Lifelong Management of Right Ventricular Outflow Tract Dysfunction in Adults with Congenital Heart Disease

Right ventricular outflow tract (RVOT) dysfunction is a common and clinically significant late complication in adults with congenital heart disease (ACHD), often after repair of conotruncal anomalies, Ross intervention, or isolated pulmonary valve disease. Pulmonary regurgitation, stenosis, or mixed lesions can result in RV dilation, dysfunction, arrhythmias, and exercise limitation. RVOT dysfunction often requires either surgical or transcatheter intervention, with careful patient selection, imaging, and planning. Surgical pulmonary valve replacement remains the reference standard for complex anatomies, whereas transcatheter pulmonary valve replacement offers a less invasive, repeatable solution in suitable conduits, bioprostheses, and increasingly often in patched RVOTs. Long-term outcomes have improved with advances in imaging, device technology, and perioperative care; however, complications such as valve degeneration, infective endocarditis, and arrhythmias persist. This review provides a comprehensive synthesis of epidemiology, pathophysiology, indications, surgical and transcatheter management strategies, and lifelong complications after RVOT reintervention in ACHD patients.