Lung transplantation for pediatric pulmonary arterial hypertension—quo vadis?

Abstract

In children with pulmonary arterial hypertension, lung transplantation illustrates a feasible treatment option once pharmacological therapy is exhausted. Timing of listing for lung transplantation in children remains difficult since hemodynamic deterioration often occurs abruptly and the time on the waiting list is usually hard to predict. Clear contraindications for lung transplantation are recent history of malignancies as well as irreversible end-organ failure. Generally, patients with pulmonary arterial hypertension in the absence of structural cardiac defects can safely undergo bilateral lung transplantation, combined heart-lung transplantation remains a procedure with a higher perioperative risk and should only be performed in selected cases with irreversible structural defects. Donor selection in recent years shows donors with extended criteria as well as lobar transplantation with good outcome, having the positive effect of broadening of the donor pool. Bridging to lung transplantation with veno-arterial ECMO treatment is feasible and has a good outcome in experienced transplant centers. Surgical considerations should include the risk of hemodynamic decompensation upon anesthesia induction and the need for extracorporeal support pre-, intra- and postoperative. Lung transplantation should be performed on veno-arterial ECMO support with either peripheral (>20 kg) or central cannulation (<20 kg). The surgical transplantation procedure includes the bronchial anastomosis as well as anastomoses of the pulmonary artery and the left atrium. Postoperative prolonged veno-arterial ECMO treatment for the immediate postoperative period allows for left ventricular remodeling given the new hemodynamic circumstances with lower pulmonary vascular resistance. Standard triple immunosuppression in most lung transplant programs currently includes steroids, mycophenolate mofetil and tacrolimus. Survival after pediatric lung transplantation for IPAH is comparable to pediatric lung transplants for other underlying diseases with a 1-year survival of approx. 80% and a 5-year survival of 64-65%. Therefore, evolving techniques in the field of lung transplantation led to overall improved survival prospects in children with end-stage pulmonary vascular disease.