Hereditary hemorrhagic telangiectasia with pulmonary arteriovenous malformations

Background: Most congenital pulmonary arteriovenous malformations are assoc iated with hereditary hemorrhagic telangiectasia. During pregnancy, pulmona ry hemorrhage can occur, compromising maternal and fetal health. Cases: We studied three pregnancies in two women with hemorrhagic telangiec tasia complicated by pulmonary arteriovenous malformations. A 28-year-old p rimigravida's fetus died at 25 weeks' gestation, and she had embolotherapy with coil springs, which corrected the hypoxemic state. In a subsequent pre gnancy she delivered a healthy 2315-g infant at 38 weeks' gestation. A 19-y ear-old primigravida had spontaneous hemothorax at 26 weeks' gestation with severe hypoxemia and a growth-restricted fetus without umbilical artery di astolic now. 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 diastol ic flow improved, and she had term delivery of a healthy 2250-g infant. Conclusion: Antenatal diagnosis and treatment of women with hereditary hemo rrhagic telangectasia and pulmonary arteriovenous malformations might preve nt potentially life-threatening fetomaternal complications. (C) 2001 by The American College of Obstetricians and Gynecologists.