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Background Profound pulmonary arterial hypertension with end-stage right heart failure is considered to be the main cause of death in children with un-repaired congenital heart disease, and the traditional surgical treatment is heart–lung transplantation. We performed bilateral lung transplantation (LTx) with concomitant cardiac repair, and the patient has uplifting outcome. Case presentation We have reported the case of a patient with congenital hypoplasia of the bilateral pulmonary arteries and patent ductus arteriosus. The patient’s clinical condition was gradually worsening and severely limited his ability to perform the activities of daily life. Bilateral LTx with concomitant patent ductus arteriosus repair was performed at the age of 11 years. The postoperative course was smooth and cardiopulmonary function nearly returned to normal according to radiological and laboratory examinations. Conclusions Bilateral LTx with concomitant cardiac repair may be superior to heart–lung transplantation in the case of the specific congenital heart disease.

Lung transplantation with concomitant cardiac repair for congenital hypoplasia of bilateral pulmonary arteries and patent ductus arteriosus