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Development of hepatopulmonary syndrome and portopulmonary hypertension in a paediatric liver transplant patient
Author(s) -
Shah Tahir,
Isaac John,
Adams David,
Kelly Deirdre
Publication year - 2005
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2004.00221.x
Subject(s) - portopulmonary hypertension , hepatopulmonary syndrome , medicine , liver transplantation , portal hypertension , pediatrics , intensive care medicine , transplantation , cirrhosis
Background: Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) are pulmonary vascular disorders which occur in patients with severe liver disease and/or portal hypertension. Although these syndromes are frequently diagnosed in patients undergoing assessment for liver transplantation, they seldom occur in the same patient. Method: This report describes a female paediatric patient, born with extra‐hepatic biliary atresia, who required liver transplantation, at the age of 15, for secondary biliary cirrhosis. She had severe HPS prior to her first liver transplant, which resolved rapidly following surgery, as well as indirect evidence for PPH. She required a second liver transplant 1 yr later for chronic rejection. Whilst evaluating the patient for a third liver transplant, 4 yr later, severe PPH was discovered. The patient died 3 months later from right heart failure. Conclusion: HPS and PPH may coexist however they may show differing responses to liver transplantation with progression of PPH despite the resolution of HPS.