Living-related lobar transplantation and simultaneous atrial septal defect closure in a young patient with irreversible pulmonary hypertension: a case report

Abstract

A 6-year-old boy was diagnosed as having atrial septal defect with oversystemic pulmonary hypertension, and gradually developed hypoxia and heart failure. At the age of 11, living-related bilateral lobar lung transplantation from his parents was indicated, because of his critical condition. The estimated forced vital capacity calculated by the donors’ lower lobes was 104% of his age. The operation was carried out with simultaneous closure of the atrial septal defect under full cardiopulmonary bypass. The postoperative course was complicated with pulmonary edema and phrenic nerve palsy, which were eventually resolved. Six months after surgery he was free from heart failure and rehabilitating at home without oxygen. The final diagnosis was primary pulmonary hypertension with atrial septal defect. Living-related bilateral lobar lung transplantation with simultaneous intracardiac repair may be an optional strategy for children with Eisenmenger syndrome or primary pulmonary hypertension with intracardiac defect when cadaveric transplantation is not possible.