Document Detail


Portopulmonary hypertension: An update.
MedLine Citation:
PMID:  22674534     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Portopulmonary hypertension is a serious complication of cirrhosis that is associated with mortality in excess of the MELD score. Increased pulmonary vascular resistance may be initiated by pulmonary vasoconstriction, altered levels of circulating mediators, or shear stress, eventually leading to the classic vascular remodeling (plexiform lesion) that characterizes portopulmonary hypertension. Portal hypertension is a prerequisite for the diagnosis of portopulmonary hypertension although the severity of the pulmonary hypertension is unrelated to the severity of portal hypertension or the nature or severity of liver disease. Portopulmonary hypertension precludes liver transplantation unless the mean pulmonary artery pressure can be reduced to a safe level. The concept of an acceptable pressure has changed - we now consider both the mean pulmonary artery pressure and the pulmonary vascular resistance, and we include the transpulmonary pressure gradient to factor in fluid overload and left ventricular failure. Pulmonary vasodilator therapy includes oral, inhaled and parenteral agents, and one or more of these agents may significantly lower pulmonary artery pressures to the point that liver transplantation becomes possible. UNOS has recommended MELD exception points for patients with medically controlled portopulmonary hypertension, but this varies by region. Patients who undergo liver transplantation need specialized intraoperative and postoperative management including the availability of intraoperative transesophageal echocardiography to assess right ventricular function, and rapidly acting vasodilators like inhaled nitric oxide and/or epoprostenol. Published case series suggest that liver transplantation has an excellent outcome in patients who respond to medical therapy. © 2012 American Association for the Study of Liver Diseases.
Authors:
Norman Sussman
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-6-4
Journal Detail:
Title:  Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society     Volume:  -     ISSN:  1527-6473     ISO Abbreviation:  -     Publication Date:  2012 Jun 
Date Detail:
Created Date:  2012-6-7     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100909185     Medline TA:  Liver Transpl     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 American Association for the Study of Liver Diseases.
Affiliation:
Baylor College of Medicine 6550 Fannin, Suite 1661 Houston, TX 77030. normans@bcm.edu.
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