PORTAL-HYPERTENSION AND PULMONARY-HYPERTENSION - REPORT OF 3 NEW PEDIATRIC CASES

Pulmonary hypertension is a rare complication of portal hypertension, mainly in the pediatric age. We report 3 new pediatric cases of this this association, in which the cause of portal hypertension was portal vein obstruction in 2 and biliary cirrhosis in 1. One patient had been treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The clinical picture is highly variable and may evolve rapidly, as in our first patient, or more slowly, as in the other 2 cases, in which pulmonary hypertension is still relatively well tolerated. The diagnosis of pulmonary hypertension has been done by Doppler echocardiography. The therapeutic attempts of this complication by the use of vasodilators or beta-blocking agents have been until now unsatisfactory in the majority of patients including two of our 3 cases.