Efficacy and safety of tadalafil in portopulmonary hypertension
Abstract
Data on efficacy and safety of tadalafil in portopulmonary hypertension (PoPH) are lacking. The aim of our study was to analyze short- and long-term efficacy and safety of an initial monotherapy with tadalafil in patients with PoPH. All consecutive patients with PoPH initiated with tadalafil were retrospectively analyzed. Clinical and haemodynamic characteristics were assessed at the time of diagnosis (baseline) and during follow-up. Overall survival was analyzed. Thirty-eight patients were analyzed (71% males, mean age 54±11 years). Fourteen patients (37%) were in Child-Pugh (CP) stage A and twenty-four (63%) in CP stage B or C. The mean MELD score was 14±5. Baseline NYHA functional class (FC) distribution was I-II (34%), III (58%) and IV (8%). Baseline 6-min walk distance (6MWD) was 380±120 m, mean pulmonary artery pressure (mPAP) 44±8 mmHg, cardiac index (CI) 3.1±0.9 L/min/m2 and pulmonary vascular resistance (PVR) 6.6±2.8 WU. After a median follow-up of 4 months, 33 patients have been reevaluated. There were significant improvements from baseline in FC (p<0.001), mPAP (-8±8 mmHg, p=0.0001), CI (+0.7±0.3 L/min/m², p=0.005) and PVR (-35±27%, p=0.0001). There was no significant change in 6MWD. Change in PVR was similar irrespective of CP stage. Three patients had significant fluid retention, but no other significant side-effects were observed. During a study follow-up of 18±12 months, 1/14 patients with CP A and 14/24 with CP B-C cirrhosis had died. Overall survival was 73%…
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