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Aortic distensibility and dilation in Turner’s syndrome

Published online by Cambridge University Press:  27 October 2009

Jayendra Sharma ,
Deborah Friedman ,
Swati Dave-Sharma  and
Madeleine Harbison
Jayendra Sharma*
Affiliation:
Department of Pediatrics, Division of Cardiology
Deborah Friedman
Affiliation:
Department of Pediatrics, Division of Cardiology
Swati Dave-Sharma
Affiliation:
Endocrinology, New York Presbyterian Hospital – Cornell Medical Center, 525, East 68th street, New York, NY 10021
Madeleine Harbison
Affiliation:
Endocrinology, New York Presbyterian Hospital – Cornell Medical Center, 525, East 68th street, New York, NY 10021
*
Correspondence to: Jayendra Sharma, MD, FACC, Director, Pediatric Cardiology, Jamaica Hospital Medical Center, 8900, Van Wyck expressway, Cardiology Suite-First floor, Jamaica, NY 11418. Tel: 718-206-7138; Fax: 718-206-7144; E-mail: [email protected]
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Abstract

Background

Aortic dilation and dissection is reported in patients with Turner’s syndrome, both with and without cardiovascular risk factors. The bicuspid aortic valve is closely associated with dilated aortic root, although expression of aortic dilation is variable. The determinants for variable expression of aortic dilation in individuals with Turner’s syndrome, however, are unknown.

Hypothesis

A primary mesenchymal defect is prevalent in individuals with Turner’s syndrome, suggested by having abnormalities in bone matrix, and lymphatic and peripheral blood vessels. We hypothesize that an abnormal intrinsic elastic property of aorta is a forerunner of aortic dilation in Turner’s syndrome.

Objective

Assess utility of aortic distensibility as a measure of aortic elasticity for the stratification of the risk for aortic dilation, and its relationship with age, karyotype, and hormonal therapy.

Design

Prospective cross-sectional study.

Patients and method

We performed cross-sectional M-mode and Doppler echocardiography in 24 individuals with Turner’s syndrome. Dimensions of the aortic root, and its distensibility, were calculated using standard techniques. We also examined a control group of 24 age matched normotensive patients with structurally normal hearts, who had been referred for evaluation of cardiac murmurs or chest pain.

Results

Aortic dilation was the most common cardiac anomaly, seen in 11 of 24 (46%) individuals with Turner’s syndrome, and none in control group. Of these individuals, 5 without cardiovascular risk factors had aortic dilation. In 2 young girls, aortic dimensions were normal, albeit with reduced distensibility. Aortic dilation correlated inversely with aortic distensibility, but not with age, karyotype or hormonal therapy.

Conclusion

Individuals with Turner’s syndrome, even without cardiovascular risk factors, do develop aortic dilation accompanied by decreased aortic distensibility, suggestive of an intrinsic abnormality in elastic property of the ascending aorta.

Keywords

Aortic elasticityconnective tissue defectbicuspid aortic valveaortic dissection
Type
Original Articles
Information
Cardiology in the Young , Volume 19 , Issue 6 , December 2009 , pp. 568 - 572
Copyright
Copyright © Cambridge University Press 2009

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