Should We Worry About Bicuspid Aortic Valve?

Discussion The aortic valve usually has 3 leaflets. In bicuspid aortic valve (BAV) there are 2 asymmetric leaflets with a fish-mouthed orifice between them which may not open fully. It occurs in about 0.5-2% of the population making it one of the most common congenital heart anomalies and the most common one in adults. Transmission is autosomal dominant yet males are more likely to have BAV, indicating potential reduced penetrance in females. “BAVs are different, however, in that the tissue pathology is not limited to the valves’ leaflets but extends from the left ventricular outflow tract to the ascending thoracic aorta” and therefore can be associated with other congenital heart disease. With BAV, a murmur may be heard which classically is a grade 1-2/6 systolic ejection murmur best at the right upper sternal border associated with an early systolic click. BAV may not be clinically apparent though. Learning Point BAV is usually considered a benign condition as it may not cause any problems. However as patients age, BAV does have an increased risk of aortic valve stenosis (AVS) and/or aortic valve insufficiency (AVI), calcification of the leaflets, aortic root dilatation, aortic aneurysm, aortic dissection and can also be a nidus for infective endocarditis. AVS is associated with BAV in 70-85% of pediatric patients and about 50% of adult patients. AVS can cause left ventricular outlet obstruction with compensatory ventricular hypertrophy and potentially ca...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

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