BACKGROUND: Hepatopulmomary syndrome is defined by the triad of chronic liver disease, increased alveolar-arterial gradient, and evidence of intrapulmonary vasodilation. It is commonly seen in association with cirrhosis (90%). Four percent to 8% of the hepatopulmomary syndrome cases are reported in noncirrhotic portal hypertension. The management of patients with hepatopulmomary syndrome due to noncirrhotic portal hypertension is not well described. METHODS: We report a case of a 26-year-old woman who underwent liver transplantation for hepatopulmomary syndrome due to noncirrhotic portal hypertension. The patient presented with dyspnea and platypnea, requiring home oxygen therapy. She had orthodexia, severe hypoxemia, and positive bubble echocardiography consistent with hepatopulmomary syndrome. Her Model for End-stage Liver Disease score was 10. Liver biopsy revealed diffuse nodular regenerative hyperplasia. RESULTS: The patient underwent liver transplantation with Model for End-stage Liver Disease exception points. Her oxygen requirements gradually improved during the postoperative period. The patient's symptoms and hypoxemia resolved at 15-month follow-up posttransplantation. CONCLUSION: We suggest hepatopulmonary syndrome in this setting is an indication for liver transplantation despite the absence of cirrhosis.
BACKGROUND:Hepatopulmomary syndrome is defined by the triad of chronic liver disease, increased alveolar-arterial gradient, and evidence of intrapulmonary vasodilation. It is commonly seen in association with cirrhosis (90%). Four percent to 8% of the hepatopulmomary syndrome cases are reported in noncirrhotic portal hypertension. The management of patients with hepatopulmomary syndrome due to noncirrhotic portal hypertension is not well described. METHODS: We report a case of a 26-year-old woman who underwent liver transplantation for hepatopulmomary syndrome due to noncirrhotic portal hypertension. The patient presented with dyspnea and platypnea, requiring home oxygen therapy. She had orthodexia, severe hypoxemia, and positive bubble echocardiography consistent with hepatopulmomary syndrome. Her Model for End-stage Liver Disease score was 10. Liver biopsy revealed diffuse nodular regenerative hyperplasia. RESULTS: The patient underwent liver transplantation with Model for End-stage Liver Disease exception points. Her oxygen requirements gradually improved during the postoperative period. The patient's symptoms and hypoxemia resolved at 15-month follow-up posttransplantation. CONCLUSION: We suggest hepatopulmonary syndrome in this setting is an indication for liver transplantation despite the absence of cirrhosis.
Authors: Berrie Meijer; Melek Simsek; Hans Blokzijl; Robert A de Man; Minneke J Coenraad; Gerard Dijkstra; Carin Mj van Nieuwkerk; Chris Jj Mulder; Nanne Kh de Boer Journal: United European Gastroenterol J Date: 2016-11-16 Impact factor: 4.623
Authors: José Leonardo Faustini Pereira; Lucas Homercher Galant; Danusa Rossi; Luis Henrique Telles da Rosa; Eduardo Garcia; Ajácio Bandeira de Mello Brandão; Cláudio Augusto Marroni Journal: Can J Gastroenterol Hepatol Date: 2016-07-31
Authors: Orlando Luis de Andrade Santarém; Roberto de Cleva; Flávia Megumi Sasaya; Marianna Siqueira de Assumpção; Meive Santos Furtado; Alfonso Julio Guedes Barbato; Paulo Herman Journal: PLoS One Date: 2016-04-27 Impact factor: 3.240