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Open Access Case report

Phosphodiesterase 5 inhibitors lower both portal and pulmonary pressure in portopulmonary hypertension: a case report

Hinrich C Bremer1, Wolfgang Kreisel2*, Kai Roecker3, Michael Dreher1, Daniel Koenig3, Anna Katharina Kurz-Schmieg2, Hubert E Blum2, Martin Roessle4 and Peter Deibert3

Author Affiliations

1 Department of Pneumology, University Hospital, Freiburg, Germany

2 Department of Gastroenterology, Hepatology, Endocrinology and Infectious Diseases, University Hospital, Freiburg, Germany

3 Department of Rehabilitative and Preventive Sports Medicine, University Hospital, Freiburg, Germany

4 Gastrointestinal and Endocrinological Center, Freiburg, Germany

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Journal of Medical Case Reports 2007, 1:46  doi:10.1186/1752-1947-1-46

Published: 10 July 2007

Abstract

Background

Portopulmonary hypertension (PPHTN) is a severe complication in liver cirrhosis. PDE5 inhibitors lower pulmonary arterial pressure (PAP) in PPHTN. However, their effect on portal hypertension has not yet been investigated.

Case presentation

A 55 year old male patient presented with PPHTN and alcoholic liver cirrhosis. 10 mg of Tadalafil, a PDE5 inhibitor with a long half-life, was administered orally under continuous monitoring of pulmonary and portal hemodynamics. For maintenance therapy the patient received Sildenafil 20 mg bid.

Tadalafil lowered mean PAP from 45 to 39 mmHg within 60 minutes. Cardiac output (CO) increased from 6.8 to 7.9 l/min. Central venous pressure (CVP) remained stable at 3 mmHg. Systolic and diastolic blood pressure was lowered from 167/89 to 159/86 mmHg. Pulse rate increased from 75 to 87 per min. Wedged hepatic vein pressure (WHVP) decreased from 21 to 18 mm Hg, hepatovenous pressure gradient (HVPG) decreased from 10 to 7 mmHg. Hemodynamic monitoring after 6 months of Sildenafil therapy revealed a sustained lowering of mean PAP. HVPG remained constant at 10 mmHg. Cardiac and pulmonary performance had further improved.

Conclusion

This case report shows for the first time, that phosphodiesterase 5 inhibitors lower both portal and pulmonary pressure in portopulmonary hypertension.