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The number of adults with congenital heart disease (CHD) living in the United States is estimated to be at least 1.4 million, with just over one in five having a complex form of CHD. The majority of adults with CHD were diagnosed in childhood, although a substantial percentage may have CHD first recognized as adults. Lifelong follow-up in coordination with, or directly by, clinicians with expertise in adult congenital heart disease (ACHD) is recommended. In this chapter, we will review the current field of ACHD, with an introduction to CHD nomenclature and cardiac development. This is followed by a summary of the more common CHD lesions that may be diagnosed in adulthood. Lastly, some of the common repaired CHD lesions that are encountered in adults are discussed. Throughout the chapter, to aid in the understanding of congenital cardiac anatomy and physiology, we include figures displaying the passage of blood flow between blood vessels and cardiac chambers in various disorders (Fig. 264-1).

Figure 264-1

Normal heart. Understanding of congenital cardiac anatomy and physiology is facilitated by use of box diagrams, displaying passage of blood flow between blood vessels and cardiac chambers. Labeling (e.g., structure names, arrows to denote direction of flow, coloring to represent oxygen saturation, connections or obstructions, chamber or vascular pressures, oxygen saturations) can aid in representation. Ao, aorta; IVC, inferior vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PV, pulmonary veins; RA, right atrium; RV, right ventricle; SVC, superior vena cava.


A Relatively New Subspecialty in Cardiovascular Disease

Over the past decade, the field of caring for adults with CHD (ACHD) has blossomed, and several nationwide initiatives have been initiated in an attempt to standardize care. The American College of Cardiology and American Heart Association developed guidelines for the care of adults with CHD, first published in 2008. These guidelines emphasize the need for collaboration among primary care practitioners, cardiologists, and ACHD subspecialty cardiologists. The body of medical knowledge and competencies attendant with ACHD combined with skill acquisition in coordination of complex care over a patient’s medical lifetime led in 2015 to both ACHD board certification examinations by the American Board of Medical Subspecialties, as well as the establishment of requirements for 2-year subspecialty fellowship training in ACHD care, by the Accreditation Council for Graduate Medical Education. In temporal association, the Adult Congenital Heart Association (ACHA) developed a process for ACHD care program accreditation based upon standardization of infrastructural components felt requisite to achieve quality outcomes for ACHD.


Adults with CHD may not recognize subtle changes in their exercise capacity, some of which are associated with worse survival; by the time symptoms are recognized, irreversible physiological changes may have ...

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