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Unicommissural aortic valve in neonates and its association with other congenital heart disease

Published online by Cambridge University Press:  19 August 2008

Ana C. Durán ,
Luciano Daliento ,
Carla Frescura ,
Giovanni Stellin ,
Valentín Sans-Coma ,
Annalisa Angelini ,
Ornella Milanesi  and
Gaetano Thiene
Ana C. Durán
Affiliation:
From the Departments of Pathology, Faculty of Science, University of Málaga, Málaga
Luciano Daliento
Affiliation:
Cardiology, Faculty of Science, University of Málaga, Málaga
Carla Frescura
Affiliation:
From the Departments of Pathology, Faculty of Science, University of Málaga, Málaga
Giovanni Stellin
Affiliation:
Cardiac Surgery, Faculty of Science, University of Málaga, Málaga
Valentín Sans-Coma
Affiliation:
University of Padua Medical School, Padua and the Department of Animal Biology, Faculty of Science, University of Málaga, Málaga
Annalisa Angelini
Affiliation:
From the Departments of Pathology, Faculty of Science, University of Málaga, Málaga
Ornella Milanesi
Affiliation:
Paediatrics, Faculty of Science, University of Málaga, Málaga
Gaetano Thiene*
Affiliation:
From the Departments of Pathology, Faculty of Science, University of Málaga, Málaga
*
Dr. Gaetano Thiene, Istituo di Anatomia Patologica, Universitá degli Studi di Padova, Via A. Gabelli, 61, 35121 Padova, Italy. Tel. and Fax. 39 49 8754086.
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Abstract

The morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.

Keywords

Congenital heart diseaseaortic valve stenosisaortic valve
Type
Original Articles
Information
Cardiology in the Young , Volume 5 , Issue 2 , April 1995 , pp. 132 - 139
Copyright
Copyright © Cambridge University Press 1995

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