What is the role of lung transplantation in treatment of pulmonary arterial hypertension (PAH)?

Updated: Aug 25, 2020
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Although lung transplantation is reserved for patients with severe pulmonary arterial hypertension (PAH), a number of patients have undergone successful transplantation at several centers. These patients had PAH due to collagen-vascular disease, drug-induced PAH, or pulmonary veno-occlusive disease (PVOD). The stability of the underlying causative disorder and the ability of the patient to tolerate an extensive operation are prerequisites. Heart-lung transplantation has been performed in patients with PAH due to congenital cardiac disease or severe left ventricular dysfunction. [32]

Although lung transplantation has historically been the treatment of choice for severe PAH, at present it is typically needed only for patients who are still in New York Heart Association (NYHA) functional class IV after 3 months of therapy with epoprostenol. The long-term outcomes of lung transplantation remain disappointing, with 50% survival at 5 years.

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