Pulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD). In patients with ASD, PAH and right ventricle failure, heart-lung transplantation (HLT) is currently viewed as the best ultimate procedure but is limited by the low number of grafts. We aim to report our preliminary experience of double lung transplantation (DLT) followed by percutaneous ostium secundum ASD closure in patients with end-stage ASD associated PAH.

We report 6 patients with end-stage ASD associated PAH treated with DLT followed by percutaneous ASD closure.

Median ages at ASD diagnosis and PAH diagnosis were respectively 22 y.o (min 0, max 37) and 16 (min 2 max 40). Median mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance before DLT were 78mmHg (min 49, max108) and 14.0WU.m² (min 12.5, max 17.4) respectively. Median age at DLT was 29 y.o (min 20, max 44). Severe primary graft dysfunction occurred in 3 (50%) patients and was successfully managed medically. Post operative extracorporeal membrane oxygenation was required in 5 (83%) patients. Median ventilation time was 12 days (min 2, max 20). Median delay between DLT and percutaneous ASD closure occlusion was 4.4 months (min 3.9 max 18.7). Percutaneous closure was achieved using Amplatzer septal occluder, size running from n°16 to 40. mPAP was within normal range in all patient before ASD closure. All patient had improvement of their respiratory functional tests. All patients had improvement of right ventricular function (Fig. 1) and none of them developed any chronic lung allograft dysfunction. Median follow up after lung transplant was 397 months (min: 265; max: 562). One patient died of pulmonary infection complication at 14.5 months after lung transplant.

DLT followed by percutaneous ASD closure is an efficient therapeutic approach in patients with end-stage ASD associated PAH that may offer an alternative option to HLT.