(1704) ECMO Bridge to Pediatric Lung Transplant After Recent Liver Transplant: A Rare Case of HPS

Introduction: Pediatric lung transplants are rare, more so dual lung and liver transplants. We present a pediatric patient with concerns for hereditary hemorrhagic telangiectasia (HHT) receiving primarily a liver transplant, which was complicated by severe hepatopulmonary syndrome (HPS) with worsening respiratory failure requiring venovenous extracorporeal membrane oxygenation (VV-ECMO) as bridge to lung transplant.

Description: This patient is a 10-year-old female, with no significant past medical history, who developed progressive hypoxemia. She was found to be hypoxemic during a nurse visit in school, who sent her to the emergency department. During this visit, she was treated for asthma without improvement. Eventually, she was seen by a pulmonologist and diagnosed with possible HHT. A cardiac catheterization showed torrential arteriovenous shunting from both lungs, which accounted for her degree of hypoxemia demonstrated. Further studies revealed liver involvement with portal hypertension and HPS. Following these diagnoses, a decision was made for liver transplant only, expecting improvement of hypoxemia. Following liver transplant, she remained exceptionally hypoxemic, especially worsening on week 3 with partial pressure of arterial oxygen of 32. A computerized tomography of the chest showed areas of ground glass opacities and lung parenchymal disease, which was a change from prior imaging. With continued worsening, on week 4 post liver transplant, she was placed on VV-ECMO. A lung biopsy showed acute lung injury with no infectious etiology. She was re-evaluated by lung transplant team and decision was made to approve for listing. On week 8 post liver transplant, and after 32-days of VV-ECMO with respiratory support, she received bilateral lung transplant. She has shown significant improvement post-transplant with being rapidly weaned to room air on week 9.

Discussion: ECMO has been used as a bridge to lung transplantation in pediatric patients and has steadily increased in the last two decades. To our knowledge, there are no other case reports in a pediatric patient needing ECMO soon after liver transplant for HPS to be used as a bridge for lung transplant.