Le syndrome hépatopulmonaire et l’hypertension portopulmonaire

Abstract

Pulmonary vascular diseases associated with chronic liver diseases, including hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH), represent significant clinical challenges due to their impact on patient management and prognosis. HPS is characterized by hypoxemia caused by pulmonary vasodilation and intrapulmonary shunting, whereas PoPH results from pulmonary vascular remodeling, leading to increased pulmonary artery pressure and potential right heart failure. These conditions can coexist in patients with advanced liver disease, significantly worsening their functional status and survival.

The prevalence of HPS is estimated to range from 10% to 20% of chronic liver disease patients, while PoPH affects approximately 2% to 6%. Notably, the severity of these complications does not directly correlate with the extent of hepatic dysfunction, making early recognition and management complex. While liver transplantation can reverse HPS in many cases, the prognosis of PoPH remains highly variable, with some cases being irreversible despite treatment.

Given the multifactorial nature of these disorders, a multidisciplinary approach is essential to optimize patient outcomes. Early screening, accurate diagnosis, and tailored therapeutic strategies are crucial for improving prognosis and guiding the selection of candidates for liver transplantation or alternative treatments.