Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations

Most congenital pulmonary arteriovenous malformations are associated with hereditary hemorrhagic telangiectasia. During pregnancy, pulmonary hemorrhage can occur, compromising maternal and fetal health.

We studied three pregnancies in two women with hemorrhagic telangiectasia complicated by pulmonary arteriovenous malformations. A 28-year-old primigravida's fetus died at 25 weeks' gestation, and she had embolotherapy with coil springs, which corrected the hypoxemic state. In a subsequent pregnancy she delivered a healthy 2315-g infant at 38 weeks' gestation. A 19-year-old primigravida had spontaneous hemothorax at 26 weeks' gestation with severe hypoxemia and a growth-restricted fetus without umbilical artery diastolic flow. Pulmonary arteriovenous malformation was diagnosed by computed tomography of the maternal lung. She had continued pulmonary bleeding, so emergency lung lobectomy was done. Maternal hypoxemia and umbilical diastolic flow improved, and she had term delivery of a healthy 2250-g infant.

Antenatal diagnosis and treatment of women with hereditary hemorrhagic telangectasia and pulmonary arteriovenous malformations might prevent potentially life-threatening fetomaternal complications.