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复杂且成功的马凡综合征孕妇症状性孤立性腹主动脉瘤的管理。

Complex and Successful Management of a Symptomatic Isolated Abdominal Aortic Aneurysm in a Pregnant Woman with Marfan Syndrome.

机构信息

Department of Surgery, Section of Vascular Surgery and Endovascular Therapy, 42559Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Department of Radiology, Section of Interventional Radiology, 42559Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

出版信息

Vasc Endovascular Surg. 2022 Jul;56(5):529-534. doi: 10.1177/15385744221087820. Epub 2022 Apr 22.

Abstract

Aortopathies associated to Marfan syndrome (MFS) are important causes of maternal death during pregnancy. We present a 27-year-old and 24-week pregnant MFS woman who arrived to the emergency department with increasing abdominal pain; an obstetric ultrasound showed an Abdominal Aortic Aneurysm (AAA), a multislice computed tomography angiography (CTA) confirmed and demonstrated a 7.3 centimeter (cm) infrarenal AAA without evidence of dissection. A multidisciplinary committee determined that an open repair would lead to a significantly high maternal-fetal morbidity and mortality. Although endovascular repair (EVAR) in MFS patients remains controversial, an urgent bridge therapy was considered to be the best option. She was transferred to the angiography suite for EVAR to prevent AAA rupture and ensure a satisfactory pregnancy. The patient and fetus presented no complications during the procedure and were discharged 3 days later. She continued her pregnancy without eventualities and an elective C-section was performed on week 36. A CTA imaging at 12 months revealed type 1A and 3 endoleaks, we decided to perform endograft explant and a definitive open repair, there were no complications during the procedure, the patient is currently asymptomatic. Our case illustrates a complex decision and management that successfully avoid morbidity and mortality of a MFS mother and her product; additionally, this experience reinforces the need for lifelong and close surveillance in these patients.

摘要

马凡综合征(MFS)相关的主动脉病变是妊娠期间导致母亲死亡的重要原因。我们报告了一位 27 岁、妊娠 24 周的 MFS 女性,因腹痛加剧而到急诊科就诊;产科超声显示为腹主动脉瘤(AAA),多层螺旋 CT 血管造影(CTA)证实并显示了一个 7.3 厘米的肾下 AAA,没有夹层的证据。一个多学科委员会确定,开放修复会导致母亲和胎儿的发病率和死亡率显著增加。尽管 MFS 患者的血管内修复(EVAR)仍然存在争议,但紧急桥接治疗被认为是最佳选择。她被转至血管造影室进行 EVAR,以防止 AAA 破裂并确保妊娠顺利。患者和胎儿在手术过程中均未出现并发症,并于 3 天后出院。她继续妊娠,没有出现任何意外,并在第 36 周进行了选择性剖宫产。12 个月时的 CTA 成像显示 1A 型和 3 型内漏,我们决定进行血管内移植物取出和确定性开放修复,手术过程中无并发症,患者目前无症状。我们的病例说明了一个复杂的决策和管理过程,成功地避免了 MFS 母亲及其胎儿的发病率和死亡率;此外,这一经验强调了对这些患者进行终身和密切监测的必要性。

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