. Br Heart J 1988;60:57-65.
17. Yemets IM, Williams WJ, Webb GD, et al. Pulmonary valve replacement late after repair of tetralogy of Fallot. Ann Thorac Surg 1997;64:526-530.
18. Geva D, Sandweiss BM, Gauvreau K, et al. Factors associated with impaired clinical status in long term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imagining. J Am Coll Cardiol 2004;43: 1068-1174.
19. Senning A. Surgical correction of transposition of the great vessels. Surgery 1959;45:966-980.
20. Mustard WT, Keith JD, Trusler GA, et al. The surgical management of transposition of the great vessels. J Thorac Cardiovasc Surg 1964;48:953-958.
21. Shaddy RE, Webb G. Applying heart failure guidelines to adult congenital heart disease patients. Exp Rev Cardiovasc Ther 2008;6:165-174.
22. Lester SJ, McElhinney DB, Viloria B, et al. Effects of losartan in patients with a systemically functioning morphologic right ventricle after arterial repair of transposition of the great arteries. Am J Cardiol 2001;88:1314-1316.
23. Jatene AD, Fontes VF, Paulista PP, et al. Successful anatomic correction of transposition of the great vessels. A preliminary report. Arq Brasil de Cardiol 1975;28:461-464.
24. Yacoub MH, Radley-Smith R, Maclaurin R. Two-stage operation for anatomical correction of transposition of the great arteries with intact interventricular septum. The Lancet 1977; 309:1275-1278.
25. Flinn CJ, Wolff GS, Dick M, et al. Cardiac rhythm after the Mustard operation for complete transposition of the great arteries N Engl J Med 1984;3310:1635-1638.
26. Walsh EP, Cecchin F. Arrythmias in adult patients with congenital heart disease.Circulation 2007;115:534-545.
27. Gregoratos G, Abrams J, Epstein AE, et al. ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices. J Am Coll Cardiol 2002;40:1703-1719.
28. Triedman JK. Atrial reentrant tachycardias. In: Walsh EP, Saul JP, Triedman JK, eds. Cardiac Arrythmias in Children and Young Adults with Congenital Heart Disease. Philadelphia, Pa: Lippincott Williams & Wilkins;2001: 137-160.
29. Li W, Somerville J. Atrial flutter in grown-up congenital heart (GUCH) patients: Clinical characteristics of affected population. Int J Cardiol 2000;75:129-137.
30. Stephenson EA, Casavant D, Tuzi J, et al; ATTEST Investigators. Effect of atrial antitachycardia pacing using the Medtronic AT500 pacemaker in patients with congenital heart disease. Am J Cardiol 2003;92:871-876.
31. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240-248.
32. Ghanayem NS, Berger S, Tweddell JS. Medical management of the failing Fontan. Pediatr Cardiol 2007;28:65-471.
33. Khairy P, Fernandes SM, Mayer JE, et al. Long-term survival, modes of death and predictors of mortality in patients with Fontan surgery. Circulation 2008;117:85-92.
34. Fishberger SB, Wernovsky G, Gentles TL et al. Factors that influence the development of atrial flutter after the Fontan operation. J Thorac Cardiovasc Surg 1997;113: 80-86.
35. Weipert J, Noebauer C, Schreiber C, et al. Occurrence and management of atrial arrhythmia after long-term Fontan circulation. J Thorac Cardiovasc Surg 2004;127:457-464.
36. Hoffman TM, Wernovsky G, ATz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003;107:996-1002.
37. Hjortdal VE, Stenberg EV, Ravn HB, et al. Neurohumoral activation late after cavopulmonary connection. Heart 2000; 83:439-443.
38. Stewart JM, Gewitz MH, Clark BJ, et al. The role of vasopressin and atrial natriuretic factor in postoperative fluid retention after the Fontan procedure. Anesth Analg 1996;102: 821-829.
39. Bruns LA, Chrisant MK, Lamour JM, et al. Carvedilol as therapy in pediatric heart failure: an initial multicenter experience. J Pediat. 2001;138:505-511.
40. Levy M, Danel C, Laval A, et al. Nitric oxide synthase expression by pulmonary arteries: A predictive marker of Fontan procedure outcome? J Thorac Cardiovasc Surg 2003; 125:1083-1090.
41. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med 2000;342: 334-342.
42. Henrikkson P, Varendh G, Lundstron NR. Haemostatic defects in cyanotic congenital heart disease. Br Heart J 1979;41:23-27.
43. Young D, Mark H. Fate of the patient with the Eisenmenger syndrome. Am J Cardiol 1971:28;658-659.
44. Campbell M. Natural history of atrial septal defect. Br Heart J 1970;32:820-826.
45. Leachman RD, Cokkinos DV, Cooley DA. Association of ostium secundum atrial septal defects with mitral valve prolapse. Am J Cardiol 1976;38:167-169.
46. Wechsler LR. PFO and stroke: What are the data? Card Rev 2008;16:53-57.
47. Rigatelli G, Giordan M, Braggion G, et al. Incidence of extracerebral paradoxical embolism in patients with intracardiac shunts. Card Rev Med 2007;8:248-250.
48. Weidman WH, Dushane JW, Ellison RC. Clinical course in adults with ventricular septal defect. Circulation 1977;56:S1 78-79.
49. Perloff JK. Survival patterns without cardiac surgery or interventional catheterization: a narrowing base. In: Perloff JK, Childs JS, eds. Congenital Heart Disease in Adults. 2nd ed. Philadelphia: W.B. Saunders, 1998:15-53.
50. Neumayer U, Stone S, Somerville J. Small ventricular septal defects in adults. Eur Heart J 1998:19;1573-1582.
51. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med 2000;342: 256-263.
52. Subramaniam R, Olson LJ, Edwards WD. Surgical pathology of pure aortic stenosis: A study of 374 cases. Mayo Clin Proc 1984:59;683-690.
53. Reeder GS. Nitrates in the management of acute coronary syndrome. 2007. UpToDate Online. http://patients.uptodate.com/topic.asp?file=chd/15958.
54. Gross RE, Hufnagel CA. Coarctation of the aorta, experimental study regarding its surgical correction. N Engl J Med 1945;233:287-293.
55. Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg 1945;14:347-361.
56. Rothman A. Coarctation of the aorta. Curr Prob Pediatr 1998;28:37-60.
57. Toro-Salazar OH, Steinberger J, Thomas W, et al. Long-term follow-up of patients after coarctation of the aorta repair. Am J Cardiol 2002;89:541-547.
58. Wang A, Book WM, McConnell M, et al. Prevalence of hepatitis C infection in adult patients who underwent congenital heart surgery prior to screening in 1992. Am J Cardiol 2007; 100:1307-1309.
59. Knirsch W, Haas NA, Uhlemann F, et al. Clinical course and complications of infective endocarditis in patients growing up with congenital heart disease. Int J Cardio. 2005;101: 285-291.
60. Veldtman GR, Connolly HM, Grogan M, et al. Outcomes of pregnancy in women with tetralogy of Fallot. J Am Coll Card 2004;44:174-180.
61. Berghammer M, Dellborg M, Ekman I. Young adults experiences of living with congenital heart disease. Int J Cardiol 2006;110:340-347.
62. Bromberg JI, Beasly PJ, D'Angelo EJ, et al. Depression and anxiety in adults with congenital heart disease: A pilot study. Heart Lung 2003;32:105-110.
63. Daebritz S. Update in adult congenital cardiac surgery. Pediatr Cardiol 2007;28:96-104.
There has been recent emphasis in cardiology literature highlighting the importance of recognizing a new sub-specialty within the field, aimed at treating adult patients who have survived after having congenital heart disease repair.You have reached your article limit for the month. Subscribe now to access this article plus other member-only content.
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