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Bicuspid aortic valve morphology and associated cardiovascular abnormalities in fetal Turner syndrome: a pathomorphological study.
- Source :
-
Fetal diagnosis and therapy [Fetal Diagn Ther] 2014; Vol. 36 (1), pp. 59-68. Date of Electronic Publication: 2014 Jun 04. - Publication Year :
- 2014
-
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.<br />Material and Methods: We studied post-mortem heart specimens of 36 TS fetuses and 1 TS newborn.<br />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.<br />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.
- Subjects :
- Aortic Valve embryology
Aortic Valve pathology
Bicuspid Aortic Valve Disease
Cardiovascular Abnormalities embryology
Cardiovascular Abnormalities pathology
Female
Fetal Heart embryology
Heart embryology
Heart Valve Diseases embryology
Humans
Infant, Newborn
Turner Syndrome embryology
Aortic Valve abnormalities
Fetal Heart abnormalities
Fetal Heart pathology
Heart Valve Diseases pathology
Turner Syndrome pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1421-9964
- Volume :
- 36
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Fetal diagnosis and therapy
- Publication Type :
- Academic Journal
- Accession number :
- 24903004
- Full Text :
- https://doi.org/10.1159/000357706