Congenital Heart Disease

Congenital heart disease (CHD) refers to a wide range of structural defects of the heart and major blood vessels that are present at birth. Successes in repairing and managing even highly complex CHD have created a large, ever-growing, and heterogeneous population of adults with congenital heart disease (ACHD). The majority of patients with CHD are now adults. Several themes commonly arise when caring for ACHD regardless of the age or physical condition of the patient. These include screening for and management of hypertension, hyperlipidemia, and diabetes; perioperative management for non-cardiac surgeries; counseling around physical activity and employment; sexual and reproductive health; genetic counseling; and antibiotic prophylaxis for infective endocarditis. While complications directly related to CHD should be managed in conjunction with an ACHD specialist, primary care physicians (PCPs) should be comfortable managing the above scenarios in most ACHD and should be able to recognize when co-management with an ACHD specialist is indicated. This chapter presents a patient-centered primary care approach to ACHD that takes into account the presence of any associated syndrome or chromosomal abnormality, the complexity of the underlying CHD lesion, and the chronological and developmental age of the patient.