Combined lung transplantation and percutaneous septal defect closure for end-stage atrial septal defect associated pulmonary arterial hypertension

Abstract

IntroductionPulmonary 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. MethodsWe report 6 patients with end-stage ASD associated PAH treated with DLT followed by percutaneous ASD closure. ResultsMedian ages at ASD diagnosis and PAH diagnosis were respectively 22 (min:0, max: 37) and 16 (min: 2 max: 40) year-old. Median mean pulmonary artery pressure and pulmonary vascular resistance before DLT were 78mmHg (min: 49, max: 108) and 14.0 WU.m2 (min: 12.5, max: 17.4) respectively.Median age at DLT was 29 (min 20, max 44) year-old. 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). Transient tracheotomy was required in 2 patients. Median time of stay in intensive care unit was 15 days (min:7, max: 26).Median delay between DLT and percutaneous ASD closure occlusion delay was 4.4 months (min: 3.9 max: 18.7). Percutaneous closure was achieved using Amplatzer septal occluder in all patients (size running from n° 16 to 40). Mean pulmonary arterial pressure was within normal range in all patient before ASD closure. All patient had improvement of their respiratory functional tests. All patients had improvement of there 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. ConclusionDLT 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.