Share this article on:

Outcomes of Pregnancy in Women With Hereditary Hemorrhagic Telangiectasia

de Gussem, Els M. MD; Lausman, Andrea Y. MD, FRCPC; Beder, Aarin J. MD, CFPC; Edwards, Christine P. BSc; Blanker, Marco H. MD, PhD; Terbrugge, Karel G. MD, FRCPC; Mager, Johannes J. MD, PhD; Faughnan, Marie E. MD, MSc

doi: 10.1097/AOG.0000000000000120
Contents: Original Research

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

Women with hereditary hemorrhagic telangiectasia who have not been screened for arteriovenous malformations are at risk for serious pregnancy complications.Supplemental Digital Content is Available in the Text.

Toronto HHT Centre, Division of Respirology, Department of Medicine, and the Department of Obstetrics & Gynaecology and the Keenan Research Centre and the Li Ka Shing Knowledge Institute, St. Michael's Hospital, the Department of Surgery and Medical Imaging, the University of Toronto Brain Vascular Malformation Study Group, the Toronto Hospital, Western Division, and the Division of Respirology, Department of Medicine, University of Toronto, Toronto, Canada; and the Department of Pulmonology, St. Antonius Hospital, Nieuwegein, and the Department of General Practice, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands. Dr. de Gussem is currently affiliated with the Department of Internal Medicine, Faculty of Medicine, University of Manitoba, Winnipeg, Canada.

Corresponding author: Marie E. Faughnan, MD, MSc, St Michael's Hospital, 30 Bond Street, Toronto, Ontario M5B 1W8, Canada; e-mail: faughnanm@smh.ca.

Funding sources include SWORO (Dutch Foundation for scientific research on HHT) (E.M.d.G.), the Nelson Arthur Hyland Foundation (M.E.F.), the Keenan Research Centre (M.E.F.), and the Li Ka Shing Knowledge Institute (M.E.F.).

The authors thank Elaine Granatstein and Myra Slutsky for assistance in data collection.

Financial Disclosure The authors did not report any potential conflicts of interest.

© 2014 by The American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.