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遗传性出血性毛细血管扩张症伴心房间隔缺损及重度妊娠时肺动脉高压:病例报告及文献复习。

Hereditary haemorrhagic telangiectasia with atrial septal defect and pulmonary hypertension during advanced pregnancy: a case report and literature review.

机构信息

Department of Gynaecology and Obstetrics, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei Province, China.

Department of Pain, Cangzhou Hospital of Integrated TCM-WM Hebei, Cangzhou, Hebei Province, China.

出版信息

J Int Med Res. 2022 Mar;50(3):3000605221085427. doi: 10.1177/03000605221085427.

DOI:10.1177/03000605221085427
PMID:35380076
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9052829/
Abstract

Pregnancy complicated with hereditary haemorrhagic telangiectasia (HHT) is a rare condition. This case report presents an extremely rare case with the co-occurrence of HHT and congenital heart disease. In this report, a 43-year-old woman at 36 + 4 weeks of gestation experienced haemoptysis with a volume of approximately 300 ml for the first time. Uncommonly, her transthoracic echocardiogram revealed a previously unrecognized atrial septal defect (ASD) and pulmonary hypertension (PH) for the first time at 36 + 1 weeks of gestation. Chest computed tomography revealed an arteriovenous malformation (AVM) in the right lower lobe of the lung. Due to concerns of rebleeding of ruptured pulmonary arteriovenous malformations (PAVMs), the patient underwent a caesarean section at 36 + 6 weeks of gestation. A healthy male infant weighing 2800 g was delivered. To the best of our knowledge, there have been few reports about HHT with ASDs and PH during advanced pregnancy. This current case report highlights the necessity for clinicians to pay considerable attention to cardiac structural abnormalities, which can worsen PAVM in patients with HHT during pregnancy, for whom terminating the pregnancy in time may reduce the risk of PAVM rupture.

摘要

妊娠合并遗传性出血性毛细血管扩张症(HHT)较为罕见。本病例报告介绍了一种极其罕见的 HHT 合并先天性心脏病病例。本病例报告中,一名 43 岁女性,孕 36+4 周,首次出现约 300ml 的咯血。不同寻常的是,她在孕 36+1 周时首次经胸超声心动图检查发现了先前未被识别的房间隔缺损(ASD)和肺动脉高压(PH)。胸部计算机断层扫描显示右下肺动静脉畸形(AVM)。由于担心破裂的肺动静脉畸形(PAVM)再次出血,患者在孕 36+6 周时行剖宫产术,娩出一名 2800g 的健康男婴。据我们所知,妊娠晚期 HHT 合并 ASD 和 PH 的病例报告较少。本病例报告强调了临床医生需要高度关注心脏结构异常的必要性,这可能会使 HHT 患者的 PAVM 在妊娠期间恶化,及时终止妊娠可能会降低 PAVM 破裂的风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/dfac4d8ced04/10.1177_03000605221085427-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/ed318c6a1e70/10.1177_03000605221085427-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/5696c6787795/10.1177_03000605221085427-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/78f37d07800f/10.1177_03000605221085427-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/dfac4d8ced04/10.1177_03000605221085427-fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/ed318c6a1e70/10.1177_03000605221085427-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/5696c6787795/10.1177_03000605221085427-fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/78f37d07800f/10.1177_03000605221085427-fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a753/9052829/dfac4d8ced04/10.1177_03000605221085427-fig4.jpg

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