Hepatopulmonary syndrome in patients with porto-sinusoidal vascular disorder: Characteristics and outcome.

ABSTRACT:

Background & aims

Porto-sinusoidal vascular disorder (PSVD) is a rare cause of portal hypertension. Data on hepatopulmonary syndrome (HPS) in PSVD are limited. This study aimed to determine the associated factors, plasma mediators, and evolution of HPS in patients with PSVD.

Methods

Multicenter observational study of patients with PSVD with signs of portal hypertension in whom contrast-enhanced transthoracic echocardiography (CE-TTE) was performed.

Results

Among 196 patients with PSVD who underwent CE-TTE in 17 centers, 14 (7% [95% confidence interval 3-11%]) had a confirmed diagnosis of HPS. Patients with HPS more frequently had a genetic disorder associated with PSVD (50% vs. 6%, p <0.001), especially telomere biology disorders (p <0.001). Liver

SUBMITTER: Sidali S 

PROVIDER: S-EPMC11960633 | biostudies-literature | 2025 Apr

REPOSITORIES: biostudies-literature