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Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension†
Article first published online: 29 MAY 2012
Copyright © 2012 American Association for the Study of Liver Diseases
Volume 18, Issue 6, pages 686–695, June 2012
How to Cite
Hollatz, T. J., Musat, A., Westphal, S., Decker, C., D'Alessandro, A. M., Keevil, J., Zhanhai, L. and Runo, J. R. (2012), Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension. Liver Transpl, 18: 686–695. doi: 10.1002/lt.23407
Potential Conflicts of Interest: James R. Runo has served as a consultant/and or speaker for Actelion, Gilead, United Therapeutics, and Pfizer; his last activity with any company occurred in 2008, and there has been none since. He is also an investigator in a United Therapeutics–sponsored trial (Open-Label Study to Assess the Safety and Efficacy of Treprostinil to Facilitate Liver Transplantation in Patients With Portopulmonary Hypertension; ClinicalTrials.gov identifier NCT01028651), but he has not enrolled any patients. In addition, he has served as a local investigator for multicenter studies with Actelion.
- Issue published online: 29 MAY 2012
- Article first published online: 29 MAY 2012
- Accepted manuscript online: 8 FEB 2012 12:47AM EST
- Manuscript Accepted: 2 FEB 2012
- Manuscript Received: 23 SEP 2011
Portopulmonary hypertension (PoPH) refers to pulmonary arterial hypertension associated with portal hypertension with or without evidence of an underlying liver disease. Despite the potential for curing PoPH with liver transplantation, the presence of moderate or severe PoPH is associated with increased morbidity and mortality and is, therefore, a contraindication to transplantation. Previous studies have predominantly used intravenous epoprostenol for treatment in order to qualify patients for liver transplantation. In this retrospective case series, we describe the clinical course of 11 patients whom we successfully treated (predominantly with oral sildenafil and subcutaneous treprostinil) in order to qualify them for liver transplantation. The mean pulmonary artery pressure significantly improved from 44 to 32.9 mm Hg, and the pulmonary vascular resistance decreased from 431 to 173 dyn second cm−5. There were significant improvements in the cardiac output and the transpulmonary gradient with these therapies as well. All 11 patients subsequently received liver transplants with a 0% mortality rate to date; the duration of follow-up ranged from 7 to 60 months. After transplantation, 7 of the 11 patients (64%) were off all pulmonary vasodilators, and only 2 patients required transiently increased doses of prostacyclins. In conclusion, an aggressive approach to the treatment of PoPH with sildenafil and/or treprostinil and subsequent liver transplantation may be curative for PoPH in some patients. Liver Transpl 18:686–695, 2012. © 2012 AASLD.