Post-surgical shunt hepatopulmonary syndrome in a case of non-cirrhotic portal hypertension: lack of efficacy of shunt reversal

Hepatopulmonary syndrome, a consequence of significant liver disease and po rtal hypertension, is thought to be secondary to the effects of vasoactive substances, normally inactivated in the liver, on the pulmonary vasculature , We report a patient with preserved hepatic function who underwent a decom pressive surgical portosystemic shunt for non-cirrhotic portal hypertension . This patient developed hepatopulmonary syndrome with dyspnoea and oxygen desaturation 2 years post-surgical shunt. Over the next 7 years, the patien t's respiratory function became increasingly impaired although hepatic func tion remained preserved. Because of the hypothesized role of porto-systemic shunting in the aetiology of this syndrome, the surgical shunt was success fully reversed angiographically, No improvement in dyspnoea or oxygen satur ation occurred and liver transplantation was undertaken. Six months post-tr ansplant, the patient has decreased his oxygen requirements and is free of dyspnoea, Our experience supports the causal role of porto-systemic shuntin g in the pathogenesis of hepatopulmonary syndrome but suggests that merely decreasing the extent of porto-systemic shunting is not beneficial. Liver t ransplantation remains the only reliable therapeutic modality available to these patients. fur J Gastroenterol Hepatol 11:1425-1427 (C) 1999 Lippincot t Williams & Wilkins.