Hepatopulmonary Syndrome in children: a comparative study of non-cirrhotic vs. cirrhotic portal hypertension.

Abstract

Hepatopulmonary syndrome in children has not been extensively studied particularly in different subsets of portal hypertension. We prospectively studied hepatopulmonary syndrome in children with cirrhosis and extrahepatic portal venous obstruction. Their comparison might shed light on the pathogenesis of hepatopulmonary syndrome. Between 1 to 18 years of age, 135 children with portal hypertension (cirrhosis 35, extrahepatic portal venous obstruction 100) were studied. Hepatopulmonary syndrome was diagnosed by the presence intrapulmonary shunt using transthoracic contrast echocardiography and hypoxia/orthodeoxia (in arterial blood gas examination). Comparative analyses were done for both the groups, and also intra- and intergroups with and without hepatopulmonary syndrome. Of 135 children, hepatopulmonary syndrome occurred more frequently in cirrhosis (40%) than extrahepatic portal venous obstruction (13%) (P = 0.01). Cirrhotics with hepatopulmonary syndrome were symptomatic (85% vs. 14%, P < 0.001) and had longer disease duration [16(1-120) months vs. 28 (1-168) months, P = 0.01] as compared to cirrhotics without hepatopulmonary syndrome. Prevalence of hepatopulmonary syndrome among cirrhotics did not correlate with the severity of liver disease as assessed by both paediatric end-stage liver disease score and Child-Turcotte-Pugh score. Patients with extrahepatic portal venous obstruction and hepatopulmonary syndrome remain asymptomatic manifesting with clubbing alone (38%). Hepatopulmonary syndrome occurs more frequently in cirrhosis, a majority being symptomatic with more severity in comparison to extrahepatic portal venous obstruction having no symptoms and less severity. There seems to be a role of liver dysfunction besides portal hypertension as an added factor in the pathogenesis of hepatopulmonary syndrome.