Background: Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about optimal perioperative management and midterm clinical outcomes. Methods: Prospective observational study on consecutive children with PAH who underwent LuTx with scheduled postoperative VA-ECMO support at Hannover Medical School from December 2013 to June 2020.Results: Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9-17.8). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH associated with congenital heart disease (n = 2), pulmonary veno-occlusive disease (n = 1), and pulmonary capillary hemangiomatosis (n = 1). The mean waiting time was 58.5 days (range 1-220d). Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO/cardiopulmonary bypass was applied and VA-ECMO was continued postoperatively in all patients (mean ECMO-duration 185 h; range 73-363 h; early extubation). The median postoperative ventilation time was 28 h (range 17-145 h). Echocardiographic conventional and strain analysis showed that 12 months after LuTx, all patients had normal biventricular systolic function. All PAH patients are alive 2 years after LuTx (median follow-up 53 months, range 26-104 months).Abbreviations CLAD, chronic lung allograft dysfunction; CPB, cardiopulmonary bypass; FEV1, forced expiratory volume in one second; IPAH/HPAH, idiopathic/heritable pulmonary arterial hypertension; LuTx, lung transplantation; LV, left ventricle; LVLS, left ventricular longitudinal strain; PAH-CHD, pulmonary arterial hypertension associated with congenital heart disease; PVD, pulmonary vascular disease; PVOD/PCH, pulmonary venoocclusive disease/pulmonary capillary hemangiomatosis; RV, right ventricle; RVAWD, right ventricular anterior wall diameter (in diastole); RVEDD, right ventricular end-diastolic diameter; RVH, right ventricular hypertrophy; RV/LV end-systolic ratio; ratios of inner diameters of RV over LV in end-systole; RVEF, right ventricular ejection fraction; RVFWLS, right ventricular free wall longitudinal strain; RVFWSR, right ventricular free wall longitudinal strain rate; RVGLS, right ventricular global longitudinal strain; RVGLSR, right ventricular global longitudinal strain rate; VA-ECMO, veno-arterial extracorporeal membrane oxygenation.
