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Published on: December 13, 2019
Aortic stiffness and left ventricular diastolic function in children with well-functioning bicuspid aortic valves
Constance G Weismann1, Kristin C Lombardi2, Bernhard S Grell3
1Division of Pediatric Cardiology, Department of Pediatrics, Yale School of Medicine, 333 Cedar Street, LLCI 302, New Haven, CT 06510, USA constance.weismann@yale.edu.
Insights
Children with bicuspid aortic valves (BAV) show abnormal aortic elasticity and diastolic function. However, this study found no direct relationship between these aortic abnormalities and diastolic dysfunction in pediatric BAV patients.
Area of Science:
- Cardiovascular Medicine
- Pediatric Cardiology
- Echocardiography
Background:
- Bicuspid aortic valves (BAV) are associated with abnormal aortic stiffness and diastolic function in adults.
- Understanding these abnormalities in children with BAV is crucial for early detection and management.
Purpose of the Study:
- To investigate the relationship between aortic stiffness and left ventricular (LV) diastolic impairment in children with isolated, well-functioning BAV.
- To compare aortic elasticity and LV diastolic function in children with BAV to healthy controls.
Main Methods:
- Retrospective review of echocardiograms from 50 children with isolated BAV and 50 age-matched healthy controls.
- Analysis of LV systolic and diastolic function, ascending aortic stiffness index (SI), distensibility, and strain.
- Comparison of parameters between the BAV and control groups.
Main Results:
- Children with BAV exhibited significantly lower LV diastolic function parameters compared to controls.
- All measured parameters of proximal and distal ascending aortic elasticity were abnormal in the BAV group.
- No significant correlation was found between aortic elasticity parameters and LV diastolic function.
Conclusions:
- Pediatric patients with isolated bicuspid aortic valves demonstrate impaired aortic elasticity and diastolic function, even without significant congenital heart disease.
- Despite these co-existing abnormalities, a direct relationship between aortic elasticity and diastolic function could not be established in this pediatric cohort.
Aims:
Aortic stiffness and diastolic function are abnormal in adults with bicuspid aortic valves (BAVs). The goal of this study was to determine the relationship between aortic stiffness and left ventricular (LV) diastolic impairment in children with well-functioning BAV and no associated congenital heart disease.
Methods And Results:
This is a retrospective review of echocardiograms in children with isolated BAV (group BAV; N = 50) and healthy frequency-matched controls (group Control; N = 50). We analysed LV systolic and diastolic function, proximal and distal ascending aortic stiffness index (SI), distensibility, and strain. Age range was 0.2-20 (median 11) years. There was no significant difference in blood pressure, normalized LV size and systolic function between the groups. Several parameters of LV diastolic function were lower in group BAV compared with group Control (e.g. septal E': BAV 12 ± 2.3 cm/s; Control 13.5 ± 1.8 cm/s, P < 0.001). All parameters of proximal and distal ascending aortic elasticity were abnormal in group BAV vs. Control (SI proximal ascending aorta: BAV 4.2 ± 1.6; Control 3.0 ± 0.9, P < 0.001). There was no significant correlation between parameters of aortic elasticity and diastolic function. In a subgroup analysis of children with fusion of the right-non vs. right-left coronary cusps, there was no significant difference for any of the parameters analysed.
Conclusion:
Even children with well-functioning isolated BAV have abnormalities in aortic elasticity and diastolic function when compared with the Control group. However, a relationship between the two could not be established.
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The AV valves prevent the backflow of blood from the ventricles to the atria during ventricular contraction. These valves function with the assistance of the chordae tendineae and papillary muscles. When the ventricles are relaxed, the chordae tendineae are slack, allowing blood to flow from the atria into the...

