Aortopathy Is Prevalent in Relatives of Bicuspid Aortic Valve Patients

Objectives This study aimed to determine the prevalence of dilation and abnormal elastic properties of aortic root in first-degree relatives (FDRs) of bicuspid aortic valve (BAV) patients. Background Evidence indicates that BAV is a genetic disorder. Although FDRs of affected individuals have an inc...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 2009-06, Vol.53 (24), p.2288-2295
Main Authors: Biner, Simon, MD, Rafique, Asim M., MD, Ray, Indraneil, MD, Cuk, Olivera, MD, Siegel, Robert J., MD, Tolstrup, Kirsten, MD
Format: Article
Language:English
Subjects:
Adult
Age
Analysis of Variance
aorta
Aortic Valve - abnormalities
Aortic Valve - diagnostic imaging
Aortic Valve - pathology
Aortic Valve Stenosis - epidemiology
Aortic Valve Stenosis - pathology
Biological and medical sciences
Blood pressure
Cardiology
Cardiology. Vascular system
Cardiovascular
Cardiovascular disease
Case-Control Studies
Confidence intervals
Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava
Coronary vessels
Dilatation, Pathologic - diagnostic imaging
Dilatation, Pathologic - epidemiology
Dilatation, Pathologic - pathology
echocardiography
Elasticity
Endocardial and cardiac valvular diseases
Female
genetics
Heart
Heart Defects, Congenital - diagnostic imaging
Heart Defects, Congenital - epidemiology
Heart Defects, Congenital - pathology
Heart Valve Diseases - diagnostic imaging
Heart Valve Diseases - epidemiology
Heart Valve Diseases - pathology
Humans
Hypertension
Internal Medicine
Male
Medical sciences
Middle Aged
Population
Prevalence
Prospective Studies
Risk Factors
Sinuses
Surgery
Ultrasonic imaging
Ultrasonography
United States - epidemiology
valves
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Summary:Objectives This study aimed to determine the prevalence of dilation and abnormal elastic properties of aortic root in first-degree relatives (FDRs) of bicuspid aortic valve (BAV) patients. Background Evidence indicates that BAV is a genetic disorder. Although FDRs of affected individuals have an increased prevalence of BAV, their risk of aortic root abnormalities is unknown. Methods We studied dimensions as well as the elastic properties of the ascending aorta in 48 FDRs with morphologically normal tricuspid aortic valves, 54 BAV patients, and 45 control subjects using 2-dimensional echocardiography. Results The prevalence of aortic root dilation was 32% in FDRs and 53% in BAV patients, whereas all control subjects showed normal aortic dimensions (p < 0.001). The FDRs and BAVs had significantly lower aortic distensibility (1.7 ± 1.4 × 10−3 mm Hg and 1.4 ± 2.0 × 10−3 mm Hg vs. 2.5 ± 1.6 × 10−3 mm Hg, p < 0.001) and greater aortic stiffness index (26.7 ± 25.8 and 55.9 ± 76.8 vs. 18.7 ± 40.1, p = 0.001) compared with control subjects. This difference remained significant in subjects without aortic root dilation or hypertension (p = 0.002 and p = 0.004, respectively). Conclusions The aortic root is functionally abnormal and dilation is common (32%) in first-degree relatives of patients with BAV. Screening of FDRs by transthoracic 2-dimensional echocardiography should be considered for detection of aortic valve malformation and dilated ascending aorta.
ISSN:0735-1097
1558-3597
DOI:10.1016/j.jacc.2009.03.027