Lung transplantation for pulmonary hypertension

From its identification as a distinct disease entity, understanding and management of pulmonary hypertension has continuously evolved. Diagnostic and therapeutic interventions have greatly improved the prognostic implications of this devastating disease, previously rapidly and uniformly fatal to one chronically managed by multi-disciplinary teams. Improved diagnostic algorithms and active research into biochemical signatures of pulmonary hypertension (PH) have led to earlier diagnosis of PH. Medical therapy has moved from upfront use of continuous intravenous prostaglandins to administration of combinations of oral medications targeting multiple pathways underlying this disease process. In addition to improved medical therapies, recently introduced interventions such as pulmonary endarterectomy and pulmonary artery balloon angioplasty for chronic thromboembolic pulmonary hypertension (CTEPH) give patients an increasing array of treatment options. Despite these many advances, lung transplantation remains the definitive treatment for patients with disease refractory to or progressing on best medical therapy. As our understanding of medical therapy has advanced, so to have best practices for lung transplantation. Recipient selection and approach to organ transplantation techniques have continuously evolved. Mechanical circulatory support has become increasingly employed to bridge patients through lung transplantation in the immediate post transplantation recovery. In this review, we give a history of lung transplantation for PH, an overview of PH, discuss current best practices and look to the future for insights into the care of these patients.