Original Article

Treatment with sildenafil and treprostinil allows successful liver transplantation of patients with moderate to severe portopulmonary hypertension†

  1. Trina J. Hollatz M.D.1,
  2. Alexandru Musat M.D.2,
  3. Susanne Westphal R.N.1,
  4. Catherine Decker Pharm.D1,
  5. Anthony M. D'Alessandro M.D.3,
  6. Jon Keevil M.D.4,
  7. Li Zhanhai PhD5,
  8. James R. Runo M.D.1,‡,*

DOI: 10.1002/lt.23407

Additional Information

Author Information

  1. 1

    Division of Pulmonary and Critical Care, Department of Medicine, University of Wisconsin-Madison, School of Medicine and Public Health, Madison, WI

  2. 2

    Division of Gastroenterology and Hepatology, Department of Medicine, University of Wisconsin-Madison, School of Medicine and Public Health, Madison, WI

  3. 3

    Division of Transplant Surgery, Department of Surgery, University of Wisconsin-Madison, School of Medicine and Public Health, Madison, WI

  4. 4

    Division of Cardiology, Department of Medicine, University of Wisconsin-Madison, School of Medicine and Public Health, Madison, WI

  5. 5

    Department of Biostatistics and Medical Informatics, University of Wisconsin-Madison, School of Medicine and Public Health, Madison, WI

  1. ‡

    Ph: 608-263-3035; Fax: 608-263-3104

*600 Highland Avenue MC 9988, Madison, WI 53719

  1. †

    Conflict of interest: James R. Runo has served as a consultant/and or speaker for Actelion, Gilead, United Therapeutics, and Pfizer with last activity with any company in 2008 and none since. He is also an investigator in “An Open-Label Study to Assess the Safety and Efficacy of Treprostinil to Facilitate Liver Transplantation in Patients with Portopulmonary Hypertension” (clinical trial NCT01028651) sponsored by United Therapeutics but has not enrolled a patient. He also has served as a local investigator for multicenter studies with Actelion.Financial support: none

Publication History

  1. Accepted manuscript online: 8 FEB 2012 12:47AM EST
  2. Manuscript Accepted: 2 FEB 2012
  3. Manuscript Revised: 27 JAN 2012
  4. Manuscript Received: 23 SEP 2011

SEARCH

SEARCH BY CITATION

ARTICLE TOOLS

Get PDF (477K)

Abstract

Backgrounds and Aims:

Portopulmonary hypertension refers to pulmonary arterial hypertension associated with portal hypertension, with or without evidence of underlying liver disease. Despite the potential for cure of portopulmonary hypertension with liver transplantation, the presence of moderate or severe portopulmonary hypertension is associated with increased morbidity and mortality and therefore is a contraindication to transplantation. Previous studies have predominantly utilized intravenous epoprostenol for treatment in order to qualify for liver transplantation.

Methods:

In this retrospective case series, we describe the clinical course of 11 patients that we successfully treated, predominantly with oral sildenafil and subcutaneous treprostinil, in order to qualify for liver transplantation.

Results:

The mean pulmonary artery pressure significantly improved from 44 mmHg to 32.9 mmHg and pulmonary vascular resistance decreased from 431 to 173 dyn s cm−5. There was significant improvement in the cardiac output and transpulmonary gradient with these therapies as well. All 11 patients subsequently received liver transplants with zero mortality to date with duration of follow-up ranging from 7 to 60 months. Post-transplant seven of eleven patients (64%) are off all pulmonary vasodilators, while only two patients required transient increased doses of prostacyclins.

Conclusion:

An aggressive approach for treatment of portopulmonary hypertension with sildenafil and/or treprostinil and subsequent liver transplant may be curative for PoPH in some patients. Liver Transpl, 2012. © 2012 AASLD

Get PDF (477K)