PULMONARY-HYPERTENSION AS A FATAL COMPLICATION OF EXTRAHEPATIC PORTAL-HYPERTENSION

Pulmonary hypertension is an uncommon complication of portal hypertens ion 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. Fur ther investigations revealed high serum concentrations of prostaglandi n F-2a, thromboxane B-2 in the inferior vena cava, and angiotensin I i n the inferior vena cava and right ventricle. The increased levels of these vasoconstrictive substances strongly suggest that the possible m echanism for the pulmonary hypertension in such patients with extrahep atic portal hypertension include shunting of vasoactive agents from th e splanchnic circulation to the pulmonary vascular bed.