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Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation

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Abstract

Severe pulmonary hypertension is recognized by focusing on the clinical signs and tests that demonstrate decompensated right ventricular failure or, in the worst-case scenario, shock. An aggressive treatment regimen including a prostacyclin infusion is required for these patients. Once admitted to the hospital, or the ICU for decompensated right ventricular failure, the short- and long-term outcomes for PAH patients are poor. For those who are candidates, urgent lung transplantation, or extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplantation may be rescue therapy.

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Acknowledgments

Dr. Torres has received research grants from Gilead, United Therapeutics, Medtronic, GeNO, Arena, NIH. He has received a speaker honorarium from Actelion and Bayer. He has participated in advisory boards for Bayer, Gilead, Actelion, SteadyMed, Reata. Dr. Bartolome is on the speaker’s bureaus and has participated in consulting for Actelion, Bayer and Gilead Pharmaceuticals. She also receives research funding through the UTSW Pulmonary Hypertension Program from GeNO, Gilead, Actelion, Bayer, Pfizer, United Therapeutics, and Reata.

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  1. Pulmonary and Critical Care Medicine, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Rm HQ1.200, Dallas, TX, 75390-8550, USA

    Sonja Darrell Bartolome & Fernando Torres

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  1. Sonja Darrell Bartolome
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  2. Fernando Torres
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Correspondence to Sonja Darrell Bartolome.

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Bartolome, S.D., Torres, F. Severe pulmonary arterial hypertension: stratification of medical therapies, mechanical support, and lung transplantation. Heart Fail Rev 21, 347–356 (2016). https://doi.org/10.1007/s10741-016-9562-7

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