PURPOSE: The prevalence of portopulmonary hypertension (PPHTN) in patients with end stage liver disease is 3.5% to 12.5%. Elevated pulmonary arterial pressures is associated with significant morbidity and mortality and may preclude patients from receiving liver transplantation. The purpose of this study was to observe the effects of Sildenafil therapy on symptoms, pulmonary hemodynamics, and exercise tolerance in patients with PPHTN.
METHODS: We retrospectively reviewed the medical records of 10 patients with PPHTN treated with Sildenafil, at 3 centers, from 2001-2005. Study variables included patient demographics, symptoms, WHO class, pulmonary hemodynamics, and six minute walk test results (6MWT). For all continuous variables we report means ±SD and use frequencies to describe categorical data. We use the Wilcoxon test to compare differences in continuous variables before and after treatment of PPHTN. A two tailed p <0.05 is considered statistically significant for all analyses.
RESULTS: 70% of patients were female with a mean age of 55 ± 10 years. Prior to treatment, 90% of patients reported dyspnea with activity, 60% had fatigue, one patient was WHO class 4, 6 were class 3, 2 were class 2 and only 1 was class 1. Mean RVSP was 66 ± 21 mmHg, mPAP 49 ± 15 mmHg, and PVR 812 ± 502 dynes.sec/cm5. The mean 6MWT, available for 5 patients was 319 ± 196 m. Patients received a mean dose of 155±75 mg/day of Sildenafil for an average of 152 days prior to repeat measurements. Post treatment, there was improvement in symptoms with 60% of patients WHO class 2 and 40% WHO class 1. Although there was no statistically significant difference in pulmonary hemodynamics and 6MWT, pre and post therapy, there was a trend towards reduction of mPAP and PVR and improvement in 6MWT.
CONCLUSION: Sildenafil was associated with symptomatic improvement in PPHTN and a trend towards decrease in mPAP.
CLINICAL IMPLICATIONS: Sildenafil is effective in reducing symptoms of PPHTN. Prospective studies are needed to further explore these results.
DISCLOSURE: Orlando Rodriguez, None.