Question

Which of the following statements regarding aortic stenosis in children is correct?

• A. Subvalvular type is more common
• B. Can be associated with co-arctation of aorta
• C. S2 split is notable and non paradoxical
• D. Bicuspid aortic valve is rare and not familial

Hint:

Left-sided heart lesions cluster together! Valvular AS, Bicuspid Aortic Valve, Coarctation of Aorta, and Mitral stenosis are frequently co-associated (e.g., in Turner Syndrome and Shone complex).

Answer 1

The Correct Option is B

Concept:
Congenital aortic stenosis (AS) encompasses a spectrum of left ventricular outflow tract (LVOT) obstructions, anatomically categorized into valvular ($70\%$), subvalvular ($15$--$20\%$), and supravalvular ($5$--$10\%$) levels.
Valvular AS is the most common form in children, predominantly caused by a congenital bicuspid or dysplastic aortic valve.
Explanation:
• Congenital valvular aortic stenosis is frequently associated with other left-sided obstructive cardiac lesions, most notably Coarctation of the Aorta (found in up to $20$--$30\%$ of cases, as part of the Shone complex).

• Valvular aortic stenosis is the most common anatomical subtype, making the statement that subvalvular is more common incorrect.

• Severe aortic stenosis delays left ventricular ejection time, causing delayed closure of the aortic valve component ($A_2$) relative to the pulmonic component ($P_2$), leading to paradoxical (reversed) splitting of $S_2$ (splits during expiration and closes during inspiration).

• Bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly overall (prevalence of $1$--$2\%$) and exhibits high familial heritability (autosomal dominant pattern with variable penetrance, linked to NOTCH1 mutations).
Final Answer:
Congenital aortic stenosis is well known to be frequently associated with coarctation of the aorta.