Adult congenital heart disease--challenges and opportunities for pathologists

Adults living with congenital heart disease (CHD) are numerous, at a prevalence of about four per 1000 population. Many of these patients are now over the age of 40 years. Typically, these adult CHD patients have had or require one or more invasive interventions, and thus most of them have modified anatomy and physiology. Despite professional education programs aimed at cardiologists, surgeons, radiologists, pathologists, and others to improve awareness of "grown-up kids" with CHD, there is still a paucity of caregivers who feel competent with these patients. An appreciation by pathologists of the main types of clinically modified congenital lesions seen in adult patients with CHD is of great importance in terms of helping clinicians understand why patients ultimately die (often prematurely) and in explaining the dysfunction and deterioration of biomaterials and devices required for care. Among the most common lesions observed in adults, atrial and ventricular septal defects, patent ductus arteriosus, coarctation of the aorta, tetralogy of Fallot, pulmonary atresia, transposition of the great arteries, single ventricle and Fontan circulation, and Ebstein anomaly are especially worthy of mention. General considerations needing attention for patients with adult CHD include tracking and follow-up, pregnancy guidance and counseling, noncardiac surgery risks, thrombosis, pulmonary hypertension, endocarditis risk, and arrhythmia management. Pathologists may contribute to supporting the families who are affected. As well, many of the adult patients with CHD now live long enough to develop atherosclerosis and all of its attendant morbidities and pathologies.