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Analysis of coronary flow haemodynamics in homozygous familial hypercholesterolaemic adolescents with aortic supravalvular stenosis

Published online by Cambridge University Press:  30 May 2012

Zheng Wang ,
Ya Yang ,
Zhi'an LI ,
Xiaoshan Zhang ,
Jie Lin  and
Lvya Wang
Zheng Wang
Affiliation:
Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
Ya Yang*
Affiliation:
Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
Zhi'an LI
Affiliation:
Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China
Xiaoshan Zhang
Affiliation:
Department of Ultrasound, Affiliated Hospital of Inner Mongolia Medical College, Hohehot, Inner Mongolia Municipality, China
Jie Lin
Affiliation:
The Key Laboratory of Remodeling-related Cardiovascular Diseases, Beijing Anzhen Hospital, Capital Medical University, Ministry of Education, Beijing, China
Lvya Wang
Affiliation:
The Key Laboratory of Remodeling-related Cardiovascular Diseases, Beijing Anzhen Hospital, Capital Medical University, Ministry of Education, Beijing, China
*
Correspondence to: Dr Ya Yang, Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. Tel: +86 010 64456450; Fax: +86 010 64456447; E-mail: yayang0912@126.com
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Abstract

Objective

To study coronary artery haemodynamics in adolescents with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis.

Methods

Patients diagnosed with familial hypercholesterolaemia who were younger than 16 years and who had undergone transthoracic echocardiography from 2007 to 2010 were included in this study. We included patients with homozygous familial hypercholesterolaemia and aortic supravalvular stenosis and those with heterozygous familial hypercholesterolaemia. All patients underwent stress echocardiography, and left anterior descending coronary artery flow was successfully detected. Coronary flow velocity reserve was calculated as the ratio of hyperaemic mean diastolic flow velocity after injection of adenosine to basal mean diastolic flow velocity. Changes in coronary haemodynamics and the relationship between lipid concentrations were determined.

Results

A total of 11 patients with homozygous familial hypercholesterolaemia were enrolled in this study. Lipid concentrations were measured, and the mean coronary flow velocity reserve was 1.97 plus or minus 0.51. Seven children were included in the group of patients with heterozygous familial hypercholesterolaemia. In these children, the mean coronary flow velocity reserve was 3.08 plus or minus 0.84.

Conclusion

The coronary flow velocity reserve of homozygous familial hypercholesterolaemic patients is lower than that of heterozygous familial hypercholesterolaemic patients, and it is associated with a high concentration of low-density lipoprotein cholesterol. Aortic stenosis and plaques compromised the ostia of the coronary artery and caused increased basal mean diastolic coronary velocity with blunted increase in peak velocity, which decreased the coronary flow velocity reserve.

Keywords

Coronary flow velocity reservetransthoracic echocardiographylipid concentrationbasal mean diastolic velocity
Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 2 , April 2013 , pp. 219 - 224
Copyright
Copyright © Cambridge University Press 2012 

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