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Review
. 2023 Jan;19(1):55-65.
doi: 10.1016/j.hfc.2022.08.017.

Struggling Between Liver Transplantation and Portopulmonary Hypertension

Arun Jose  1 , Courtney R Jones  2 , Jean M Elwing  3
Affiliations
Review

Struggling Between Liver Transplantation and Portopulmonary Hypertension

Arun Jose et al. Heart Fail Clin. 2023 Jan.

Abstract

Portopulmonary hypertension (PoPH) is a progressive, ultimately fatal cardiopulmonary disease that occurs exclusively in patients with underlying portal hypertensive liver disease. PoPH outcomes are driven by both the severity of underlying liver disease and the degree of cardiac adaptation to elevated pulmonary pressures. The mainstay of treatment in PoPH is targeted pulmonary vascular therapy. Liver transplantation (LT) can be beneficial in some patients, but is associated with considerable risks in the PoPH population, and outcomes are variable. The optimal management strategy for PoPH, LT, or medical therapy alone, is unclear, and further research is needed to help guide clinical decision-making.

Keywords: Liver transplantation; Portopulmonary hypertension; Pulmonary arterial hypertension; Pulmonary vascular resistance; Reperfusion syndrome.

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Conflict of interest statement

Disclosure The authors have no commercial or financial conflicts of interest pertaining to the contents of this article. A. Jose receives funding from the National Institutes of Health (2UL1RT00424, 2KL2TR001426), the Parker B. Francis Foundation, and the United Therapeutics Corporation through an investigator-sponsored grant. C.R. Jones has no funding disclosures. J.M. Elwing receives funding from United Therapeutics, Altavant, Aerovate, Bayer, and Gossamer Bio and serves as a consultant for Janssen, United Therapeutics, Liquidia, Phase Bio, Gossamer Bio, Bayer, Acceleron, and Altavant.

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