Severe pulmonary hypertension associated with hepatic arteriovenous malformation in a patient with hereditary haemorrhagic telangiectasia

We report a case of 46-year-old Asian woman with a history of recurrent epistaxis who presented with dyspnoea on exertion. Physical examination revealed mucocutaneous telangiectasias and signs of heart failure. Further evaluation showed huge hepatic arteriovenous malformation and severe pulmonary hypertension. This case demonstrates an uncommon manifestation of hereditary haemorrhagic telangiectasia presented with severe pulmonary hypertension.

Regression of a Large Congenital Hepatic Arteriovenous Malformation

Congenital hepatic arteriovenous malformations are rarely seen in association with persistent neonatal pulmonary hypertension. We report the case of a full-term female newborn who presented with heart failure and respiratory distress soon after birth. Echocardiographic investigation revealed severe persistent pulmonary hypertension and patent ductus arteriosus. Here we report spontaneous regression in size of both the feeder vessel and the vascular bed of the congenital hepatic arteriovenous malformation. We postulate that our conservative use of oral heart failure therapy, in the form of diuretic agents and captopril, decreased the congestion and diameter of the affected vessels.