Publication:


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.

Copyright © 2012 American Association for the Study of Liver Diseases.

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