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Portopulmonary hypertension and serum endothelin-1 levels in patients with liver cirrhosis
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Published: 10 June 2026
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A broad body of evidence has accrued, demonstrating the role of endothelin-1 (ET-1) in the pathophysiology of pulmonary arterial hypertension. Furthermore, the role of ET-1 as a causative pathophysiological mechanism of portopulmonary hypertension (POPH) has been consistently implicated; however, there is a lack of satisfactory evidence supporting this assertion. Thus, the present study aims to determine the prevalence of POPH among patients with hepatic cirrhosis, determine the serum ET-1 levels among these patients, and investigate the association between ET-1 and POPH in subjects with hepatic cirrhosis. A prospective, observational study was conducted from September 2017 to August 2018. Detailed history and examination with relevant investigations, including echocardiography, were performed for patients with and without POHP. ET-1 levels were significantly higher among patients with POPH compared to those without POPH. Moreover, the ET-1 cut-off level of 82 pg/mL correctly predicted the 9.3% prevalence of POPH in patients with hepatic cirrhosis with 100% sensitivity and 100% negative predictive value. The ET-1 cut-off level of 82 pg/mL may be used as a biomarker of POPH in patients with hepatic cirrhosis.
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How to Cite
“Portopulmonary Hypertension and Serum Endothelin-1 Levels in Patients With Liver Cirrhosis”. 2026. Monaldi Archives for Chest Disease, June. https://doi.org/10.4081/monaldi.2026.3093.
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