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Congenital heart disease (CHD) is a term used to describe a malformation of the heart or great vessels present since birth. In adults with congenital heart disease (ACHD), one must consider not only the original CHD anatomy, but also the type of surgical repair(s), as well as understand the natural history of both the underlying anatomy and surgical palliation.

The overall profile of CHD in the United States has shifted strikingly in the last decade whereby there has been an increase in those living with CHD, and now greater proportion of adults versus children (Figure 17-1). This observation foreshadows the future growth of the ACHD population in the United States. In fact, by current estimates, the number of adults living with CHD is substantial. Canadian data reported that 40,000 ACHD patients comprised 66% of CHD care nationally in Canada in 2010 (Figure 17-2).2 If these data are extrapolated and applied to the U.S. population, the number of potential ACHD patients seeking care in 2010 is on the order of 1.5 million. Not only have overall numbers of ACHD patients increased, but there has also been a marked jump in adults with severe forms of CHD. In adults the overall prevalence of severe CHD increased 85% versus 22% for children.3 For the first time ever, data were released in late 2014 showing that patients who received their care at a specialized ACHD center had an independent reduction in mortality.4 This shift in mortality benefit occurred after the release of national consensus guidelines, indicating that both expert opinion and evidence-based medicine in ACHD had an impact on overall survival. With increasing numbers of adults surviving CHD, there is a substantial need for better understanding of CHD in the adult population as they continue to age.5 Ultimately, this calls for better understanding and recognition of CHD, surgical repair(s), and late sequelae related to underlying anatomy and palliation. Here we review the most common ACHD lesions that present to adult cardiology clinics, along with exam findings, special testing, and considerations for expected management in the setting of underlying anatomy and prior surgical repair. Several practice management guidelines are outlined as part of this review.6-11

Figure 17-1

Proportion of adults with congenital heart disease. The adult population with congenital heart disease is expanding at a greater rate than the pediatric population with congenital heart disease. Proportion of adults with congenital heart disease as compared to children with congenital heart disease in 2000 (A) and in 2010 (B). (Data from Williams RG, Person GD, Barst RJ, et al. Report of the national heart blood lung institute working group on research in adult congenital heart disease. J Am Coll Cardiol. 2006;47:701-707.)

Figure 17-2

Number of people living with congenital heart disease. Data from ...

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