Despite recent advances in medical management, lung and heart–lung transplantation remains an established treatment option in patients with end-stage pulmonary arterial hypertension (PAH) despite optimal medical therapy. The optimal timing of referral and listing patients for transplantation must take into consideration the co-morbidities, the aetiology of PAH, and the regular risk assessment based on a multidimensional approach. With the exception of PAH due to complex congenital heart disease, double-lung transplantation is the preferred option over combined heart–lung transplantation as cardiac function recovers after transplantation in most cases. The use of extracorporeal life support as a bridge to urgent transplantation may be considered in expert centres in selected patients with refractory right heart failure despite optimal medical management in an intensive care unit. With a survival rate at 5 years varying from 50% to 75%, transplantation in PAH can be considered as a life-saving procedure for patients with severe symptoms.
Extracorporeal life support as bridge to lung transplantation: a systematic review
