马凡综合征患者主动脉根部置换术后妊娠：一例病例报告及文献综述

Pregnancy in Marfan syndrome after aortic root replacement: a case report and review of the literature.

作者信息

Volach Vania, Elami Amir, Gilon Dan, Pollak Arthur, Ginosar Yehuda, Ezra Yossef

机构信息

Obstetrics and Gynecology, Hadassah Hebrew University Medical Center, Jerusalem, Israel.

出版信息

Congenit Heart Dis. 2006 Jul;1(4):184-8. doi: 10.1111/j.1747-0803.2006.00032.x.

DOI:10.1111/j.1747-0803.2006.00032.x

PMID:18377545

Abstract

OBJECTIVE

Marfan syndrome is an inheritable connective tissue disorder with multisystem involvement and variable expression of signs and symptoms, caused by mutations within the fibrillin gene on chromosome 15q21. The reported incidence of severe cardiovascular events during pregnancy in Marfan patients' series is 3-7%, and the overall risk of fatal complication is approximately 1%. Although acute aortic dissection is a rare complication, an association between pregnancy and aortic dissection has been reported even in the absence of preconception aortic root dilatation.

DESIGN

Case report and literature review.

RESULTS

A 29-year-old pregnant woman with Marfan syndrome after replacement of ascending aorta with aortic valve conservation. She had a normal pregnancy and was delivered at term by cesarean section.

CONCLUSION

Successful pregnancy and delivery can be achieved in Marfan's patients after aortic root replacement.

摘要

目的

马凡综合征是一种可遗传的结缔组织疾病，累及多系统，体征和症状表现多样，由15号染色体q21区原纤维蛋白基因突变引起。在马凡综合征患者系列报道中，孕期严重心血管事件的发生率为3% - 7%，致命并发症的总体风险约为1%。虽然急性主动脉夹层是一种罕见的并发症，但即使在孕前主动脉根部无扩张的情况下，也有妊娠与主动脉夹层相关的报道。