Bicuspid aortic valve in infants, children, and adolescents: A review for primary care providers

1. Bicuspid aortic valve (BAV) is the most common congenital heart defect in children, adolescents, and adults, present in 0.5% to 2% of individuals. (1) 2. Most patients with BAV are asymptomatic and are diagnosed due to a murmur or click on examination or due to family screening. Rarely, patients with BAV and severe disease are symptomatic. 3. BAV can lead to many complications, including aortic valve stenosis and/or regurgitation, endocarditis, and dilation of the aorta; 12% to 15% of pediatric patients with BAV may require intervention before adulthood. (6) 4. Based on American Heart Association guidelines, all first-degree family members of a patient with BAV should have echocardiographic screening, even if they have normal physical examination findings. (42) 5. Based on consensus, patients with BAV need regular pediatric cardiology and surveillance echocardiography follow-up. The frequency of this follow-up depends on valve function and aortic dimensions. 6. Based on consensus, patients with BAV should have annual follow-up with their primary care physician with special attention to the evaluation of hypertension. 7. Although patients with BAV are at higher risk for endocarditis, current guidelines do not recommend endocarditis prophylaxis before routine dental procedures or high-risk invasive respiratory procedures for patients with BAV without a history of surgery or intervention on the aortic valve.