Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up

Thomas Jack; Julia Carlens; Franziska Diekmann; Hosan Hasan; Philippe Chouvarine; Nicolaus Schwerk; Carsten Müller; Ivonne Wieland; Igor Tudorache; Gregor Warnecke; Murat Avsar; Alexander Horke; Fabio Ius; Dmitry Bobylev; Georg Hansmann

doi:10.3389/fcvm.2023.1193326

Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up

Front Cardiovasc Med. 2023 Jun 27:10:1193326. doi: 10.3389/fcvm.2023.1193326. eCollection 2023.

Authors

Thomas Jack^#^{1

2}, Julia Carlens^#³, Franziska Diekmann^#^{1

2}, Hosan Hasan^{1

2}, Philippe Chouvarine^{1

2}, Nicolaus Schwerk³, Carsten Müller³, Ivonne Wieland⁴, Igor Tudorache⁵, Gregor Warnecke⁶, Murat Avsar⁷, Alexander Horke⁷, Fabio Ius⁷, Dmitry Bobylev⁷, Georg Hansmann^{1

2}

Affiliations

¹ Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany.
² European Pediatric Pulmonary Vascular Disease Network, Berlin, Germany.
³ Department of Pediatric Pulmonology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.
⁴ Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany.
⁵ Department of Cardiac Surgery, University Hospital of Zürich, Zürich, Switzerland.
⁶ Department of Cardiac Surgery, Ruprecht-Karls-University, Heidelberg, Germany.
⁷ Department of Cardiothoracic, Transplantation and Vascular Surgery, Hannover Medical School, Hannover, Germany.

^# Contributed equally.

Abstract

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).

Conclusion: LuTx in children with end-stage PAH resulted in excellent midterm outcomes (100% survival 2 years post-LuTx). Postoperative VA-ECMO facilitates early extubation with rapid gain of allograft function and sustained biventricular reverse-remodeling and systolic function after RV pressure unloading and LV volume loading.

Keywords: awake ECMO; children; extracorporeal membrane oxygenation (ECMO); lung transplantation; pediatric; pulmonary arterial hypertension.

Grants and funding

This study was supported by the German Research Foundation (DFG KFO311; HA4348/6-2 to GH) and the European Pediatric Pulmonary Vascular Disease Network (www.pvdnetwork.org). GH received additional funding from the German Research Foundation (DFG; HA4348/2-2) and the Federal Ministry of Education and Research (BMBF ViP+ program 03VP08053; BMBF 01KC2001B).