Resolution of Severe Portopulmonary Hypertension With Inhaled Treprostinil and Liver Transplantation
Publication Date
3-14-2024
Journal
The Texas Heart Institute Journal
DOI
10.14503/THIJ-23-8209
PMID
38483473
Publication Date(s)
January-June, 2024
Language
English
PMCID
PMC11075503
PubMedCentral® Posted Date
3-14-2024
PubMedCentral® Full Text Version
Post-Print
Published Open-Access
yes
Keywords
Heart failure, hypertension, pulmonary, hypertension, portal, pulmonary arterial hypertension
Copyright
This work is licensed under a Creative Commons Attribution-NonCommercial-No Derivative Works 4.0 International License.
Abstract
Portopulmonary hypertension is a rare condition with a poor prognosis. Prompt management is essential for liver transplantation eligibility, a potentially curative option. This report presents a case of severe portopulmonary hypertension that resolved with a conservative therapeutic regimen of tadalafil, macitentan, and inhaled treprostinil, which ultimately enabled successful liver transplantation. There was no recurrence of pulmonary hypertension after transplantation, and the patient was weaned off most pulmonary arterial hypertension therapies. This case report is the first to provide evidence that inhaled treprostinil is a safe and effective alternative to continuous intravenous prostacyclins in portopulmonary hypertension.