Heart and Lung Transplant in Patient with PAPVR

Heart-lung transplantation is a life-saving procedure for select patients with complex congenital heart disease and end-stage pulmonary vascular disease, such as those with partial anomalous pulmonary venous connection (PAPVC). PAPVC is a congenital anomaly in which one or more pulmonary veins drain into the systemic venous circulation instead of the left atrium, often leading to chronic left-to-right shunting, right heart volume overload, and progressive pulmonary hypertension. In patients with longstanding undiagnosed or unrepaired PAPVC, the combination of severe pulmonary vascular remodeling and right ventricular dysfunction may render them ineligible for isolated lung transplantation due to the irreversible cardiac involvement. In such cases, heart-lung transplantation becomes the preferred therapeutic option, as it simultaneously addresses the anatomical defect, the failing right ventricle, and the diseased pulmonary vasculature. Surgical success depends on meticulous preoperative imaging, careful management of cardiopulmonary bypass during explantation and implantation, and coordinated postoperative care. This approach offers selected patients with complex congenital and acquired cardiopulmonary disease the best chance for extended survival and improved quality of life.