Bicuspid aortic valve morphology and associated cardiovascular abnormalities in fetal Turner syndrome: a pathomorphological study.
Fetal Diagn Ther
; 36(1): 59-68, 2014.
Article
em En
| MEDLINE
| ID: mdl-24903004
ABSTRACT
INTRODUCTION:
Bicuspid aortic valve (BAV) is common in Turner syndrome (TS). In adult TS, 82-95% of BAVs have fusion of the right and left coronary leaflets. Data in fetal stages are scarce. The purpose of this study was to gain insight into aortic valve morphology and associated cardiovascular abnormalities in a fetal TS cohort with adverse outcome early in development. MATERIAL ANDMETHODS:
We studied post-mortem heart specimens of 36 TS fetuses and 1 TS newborn.RESULTS:
BAV was present in 28 (76%) hearts. BAVs showed fusion of the right and left coronary leaflet (type 1 BAV) in 61%, and fusion of the right coronary and non-coronary leaflet (type 2 BAV) in 39%. There were no significant differences in occurrence of additional cardiovascular abnormalities between type 1 and type 2 BAV. However, all type 2 BAV hearts showed ascending aorta hypoplasia and tubular hypoplasia of the B segment, as opposed to only 55 and 64% of type 1 BAV hearts, respectively.DISCUSSION:
The proportion of type 2 BAV seems higher in TS fetuses than in adults. Fetal type 2 BAV hearts all had severe aortic pathology, possibly contributing to a worse prognosis of type 2 than type 1 BAV in TS.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Valva Aórtica
/
Síndrome de Turner
/
Coração Fetal
/
Doenças das Valvas Cardíacas
Tipo de estudo:
Risk_factors_studies
Limite:
Female
/
Humans
/
Newborn
Idioma:
En
Revista:
Fetal Diagn Ther
Assunto da revista:
DIAGNOSTICO POR IMAGEM
/
OBSTETRICIA
/
PERINATOLOGIA
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Holanda