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Congenital cardiac anomalies are the most common birth defects in humans, affecting approximately 0.7 in 100 live births. While the incidence of congenital heart disease is expected to decline as a consequence of improved prenatal diagnosis, the number of patients surviving with congenital heart disease, both in the United States and worldwide, has increased significantly over the past three decades. Over 85% of infants born with cardiovascular anomalies now can expect to reach adulthood. Reduced mortality rates can be attributed to improved diagnostic abilities, enhanced surgical and nonsurgical therapies, and improvements in intensive care. For the first time, the number of adults with congenital heart disease exceeds the number of children with the disorder.


The increase in the number of adults with congenital heart disease requires that the physician managing the care of these patients have improved knowledge of simple and complex anatomy and physiology. Although actual numbers are difficult to ascertain, it has been estimated that approximately 1 million adults in the United States alone currently have congenital heart disease and that the number of patients reaching adulthood with treated congenital heart disease will increase by approximately 9000 per year.


These patients fall into several broad categories: those surviving into adulthood without intervention, those surviving with curative surgical or nonsurgical intervention, and those surviving with palliative surgical or nonsurgical intervention. Nonsurgical interventions may include catheter-based valvuloplasty, stenting, coiling, or device occlusion. The patients who are today making the transition into the adult congenital heart disease population have hemodynamic and cardiac problems differing from those in previous eras. Surgical techniques have evolved, intervention occurs earlier and is often definitive rather than palliative, and a greater number of patients with complex single-ventricle physiology and various modifications of cavopulmonary anastomoses (Glenn shunt, Fontan procedure) will reach adulthood.


Although many patients are referred by pediatric cardiologists, others may seek medical attention for the first time in adulthood. Examples of cardiac pathology that may not be readily apparent in childhood include secundum atrial septal defects (ASD), coarctation of the aorta, Ebstein anomaly of the tricuspid valve, congenitally corrected transposition (ventricular inversion), and coronary artery anomalies.


The risk of infective endocarditis remains an issue of ongoing concern in many patients with congenital cardiac defects. The absolute magnitude of risk varies considerably from one lesion to another and is also dependent on whether the patient has been surgically treated. In 2007, the American Heart Association revised its guidelines regarding infective endocarditis prophylaxis. The revised guidelines suggest a much more conservative approach to the use of prophylactic antibiotics. Broadly speaking, infective endocarditis prophylaxis is now recommended in only three groups of patients with congenital heart disease: (1) those with unrepaired cyanotic congenital heart disease, including patients with palliative shunts and conduits, (2) those with a defect completely repaired (either surgically or by catheter-based intervention) using prosthetic material or device, during the first 6 months after the procedure, and (3) those with repaired ...

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