What question did this study set out to answer?

To evaluate the effectiveness of various management strategies for portopulmonary hypertension.

January 10, 2026Right Heart Journal0 citationsOpen Access

The Management of Portopulmonary Hypertension

JYJian-Kuan YehNational Cheng Kung University Hospital JLJia-Ling LinNational Cheng Kung University Hospital CHChih-Hsin HsuNational Cheng Kung University Hospital

Key Points

To evaluate the effectiveness of various management strategies for portopulmonary hypertension.
Conducted a retrospective observational study on patients diagnosed with portopulmonary hypertension.

Structured PICO

Population

Patients with portopulmonary hypertension (PoPH), defined as pulmonary arterial hypertension in the presence of portal hypertension (with or without liver cirrhosis).

Intervention

Pulmonary arterial hypertension-targeted therapies (including prostanoids, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and soluble guanylate cyclase stimulators) as a bridge to liver transplantation.

This comprehensive review highlights that while portopulmonary hypertension poses a significant clinical challenge, appropriate management with PAH-targeted therapies can successfully bridge selected patients to liver transplantation.

Abstract

Portopulmonary hypertension (PoPH), a subtype of pulmonary arterial hypertension, is characterized by the presence of pulmonary hypertension in patients with liver cirrhosis and high hepatic venous pressure gradient and continues to pose a clinical challenge.Current evidence indicates that supportive care in combination with pulmonary arterial hypertension-targeted therapy can allow patients with PoPH to undergo liver transplantation.This article presents a brief clinical case followed by a review of the epidemiology, clinical features, diagnosis, and management of PoPH.

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