Bilateral lung transplantation with closure of ventricular septal defect in a patient with Eisenmenger syndrome

We report the first case with Eisenmenger syndrome secondary to an isolated perimembranous ventricular septal defect (VSD) in a patient who underwent bilateral lung transplantation and closure of the VSD in Japan. This male patient was diagnosed as having a VSD associated with severe pulmonary hypertension at the age of 7, and right unilateral pulmonary artery banding was performed at age 9. At 38 years of age, we performed bilateral cadaveric lung transplantation with patch closure of the VSD. Explant pathology revealed grade 3 for the right lung and grade 4 for the left lung by the Heath-Edwards classification. The ventricular contractility had gradually improved, and ventricular arrhythmia requiring amiodarone prior to lung transplantation had disappeared. When cardiac function is preserved, bilateral lung transplantation is an option for patients with Eisenmenger syndrome secondary to VSD and should be considered as an alternative to heart-lung transplantation, especially in Japan, where donor organ shortage is critical.