Literature DB >> 31879971

Prenatal diagnosis of Marfan syndrome by fetal echocardiography: A case report and review of cardiovascular manifestations.

Xin Wang1,2, Lin Sun1,2, Xiao-Wei Liu1,2, Jian-Feng Shang3, Yi-Hua He1,2.   

Abstract

Neonatal Marfan syndrome (nMFS), phenotypically and genotypically distinct from the classical syndrome, is rarely diagnosed prenatally, and the cardiovascular prognosis is poor. This case report described one fetus diagnosed with nMFS by fetal echocardiography. The main features were cardiomegaly, and atrioventricular valves prolapse with moderate regurgitation and dilated great vessels. Extracardiac malformations included right diaphragmatic eventration, bilateral pyelectasis, and lengthy femur. Pathological examination confirmed the findings of fetal echo, and all cardiac valves were dysplastic. Sanger sequencing revealed a deletion mutation affecting exon 30 of the fibrillin 1 (FBN1) gene. Echocardiography is essential for prenatal diagnosis, and multivalve dysplasia is common among those patients.
© 2019 Wiley Periodicals, Inc.

Entities:  

Keywords:  Marfan syndrome; fetal echocardiography; prenatal diagnosis

Mesh:

Substances:

Year:  2019        PMID: 31879971     DOI: 10.1111/echo.14577

Source DB:  PubMed          Journal:  Echocardiography        ISSN: 0742-2822            Impact factor:   1.724


  2 in total

Review 1.  [Latest advances in the diagnosis and treatment of Marfan syndrome].

Authors:  Shu-Ting Yang; Fang Luo
Journal:  Zhongguo Dang Dai Er Ke Za Zhi       Date:  2022-07-15

2.  Functional Analysis of an Intronic FBN1 Pathogenic Gene Variant in a Family With Marfan Syndrome.

Authors:  Kui Hu; Yun Wan; Fu-Tsuen Lee; Jinmiao Chen; Hao Wang; Haonan Qu; Tao Chen; Wang Lu; Zhenwei Jiang; Lufang Gao; Xiaojuan Ji; Liqun Sun; Daokang Xiang
Journal:  Front Genet       Date:  2022-04-25       Impact factor: 4.772

  2 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.