Sildenafil treatment for portopulmonary hypertension

¹ Pulmonary Vascular Diseases Unit, University of Giessen Lung Center, Department of Internal Medicine, University Hospital Giessen, Germany
² Division of Chest Medicine, University Hospital Graz, Austria

^* To whom correspondence should be addressed. E-mail: Frank.Reichenberger{at}innere.med.uni-giessen.de.

	Abstract

Porto- pulmonary hypertension belongs to pulmonary arterial hypertension, however currently established therapies have not been evaluated for this condition.

We treated 14 patients (4 male, 10 female, mean age 55, 39-75 years).with moderate (n=1) or severe (n=13) porto- pulmonary hypertension due to alcoholic liver disease (n=7), chronic viral hepatitis (n=3), autoimmune hepatitis (n=3), and hepatic manifestation of hereditary hemorrhagic teleangiectasia (n=1) with oral sildenafil. Eight patients had no pulmonary vasoactive treatment, 6 patients were on treatment with inhaled prostanoids (5 iloprost, 1 treprostinil). On treatment with sildenafil, 6 minute walk distance increased from mean 312 (SD 111) meters to 397 (99) meters after 3 months (p<0.001), and 407 (97) meters after 12 months. Pro-BNP levels decreased from 582 (315) ng·ml^-1 to 230 (278) ng·ml^-1 after 3 month, and 189 (274) ng·ml^-1 after 12 months. Two patients died after 1 and 2 months due to liver failure and cardiac failure, respectively. Comparing mono- and combination therapy, there was a similar response to sildenafil treatment after 3 and 12 months in both groups.

Sildenafil might be effective in mono- and combination therapy with inhaled prostanoids in porto- pulmonary hypertension leading to significant improvement after 3 months and sustained response over 12 months.

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