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