Children with sickle cell disease (SCD) are at increased risk for severe illness due to severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2). We describe the successful native lung recovery of a child with SCD referred for lung transplant (LTx) evaluation who was on prolonged veno-venous extracorporeal membrane oxygenation (VV-ECMO). He initially presented with acute chest syndrome complicated by SARS-CoV-2 infection that ultimately required dual-lumen, single bicaval VV-ECMO cannulation for respiratory support. Despite increased risk of hemolysis and thrombosis from SCD and SARS-CoV-2 infection, he was successfully supported on VV-ECMO for 71 days without complications leading to native lung recovery with meticulous management of his SCD therapy. This report provides new insight on our approach to VV-ECMO support in a child with SCD and SARS-CoV-2 infection. With a successful outcome, the patient has returned home but still on mechanical ventilation with LTx still an option if he is not eventually liberated from invasive respiratory support.

Lung transplantation is recognized as a treatment option for selected patients with end-stage lung disease and pulmonary vascular disorders. Overall, pulmonary hypertension (PH) is the second most frequent indication for children requiring lung transplantation. Pediatric lung transplantation can be complex; in PH patients timing for listing and the perioperative management can be particularly challenging making interdisciplinary collaboration with the referring PH team essential. Because pediatric patients typically have preserved cardiac index and exercise tolerance even with advanced disease, they should be referred early even if they do not meet the proposed criteria for listing by ISHLT published in 2015: NYHA functional class III or IV without improvement, cardiac index < 2 liters/min/m^2, mean right atrial pressure of > 15 mmHg. Bridging strategies with extracorporeal support should be determined at time of listing prior to transplantation anticipating the possibility of clinical deterioration. Bilateral lung transplantation using cardiopulmonary bypass to provide hemodynamics stability is the standard practice in pediatric centers. The immediate post-transplant period is characterized by dramatic normalization of PVR as well as changes in the RV and LV physiology and function which can be life-threatening. Induction, immunosuppression, prophylaxis and surveillance are not different from patients without PH. Overall, outcomes in pediatric lung and heart-lung transplant patients for PH are not different from those children undergoing lung or heart-lung transplant for other indications. In fact, long-term survival is superior in children with idiopathic PH compared to other diseases, there is also a dramatic improvement in quality of life in most recipients