Bicuspid Aortic Valve Characteristics in Children

Background- Bicuspid aortic valve (BAV) is the most common congenital heart disease and the most common malformation of aortic valve. In BAV, there are two cusps instead of three cusps in the aortic valve. The objectives of this study were the determination of the aortic root dilatation and other anatomic and hemodynamic characteristics and abnormalities of BAV. Methods- Thirty patients and 30 control subjects were evaluated. Aortic root dimensions were measured via two-dimensional echocardiography (2-D echo) at 4 levels, including the aortic valve annulus, sinuses of Valsalva, sinotubular junction (STJ) and proximal ascending aorta (AAO). Hemodynamic data and anatomic characteristics were measured using 2D and Doppler-echo. All the findings were matched and indexed for body surface area (BSA) and were compared with the matched data of the control subjects. Clinical and demographic findings of BAV were also determined and collected through a questionnaire. Results- Among the patients, 70% were male and the mean age and weight of the patients were 7.5 years and 22.13 kg, respectively. 86.66% of the patients had systolic ejection murmur (SEM), 76.66% systolic ejection click (SEC) and 10% had chest pain. Other congenital heart diseases (CHD) were found in 26.96% of the patients, including coarctation of the aorta (CoA) in 23% of the cases. Matched mean anatomic aortic valve area (AAVA) was 2.05cm2/m2 , and matched mean effective aortic valve area (EAVA) was 1.41cm2/m2 BSA. Maximum aortic valve pressure gradient (PG max) in systole was 56.56mmHg. Forty percent of the patients had aortic stenosis (AS): mild AS in 16.66%, moderate AS in 13.33% and intermediate AS in 10%. Prevalence of aortic insufficiency (AI) was 36.68%. When the data were compared with the control subjects, all the patients showed a meaningful larger aortic root dimension at all 4 levels (P values are presented in Table IV). Aortic root dilation was at the level of the annulus, sinuses of Valsalva, STJ and proximal AAO in 6.25%, 4.75%, 10.20% and 10.13%, respectively. Conclusion- These findings support the hypothesis that BAV and aortic root dilation may reflect a common developmental defect. AS and AI are common in BAV. Similar to other obstructive defects of the left heart, BAV is significantly more common in males. Because murmurs and clicks are common in BAV even without AS or AI, all patients with a heart murmur and/or click must be evaluated for BAV (Iranian Heart Journal 2008; 9 (1):40 -46).