Pulmonary arterial hypertension and lung transplantation.

Transplantation remains the only therapeutic option for selected patients with advanced pulmonary arterial hypertension (PAH) who continue to deteriorate despite optimal pulmonary vasodilator therapy - including intravenous prostanoids and combination therapy. Identification of poor prognostic markers in PAH, including persistence in the New York Heart Association functional class III and IV, and adverse pulmonary hemodynamics at right heart catheterization should prompt early referral for transplantation. There is a need for inclusion of more discriminatory markers of PAH prognosis in donor-lung allocation scores to identify patients at risk and optimize survival to transplantation, given the current shortage of donor organ availability worldwide. Double-lung transplantation is the recommended operation for idiopathic PAH. Heart-lung transplantation is reserved for selected patients with idiopathic PAH with severe right ventricular dysfunction, or congenital heart disease with complex or ventricular septal defect-associated PAH. Novel surgical strategies, including atrial septostomy or the pumpless Novalung® lung assist device with conduits, from the pulmonary artery to the left atrium, can be considered as a bridge to transplant for patients with rapid clinical decline, despite maximal medical therapy. Recent transplant outcomes for PAH are encouraging, albeit with early postoperative risks, a requirement for long-term surveillance, immunosuppression and transplant immunosuppression-specific morbidity.