OBJECTIVE: To describe pregnancy outcomes in women with hereditary hemorrhagic telangiectasia (HHT).
METHODS: This was a retrospective descriptive study of women with HHT (18–55 years of age) from the Toronto HHT Database using a telephone questionnaire regarding pregnancy, delivery, and neonatal outcomes.
RESULTS: A total of 244 pregnancies were reported in 87 women with HHT. Miscarriages occurred in 20%. Hereditary hemorrhagic telangiectasia–related complications included minor hemoptysis during two pregnancies (1.1%) and hemothorax during four pregnancies (2.1%). One patient presenting with a hemothorax had presented during a previous pregnancy with a transient ischemic attack, most likely resulting from paradoxical emboli. One patient presented with an intracranial hemorrhage, and one patient presented with heart failure. These complications occurred in women previously unscreened and untreated for arteriovenous malformations. Other complications not clearly related to HHT were deep vein thrombosis (n=1), pulmonary embolism (n=1), myocardial infarction (n=1), and myocardial ischemia (n=1). Women noticed an increased frequency of epistaxis and development of new telangiectases during pregnancy. Epidural or spinal anesthesia was performed in 92 of 185 deliveries (50%) without complications. None of these women had undergone screening for spinal arteriovenous malformation before anesthesia.
CONCLUSION: Women with HHT who have not been screened for arteriovenous malformations are at risk for serious pregnancy complications.
LEVEL OF EVIDENCE: III