Hereditary hemorrhagic telangiectasia and pregnancy.

Abstract

Hereditary hemorrhagic telangiectasia is a rare but life-threatening disease characterized by telangiectasias and arteriovenous malformations in different organs. When it is associated with pregnancy, this disease causes significant morbidity and mortality. We describe the pregnancy course of a primigravida with severe hereditary hemorrhagic telangiectasia. Her antepartum course was complicated by reduced pulmonary function, mild tricuspid regurgitation, and mild pulmonary hypertension. An amniocentesis was performed at the 32nd week for hydramnios. Because of a nonreassuring fetal heart rate pattern, she was delivered by cesarean of a female infant. At the beginning of the postpartum period, her pulmonary function spontaneously improved, and mother and female infant were discharged in good condition. Pregnancy is complicated in women with severe hereditary hemorrhagic telangiectasia, but can be managed successfully.