Its incidence in patients with liver cirrhosis is about 10%,2
and in the literature this rate is 10 to 20% in patients who
are candidates to liver transplantation .3,4 A few cases of
noncirrhotic portal hypertension (NCPH) complicated by
HPS have been published.5,6 Therefore the cirrhosis is not
a strict criterion for HPS identification. We present here a
patient with a classical presentation of severe HPS, which
was caused by idiopathic portal hypertension, and we discuss
the effect of paroxetine therapy on this syndrome.
Hepatopulmonary syndrome has yet not been sufficiently assessed in noncirrhotic portal hypertension
. The prevalence of hepatopulmonary syndrome was determined in 31 consecutive patients with noncirrhotic portal hypertension (19 idiopathic portal hypertension, 7 portal vein thrombosis, 5 congenital hepatic fibrosis) and 46 patients with liver cirrhosis. Contrast echocardiography was carried out in all patients. Macroaggregated albumin lung perfusion scans were performed in patients with positive contrast echocardiogram . Hepatopulmonary syndrome was detected in 5 (10.8%) cirrhotic and 3 (9.7%) noncirrhotic portal hypertensive patients (2 idiopathic portal hypertension, 1 portal vein thrombosis). All patients with hepatopulmonary syndrome had an increased shunt fraction (13-62%) and a decreased diffusion capacity of carbon monoxide (40-79%), and 7 of them were hypoxemic (PaO2, 31.6-69.8 mm Hg ). These findings show that hepatopulmonary syndrome may occur in both liver cirrhosis and noncirrhotic portal hypertension and that portal hypertension is the predominant etiopathogenic factor related to hepatopulmonary syndrome.