遗传性出血性毛细血管扩张症患者的妊娠结局。

Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia.

机构信息

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.

出版信息

Obstet Gynecol. 2014 Mar;123(3):514-520. doi: 10.1097/AOG.0000000000000120.

DOI:10.1097/AOG.0000000000000120

PMID:24499751

Abstract

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.

摘要

目的

描述遗传性出血性毛细血管扩张症（HHT）女性的妊娠结局。

方法

这是一项回顾性描述性研究，使用电话问卷调查了来自多伦多 HHT 数据库的 HHT 女性（18-55 岁）的妊娠、分娩和新生儿结局。

结果

共有 87 名 HHT 女性报告了 244 次妊娠。流产率为 20%。与 HHT 相关的并发症包括两次妊娠期间出现轻微咯血（1.1%）和四次妊娠期间出现血胸（2.1%）。一名出现血胸的患者在之前的一次妊娠中曾出现短暂性脑缺血发作，很可能是由反常栓塞引起的。一名患者出现颅内出血，一名患者出现心力衰竭。这些并发症发生在未接受动静脉畸形筛查和治疗的女性中。其他与 HHT 不明确相关的并发症包括深静脉血栓形成（n=1）、肺栓塞（n=1）、心肌梗死（n=1）和心肌缺血（n=1）。女性在怀孕期间注意到鼻出血的频率增加和新的毛细血管扩张症的发展。在 185 次分娩中有 92 次（50%）使用了硬膜外或脊髓麻醉，没有出现并发症。这些女性在麻醉前均未进行脊髓动静脉畸形筛查。