Pathophysiology and management of adults with complex congenital heart disease after biventricular repair

Surgical management of congenital heart disease encompasses a spectrum of procedures, ranging from biventricular repair to univentricular palliation, each tailored to the specific anatomical and hemodynamic features of individual cases. Among these, biventricular repair, which preserves a functional ventricle to sustain pulmonary circulation, is prioritized whenever feasible. Advances in approaches have significantly improved outcomes, enabling many patients with congenital heart disease to reach adulthood, including the majority who have undergone biventricular repair. Despite these advancements, long-term complications—such as valvular disease, arrhythmias, heart failure, outflow tract obstruction, and dysfunction of extracardiac conduits—pose persistent challenges in the lifelong care of these patients. This review examines the distinct challenges and management strategies associated with adult patients who have undergone biventricular repair for complex congenital heart disease. The discussion focuses on key conditions, including repaired tetralogy of Fallot, transposition of the great arteries following atrial or arterial switch procedures, surgically managed or untreated congenitally corrected transposition of the great arteries with significant tricuspid regurgitation necessitating intervention, pulmonary atresia with intact ventricular septum, and Ebstein's anomaly. By addressing the long-term complications and therapeutic considerations unique to this patient population, this review aims to provide a comprehensive framework for optimizing care as these individuals transition into adulthood.