Pulmonary hypertension as a fatal complication of extrahepatic portal hypertension.

Pulmonary hypertension is an uncommon complication of portal hypertension seen in cirrhotic as well as noncirrhotic patients. We report a 10-year-old girl who presented with extrahepatic portal hypertension and pulmonary hypertension in the absence of intrinsic liver disease. Further investigations revealed high serum concentrations of prostaglandin F2 alpha, thromboxane B2 in the inferior vena cava, and angiotensin I in the inferior vena cava and right ventricle. The increased levels of these vasoconstrictive substances strongly suggest that the possible mechanism for the pulmonary hypertension in such patients with extrahepatic portal hypertension include shunting of vasoactive agents from the splanchnic circulation to the pulmonary vascular bed.