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

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

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=10

YEAR=2023

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2023.1193326

DOI=10.3389/fcvm.2023.1193326

ISSN=2297-055X

ABSTRACT=<sec><title>Background</title><p>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.</p></sec><sec><title>Methods</title><p>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.</p></sec><sec><title>Results</title><p>Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9–17.8). Underlying diagnoses included idiopathic (<italic>n</italic> = 4) or heritable PAH (<italic>n</italic> = 4), PAH associated with congenital heart disease (<italic>n</italic> = 2), pulmonary veno-occlusive disease (<italic>n</italic> = 1), and pulmonary capillary hemangiomatosis (<italic>n</italic> = 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).</p></sec><sec><title>Conclusion</title><p>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.</p></sec>