Lung transplantation as an intervention for pediatric pulmonary hypertension.

Lung transplantation is a treatment option for selected children with end-stage lung disease and pulmonary vascular disorders. Overall, pulmonary hypertension (PH) is the second most frequent indication for infants and children requiring lung transplants. In pediatric PH patients, timing for listing remains a difficult decision due to patient heterogeneity and varying allocation policies across different countries. Furthermore, perioperative management can be challenging, making interdisciplinary collaboration among surgical, anesthesiology, critical care, and lung transplant teams essential. Because pediatric PH 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 criteria for listing of primarily adults by International Society for Heart and Lung Transplantation (ISHLT) published in 2015: New York Heart Association (NYHA) functional class III or IV without improvement, cardiac index < 2 L/min/m2 , mean right atrial pressure of >15 mmHg. Bridging strategies with extracorporeal support should be determined at the time of listing in anticipation of possible clinical deterioration. Bilateral lung transplantation using cardiopulmonary bypass to provide hemodynamic stability is nowadays the standard surgical approach in pediatric centers. The immediate post-transplant period is characterized by dramatic changes in the right ventricle (RV) and and left ventricle (LV) anatomy and physiology, 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 transplantation for other indications. In fact, long-term survival is superior in children with idiopathic PH compared to other diseases, providing most recipients with improved quality of life.