[Porto-pulmonary hypertension].

Area of Science:

• Cardiology
• Hepatology
• Pulmonology

Background:

• Porto-pulmonary hypertension (PoPH) is defined by elevated pulmonary artery and portal pressures, diagnosed via right heart catheterization.
• Pathophysiology remains unclear, but portal hypertension, not liver disease severity, appears crucial for PoPH development.
• PoPH presents non-specifically, often at end-stage, necessitating screening via echocardiography in liver transplant candidates.

Purpose of the Study:

• To review current understanding and recent advances in the diagnosis and management of PoPH.
• To highlight the diagnostic criteria and screening recommendations for PoPH.
• To discuss the impact of PoPH on liver transplantation and emerging therapeutic strategies.

Main Methods:

• Review of existing literature on PoPH diagnosis and treatment.
• Analysis of hemodynamic criteria for PoPH diagnosis.
• Evaluation of current and emerging therapeutic options for PoPH.

Main Results:

• PoPH diagnosis relies on specific pulmonary hemodynamic criteria.
• Prostacyclin analogues offer partial relief for pulmonary arterial hypertension in PoPH.
• Moderate to severe PoPH is a contraindication for liver transplantation due to high perioperative mortality.

Conclusions:

• Recent therapeutic advances have improved PoPH prognosis.
• Oral endothelin receptor antagonists and phosphodiesterase inhibitors are under evaluation for PoPH.
• Combination drug therapy is expected to yield better long-term outcomes for PoPH management.