Abstract Background Limited data are available on hemodynamic characteristics, outcomes, and liver transplantation candidacy for portopulmonary hypertension (PoPH). This study aimed to evaluate the hemodynamic characteristics, natural course, and outcomes following liver transplantation in PoPH patients. Methods Patients diagnosed with PoPH by right heart catheterization at two referral centers were retrospectively analyzed. PoPH was defined as Group 1 pulmonary arterial hypertension with clinical evidence of portal hypertension. Results We included 43 patients with PoPH (median age: 52 years; 19 men). Advanced liver cirrhosis was associated with higher cardiac index and tricuspid annular plane systolic excursion (TAPSE). Mean pulmonary arterial pressure (PAP) and right atrial pressure increased with increasing pulmonary vascular resistance (PVR), while the cardiac index declined but was mostly preserved within normal limits. Of the 34 patients without liver transplantation, 13 (38%) died during a median follow-up of 2.2 years. Severity of liver cirrhosis and an increased TAPSE were significant predictors of mortality (per 1 mm increase, adjusted hazard ratio 1.17, 95% confidence interval 1.01-1.35, p=0.042). Among the nine patients who underwent liver transplantation, six had a mean PAP of 35–45 mmHg and PVR >3 Wood units; of these, one died after liver transplantation, from reasons unrelated to pulmonary hypertension. Conclusion In PoPH patients, a higher cardiac index with right ventricular hypercontraction was prominent in advanced stages of liver cirrhosis. Higher TAPSE was identified as a risk factor for mortality in patients without liver transplantation. Establishing optimal PoPH candidates for liver transplantation is essential for improving patient survival.
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