Bilateral Lung Transplantation for Pediatric Pulmonary Arterial Hypertension: Perioperative Management and One-Year Follow-up

Background: Bilateral lung transplantation (LuTx) remains the only treatment option for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about the optimal perioperative management and clinical outcome.

Method: Prospective observational study on 12 consecutive children with PAH who underwent LuTx with scheduled postoperative ECMO support at Hannover Medical School from December 2013 to June 2020.

Results: Twelve PAH patients underwent LuTx (mean age: 11.9 years; range: 1.9–17.8 years; 9/12 female). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH after surgical shunt closure in 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: 1–220 days). Preoperative echocardiography showed imminent (n = 9) or acute right heart failure (n = 3), systemic/suprasystemic right ventricular (RV) pressure (n = 12), systolic RV dysfunction (n = 12), end-systolic septal shift with left ventricular (LV) compression (n = 12), and pericardial effusion in different degrees. Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO or cardiopulmonary bypass was applied and awake VA-ECMO was continued postoperatively in all patients (mean duration: 185 hours; range: 73–363 hours), allowing early extubation. The median postoperative ventilation time was 28 hours (range: 17–145 hours). As of September 1, 2022, all patients were alive (median survival: 53 months, range: 26–104 months). Pulmonary function remained stable in 10/12 patients, one patient fulfills criteria for chronic lung allograft dysfunction (CLAD) 1 and one patient underwent successful retransplantation for CLAD3 32 months after initial LuTx. Twelve months after LuTx, all patients showed normal biventricular systolic function by means of echocardiographic 2D-B-mode and -strain/strain rate analysis.

Conclusion: LuTx in children with end-stage PAH showed encouraging follow-up results. Postoperative VA-ECMO facilitates both, early extubation with rapid gain of allograft function, and cardiac adjustments to RV pressure unloading/LV volume pre-loading and contributes to excellent 1-year outcomes in our cohort, including normalization of ventricular function measured by echocardiographic 2D-B-mode and -strain/strain-rate analysis.

Publication History

Article published online:
28 January 2023