Severe pulmonary hypertension and multiple left coronary arterial fistulas in association with congenital hepatic fibrosis.

Multiple fistulous communications between the left anterior descending coronary artery and the left ventricle were found in a 55 year old woman with congenital hepatic fibrosis presenting with breathlessness. At cardiac catheterisation severe pulmonary hypertension was also found. In view of the persistent hypoprothrombinaemia, severe thrombocytopenia, and the multiple fistulas the risk of operation was thought to be unacceptable and she continues on medical treatment.