| Literature DB >> 31879971 |
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.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