Andorfer Kornelia E C, Seebauer Caroline T, Dienemann Carolin, Marcrum Steven C, Fischer René, Bohr Christopher, Kühnel Thomas S
Department of Otorhinolaryngology, Regensburg University Medical Center, Franz-Josef-Strauß-Allee 11, 93053 Regensburg, Germany.
J Clin Med. 2022 Apr 13;11(8):2178. doi: 10.3390/jcm11082178.
Appropriate management of hereditary hemorrhagic telangiectasia (HHT) is of particular importance in females, as HHT-mediated modifications of the vascular bed and circulation are known to increase the risk of complications during pregnancy and delivery. This study was undertaken to evaluate female HHT patients' awareness of and experience with HHT during pregnancy and delivery, with a focus on epistaxis. In this retrospective study, 46 females (median age: 60 years) with confirmed HHT completed a 17-item questionnaire assessing knowledge of HHT and its pregnancy-associated complications, the severity of epistaxis during past pregnancies and deliveries, and the desire for better education and counselling regarding HHT and pregnancy. Results revealed that 85% of participants were unaware of their disease status prior to the completion of all pregnancies. Further, 91% reported no knowledge of increased pregnancy-related risk due to HHT. In regard to epistaxis, 61% of respondents reported experiencing nosebleeds during pregnancy. Finally, approximately a third of respondents suggested that receiving counseling on the risks of HHT in pregnancy could have been helpful. Findings suggest that awareness of HHT and its potential for increasing pregnancy-related risk is poor. Best practices in HHT management should be followed to minimize negative effects of the disorder.
遗传性出血性毛细血管扩张症(HHT)的恰当管理对女性尤为重要,因为已知HHT介导的血管床和循环改变会增加妊娠和分娩期间并发症的风险。本研究旨在评估女性HHT患者在妊娠和分娩期间对HHT的认知及经历,重点关注鼻出血。在这项回顾性研究中,46名确诊为HHT的女性(中位年龄:60岁)完成了一份包含17个条目的问卷,该问卷评估了对HHT及其妊娠相关并发症的了解、既往妊娠和分娩期间鼻出血的严重程度,以及对有关HHT和妊娠的更好教育及咨询的需求。结果显示,85%的参与者在完成所有妊娠之前并不知晓自己的疾病状况。此外,91%的参与者表示不了解HHT会增加与妊娠相关的风险。关于鼻出血,61%的受访者报告在怀孕期间出现过鼻出血。最后,约三分之一的受访者表示,若能接受关于HHT妊娠风险的咨询可能会有所帮助。研究结果表明,对HHT及其增加妊娠相关风险的可能性的认知较差。应遵循HHT管理的最佳实践,以尽量减少该疾病的负面影响。