Bilateral Lung Transplantation and Post-op VA-ECMO: A Novel Approach for Children with End-Stage Pulmonary Arterial Hypertension (PAH)

F. Schmidt; N. Schwerk; J. Vogel-Claussen; E. Brinkmann; H. Koeditz; T. Jack; M. Sasse; H. Bertram; A. Horke; P. Beerbaum; G. Warnecke; G. Hansmann

doi:10.1055/s-0036-1571872

The Thoracic and Cardiovascular Surgeon, Table of Contents

Bilateral Lung Transplantation and Post-op VA-ECMO: A Novel Approach for Children with End-Stage Pulmonary Arterial Hypertension (PAH)

F. Schmidt ¹, N. Schwerk ², J. Vogel-Claussen ³, E. Brinkmann ¹, H. Koeditz ¹, T. Jack ¹, M. Sasse ¹, H. Bertram ¹, A. Horke ⁴, P. Beerbaum ¹, G. Warnecke ⁴, G. Hansmann ¹

¹Hannover Medical School, Pediatric Cardiology and Critical Care, Hannover, Germany
²Hannover Medical School, Pediatric Pulmonology, Allergology, Immunology and Neonatology, Hannover, Germany
³Hannover Medical School, Diagnostic and Interventional Radiology, Hannover, Germany
⁴Hannover Medical School, Division of Cardiac, Thoracic, Transplantation and Vascular Surgery, Hannover, Germany

Abstract

Objectives: Despite improvement in pharmacotherapy, bilateral lung transplantation (BLuTx) or combined heart-lung-Tx (HLuTx) remain the only established definite treatment options for children with endstage PAH. Although PAH is the second most common indication for BLuTx in children, data on the best perioperative management, pre- and postoperative cardiac function and mid/long-term outcome are lacking. We hypothesized that BLuTx followed by early extubation and awake VA-ECMO is associated with excellent short- and mid-term outcomes.

Methods: This is a retrospective study on children with PAH who underwent BLuTx at Hannover Medical School from January 2010 to September 2015. Preoperative (demographics, echocardiography, cardiac MRI, resuscitation, ECMO), perioperative (ECMO management) and postoperative (echocardiography, cardiac MRI, survival) data were collected and analyzed.

Results: Seven consecutive patients with PAH underwent minimal invasive BLuTx (mean age: 13years; range: 7–16years; 6 idiopathic, 1 after d-TGA arterial switch repair). Average time on HU waiting list was 52days (range: 1–130days); 3 patients were resuscitated prior to BLuTx, 2 of which were bridged to BLuTx on VA-ECMO. Intraoperative VA-ECMO or cardiopulmonary bypass was applied in five and two patients, respectively; five patients received scheduled post-BLuTx VA-ECMO support (mean duration: 8.4days; range: 6–12days). The goal “early extubation and awake VA-ECMO” could be performed in four of the five patients. Preoperative echocardiography and cardiac MRI showed a severely compromised enlarged right ventricle (RV), that is, suprasystemic/systemic RV pressure, endsystolic septal shift with LV compression, and pericardial effusion. Hemodynamic compromise rapidly improved after BLuTx, that is, normalization of RV volumes, and systolic function, fade of LV compression and pericardial effusion). As of September 2015, all patients are still alive post-BLuTx, with a median survival time of 2.7years (range: 0–5years).

Conclusions: BLuTx in children with endstage PAH is associated with encouraging Results in our center. Post-op “awake VA-ECMO” might help facilitating RV and LV recovery, reducing perioperative and midterm mortality, and thus allowing better outcomes.