Congenital heart disease (CHD) is the most common birth defect with an incidence ranging from 4 to 50/1000 live births, a range that is dependent on definitions and global variation.¹ With improved medical, surgical, and intensive care, over the past several decades, the survival to adulthood has exponentially improved. It is now expected that greater than 90% of children born with CHD will survive to adulthood (Figure 1-1). Consequently, in the United States, there are now greater than 1 million adults with congenital heart disease (ACHD), with a 5% increase or 20,000 new patients reaching adulthood each year (Figure 1-2). In fact, it is estimated that for the first time in history, there has been a shift in the CHD population (Figures 1-3A, 1-3B, and 1-3C) with more adults living with CHD than children (Figure 1-4)².

With the rise in the ACHD population, there has been a similar surge in clinics caring for this unique and complex population (Figure 1-5), as well as fellowship programs to train cardiologists in the field of ACHD. In the United States and around the world, there is a focused and directed effort to create comprehensive ACHD centers and establish training guidelines for cardiologists. This process was well outlined in 2000 at the American College of Cardiology 32nd Bethesda Conference and published in 2001.³ There are now established training guidelines, to become an ACHD specialist. Cardiologists have to spend two extra years training in ACHD after finishing either their general pediatric or adult cardiology fellowships.

After several years of deliberation, the first sub-specialty board exam for Adult Congenital Heart Disease will be administered in the United States in 2015 by the American Board of Internal Medicine (ABIM) and the American Board of Medical Specialties (ABMS).

LONG-TERM OUTCOMES FOR ACHD

As epidemiologic data sets clearly define the rise and continual growth of the ACHD population, at the same time, outcome data reported a more dismal outlook for the same CHD patients reaching adulthood describing early mortality and substantial morbidity.

The explanation for the outcome data is rooted in the natural history of unrepaired and repaired congenital heart defects. It is well established that many patients who have undergone total corrective surgery for mild forms of CHD with no risk factors or postoperative complications will have few, if any, hemodynamic residua (eg, atrial septal defect, ventricular septal defect, patent ductus arteriosus, mild pulmonary stenosis).