Primary pulmonary hypertension in a child with primary sclerosing cholangitis

A child who suffered from primary sclerosing cholangitis presented with sev ere portal hypertension over years. At age eleven, symptoms of right ventri cular decompensation were noted. After stabilization, orthotopic liver tran splantation was performed and resulted in severe decompensation with an inc rease of right: Ventricular pressure up to 95 mmHg. At further investigatio n, primary pulmonary hypertension with plexiform pulmonary arteriopathy was diagnosed. After ten months under oxygen and nifedipine treatment, the pat ient reached a stable cardiopulmonary state comparable to the pretransplant period fright ventricular pressure 45 mmHg) but did not improve further. Discussion: Primary pulmonary hypertension in children can occur in associa tion with severe portal hypertension. As this complication will considerabl y alter prognosis and management of children with chronic liver disorders, it must be actively searched for and monitored with regular EGG, chest X-ra y and echocardiography. To prove the diagnosis and evaluate treatment respo nse, cardiac catheterization is required.