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Congenital heart disease (CHD) remains the most common birth defect and leading cause of birth defect–related infant mortality. Nearly 40,000 infants in the United States are born each year with CHDs. With increasing medical treatment options, survival is improving with more than 90% of these patients living into adulthood, resulting in a growing population of adults with CHD (ACHD). There are currently more than 1.3 million adults with CHD and this prevalence is expected to increase 5% annually. These patients frequently develop complications characteristic of the defect or its treatment. Consequently, adult cardiologists participating in the care of these patients need a working knowledge of the more common defects. Fewer than 10% of ACHD in the United States are receiving recommended specialized care for their CHD.

Occasionally, some patients with congenital heart defects such as atrial septal defect, Ebstein's anomaly, or physiologically corrected transposition of the great arteries present for the first time in adulthood, which often creates diagnostic and therapeutic dilemmas that may be difficult to manage later in life. Adults with moderate or complex CHD are at higher risk of long-term complications, morbidity, and early mortality. More often patients previously treated in pediatric cardiology centers have transitioned to Adult Congenital Heart Disease centers for ongoing care. Some of the more important defects in this category are tetralogy of Fallot, transposition of the great arteries, functionally single ventricle defects, and coarctation of the aorta. There is a higher than expected burden of sudden cardiac death in CHD patients once reaching adulthood—with many cases occurring 20 years or more after initial surgical repair. CHD represents a medical challenge that spans the life of a patient from birth through adolescence and into adulthood, including pregnant women with CHD. This includes an increasing number of women with CHD who are in their childbearing years. Between 1998 and 2007, the number of annual deliveries in women with CHD rose by 35% as compared with a 21% increase in women with no CHD.

Through this field guide, we provide an overview of selected defects commonly seen in an adult congenital cardiology practice using clinical cases, imaging studies, and pathology specimens to provide a brief overview of the anatomy, physiology, clinical presentation, common complications, treatment options, and long-term outcomes for adults with congenital heart disease. This Atlas is meant for the general audience including internists, pediatricians, general practitioners, nurse practitioners, physician assistants, and general cardiologists. We would like to offer our heartfelt gratitude to all the authors who contributed in the preparation of this Color Atlas.

Curt J. Daniels, MD, and Ali N. Zaidi, MD

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