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July 7, 2026Journal of the American College of Cardiology255 citations

Morphology of bicuspid aortic valve in children and adolescents

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SFSusan M. FernandesSSStephen P. SandersPKPaul Khairy

Key Result

Right-coronary and non-coronary leaflet fusion in bicuspid aortic valve was associated with a higher risk of moderate or greater aortic stenosis (OR 2.4; 95% CI 1.6-3.6; p<=0.001).

Key Points

  • The aim is to evaluate the morphological characteristics of bicuspid aortic valves in a younger population.
  • Conducted echocardiographic assessments to analyze valvular structure.
  • Examined children and adolescents with diagnosed bicuspid aortic valves.
  • Identified distinct morphological features associated with bicuspid aortic valves.
  • Observed variations in valve function and structure across different age groups.

Study Design

Type

Observational (n=1,135)

PICO

P
Population
1,135 children and adolescents (median age 3 years, 33% female) with bicuspid aortic valve identifiable via echocardiography.
E
Exposure / Comparator
Right-coronary and non-coronary leaflet fusion vs Other leaflet fusion types
O
Primary Outcome
Moderate or greater aortic stenosis — OR 2.4 (1.6 to 3.6), p=<=0.001

Main Result

Odds Ratio: 2.4 (95% CI 1.6–3.6)

p-value: p=<=0.001

Abstract

OBJECTIVES: The aim of this study was to determine the relationship between aortic valve morphology and valve dysfunction. BACKGROUND: The morphology of the bicuspid or bicommissural aortic valve (BAV) may predict the severity of valve dysfunction. Therefore, we assessed the relationship between BAV, aortic coarctation, and the degree of valve pathology in children. METHODS: A retrospective review of 1,135 patients with BAV who were identified between 1986 and 1999 was performed. Patients younger than 18 years of age with BAV that was identifiable via echocardiography were included. The most recent or last study of each patient before intervention or endocarditis was reviewed. Mild stenosis was defined as a valve gradient > or =2 m/s, moderate or greater aortic stenosis as > or =3.5 m/s. Aortic regurgitation was quantified using standard criteria. RESULTS: Median age was 3 years (range, 1 day to 17.9 years), and 67% of the patients were male. Right-coronary and left-coronary leaflet fusion were the most common types of BAV (70%). Aortic stenosis that was moderate or greater was observed most often in patients with right-coronary and non-coronary leaflet fusion (odds ratio 2.4, 95% confidence interval 1.6 to 3.6; p < or = 0.001). Similarly, right-coronary and non-coronary leaflet fusion was more often associated with moderate aortic regurgitation or greater (odds ratio 2.4, 95% confidence interval 1.2 to 4.7; p = 0.01). The majority of patients with aortic coarctation had fusion of the right-coronary and left-coronary leaflets (89%), and aortic coarctation was associated with lesser degrees of valve stenosis or regurgitation. CONCLUSIONS: Analysis of BAV morphology is of clinical and prognostic relevance. Fusion of the right-coronary and non-coronary leaflets was associated with more significant valve pathology, whereas fusion of the right-coronary and left-coronary leaflets was associated overwhelmingly with aortic coarctation and less aortic valve pathology.

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Cite This Study

Fernandes et al. (2004) conducted an observational in Bicuspid aortic valve (n=1,135). Right-coronary and non-coronary leaflet fusion vs. Other leaflet fusion types was evaluated on Moderate or greater aortic stenosis (OR 2.4, 95% CI 1.6 to 3.6, p=<=0.001). Right-coronary and non-coronary leaflet fusion in bicuspid aortic valve was associated with a higher risk of moderate or greater aortic stenosis (OR 2.4; 95% CI 1.6-3.6; p<=0.001).

synapsesocial.com/papers/6a4cc494b44acf1e20daf233https://doi.org/10.1016/j.jacc.2004.05.063
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