Long-Term Outcome of Pulmonary Artery Hypertension-Specific Therapy in Patients With Portopulmonary Hypertension: A Multi-Institutional Database Case Series Study

Ding-Sen Huang, Ting-Hsin Huang, Yi-Ting Chen, Wen-Hao Liu, Sheng-Ying Chung, Cheng-I. Cheng

https://doi.org/10.1002/pul2.70301 

Abstract

Portopulmonary hypertension (PoPH) is a subtype of pulmonary arterial hypertension (PAH) associated with portal hypertension, occurring in 2%–6% of cirrhotic patients. Although guidelines recommend routine echocardiography before transplantation, the prevalence, and outcomes of PAH-specific therapy in cirrhotic patients remain unclear. We retrospectively analyzed 4,113 cirrhotic patients who underwent transthoracic echocardiography (TTE) between 2008 and 2019 using the Chang Gung Research Database. Patient characteristics, medication, echocardiographic findings, right heart catheterization (RHC) results and clinical outcome were collected from medical records and retrospectively analyzed by the investigators. Among 3243 patients with completed TTE, 709 (21.9%) had elevated tricuspid regurgitation peak gradient (TRPG ≥ 35 mmHg), suggesting pulmonary hypertension. There were 52 patients underwent RHC at our center, of whom 46 (88.5%) fulfilled hemodynamic criteria for PoPH. Additionally, seven patients with externally confirmed PoPH were included, yielding 53 patients in the final analysis. Among them, 13 patients received PAH-specific therapy with significant TRPG reduction but shorter mean survival (median survival 22.6 vs 78.9 months, p = 0.037) than those without therapy. Among patients under PAH-specific therapy, those who had higher baseline TRPG (≧ 50 mmHg), mPAP (≧ 40 mmHg) and pulmonary vascular resistance (≧ 2 Wood Units) were noted with better survival. PoPH is not rare in patients with liver cirrhosis. Exploratory subgroup analyses suggest that selected patients with severe hemodynamic impairment may potentially benefit from PAH-specific therapy; however, these findings require validation in larger prospective studies.

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