Hemodynamic Profile, Clinical Outcomes, and Potential Transplant Candidacy in Patients With Portopulmonary Hypertension

BACKGROUND AND OBJECTIVES: Data on hemodynamic characteristics, outcomes, and liver transplantation candidacy in portopulmonary hypertension (PoPH) are limited. We aimed to evaluate hemodynamics, the natural course, and post-transplant outcomes in PoPH. METHODS: Patients diagnosed with PoPH by right heart catheterization (RHC) at 2 referral centers were retrospectively analyzed. PoPH was defined as Group 1 pulmonary hypertension (PH) with clinical evidence of portal hypertension. RESULTS: We analyzed 43 patients with PoPH (median age: 52 years; 19 men; 26 with Child-Pugh class B/C). Patients with Child-Pugh class B/C had higher cardiac index and tricuspid annular plane systolic excursion (TAPSE) than those with class A (cardiac index 3.0 vs. 2.6 L/min/m², p=0.017; TAPSE 22.8 vs. 18.7 mm, p=0.004). During a median follow-up of 1.6 years, 13 patients (30%) died and 9 (21%) underwent liver transplantation. Liver cirrhosis severity was significantly associated with mortality (adjusted hazard ratio per 1-point increase in Child-Pugh score, 1.53; 95% confidence interval, 1.14-2.05; p=0.005), independent of age and mean pulmonary artery pressure (mPAP). Among the 9 patients who underwent liver transplantation, 6 had a mPAP of 35-45 mmHg on the RHC performed closest to transplantation; of these, one died from non-PH-related causes. Both PH and TAPSE decreased after successful transplantation. CONCLUSIONS: In PoPH, higher cardiac index and TAPSE were associated with more advanced liver cirrhosis. Advanced liver cirrhosis was an independent risk factor for mortality. Patients with borderline PH severity appeared to tolerate liver transplantation, suggesting that transplantation may be reasonably considered in this subgroup.