ADULT CONGENITAL DISEASE
Morphology of bicuspid aortic valve in children and adolescents
Susan M. Fernandes, MHP, PA-C*,
Stephen P. Sanders, MD ,
Paul Khairy, MPH, MD*,
Kathy J. Jenkins, MD, MPH*,
Kimberlee Gauvreau, ScD*,
Peter Lang, MD*,
Hilary Simonds, MS* and
Steven D. Colan, MD*,*
* Department of Cardiology, Children's Hospital and the Department of Pediatrics, Harvard Medical School, Boston, Massachusetts
DMCCPOspedale Pediatrico Bambino GesùRome, Italy
Manuscript received February 11, 2004;
revised manuscript received April 16, 2004,
accepted May 18, 2004.
* Reprint requests and correspondence: Dr. Steven D. Colan, Harvard Medical School, Professor of Pediatrics, Children's Hospital, 300 Longwood Avenue, Boston, Massachusetts 02115 (Email: colan{at}alum.mit.edu).
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  2 m/s, moderate or greater aortic stenosis as 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 greaterwas 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  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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Abbreviations and Acronyms
| | BAV = bicuspid or bicommissural aortic valve | | CI = confidence interval | | OR = odds ratio |
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