Valvular heart disease in congenital heart disease: a narrative review

Patients with congenital heart disease (CHD) are one of the fastest growing populations in cardiology, and valvular pathology is at the center of many congenital lesions. Derangements in valvular embryology lead to several anomalies prone to dysfunction, each with hemodynamic effects that require appropriate surveillance and management. Surgical innovation has provided new treatments that have improved survival in this population, though has also contributed to esotericism in patients who already have unique anatomic and physiologic considerations. Conduit and prosthesis durability are often monitored collaboratively with general and specialized congenital-focused cardiologists. As such, general cardiologists must become familiar with valvular disease with CHD for appropriate care and referral practices. In this review, we summarize the embryology of the semilunar and atrioventricular (AV) valves as a foundation for understanding the origins of valvular CHD and describe the mechanisms that account for heterogeneity in disease. We then highlight the categories of pathology from the simple (e.g., bicuspid aortic valve, isolated pulmonic stenosis) to the more complex (e.g., Ebstein's anomaly, AV valvular disease in single ventricle circulations) with details on natural history, diagnosis, and contemporary therapeutic approaches. Care for CHD patients requires collaborative effort between providers, both CHD-specialized and not, to achieve optimal patient outcomes.