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Caring for Patients with Bicuspid Aortic Valve

July 02, 2018

by Elizabeth Capella, APN, NP, Pediatric Nurse Practitioner, Cardiology, Cardiovascular-Thoracic Surgery, Cardiac Critical Care

Bicuspid aortic valve (BAV) is the most common congenital cardiac anomaly, with an estimated incidence of 0.5 percent to percent in the general population. Males are affected 3:1 compared to females. Individuals with BAV can develop progressive narrowing or obstruction of the valve, leaking of the valve, and around 50 percent of adults will eventually develop significant enlargement of the aorta, which can be life threatening. Patients also are at a slightly increased life-long risk for endocarditis, which is an infection that can develop in the heart. Patients with BAV can have severe valve disease in infancy, however they can also have minimal valve disease in childhood. Most commonly, valve complications typically develop in adulthood. Only 1 in 50 children will develop significant problems by adolescence; whereas 25-40 percent of adults will eventually need some form of medical or surgical intervention for BAV by age 45-50 yr. That said, however, recent and large series have confirmed that life expectancy in adult patients with BAV disease is typically similar to the general population.

The genetics of BAV are complex, and recent studies have demonstrated that BAV is likely due to mutations in different genes with dissimilar patterns of inheritance. The prevalence of BAV (or a related left heart abnormality) among first-degree relatives of affected individuals is around 9 percent — again with a male preponderance. The morphologic characteristics of BAVs are important determinants of the natural history, suggesting that differing BAV morphologies represent distinct etiological entities. The two most common morphologies are right-noncoronary commissural fusion, versus right-left commissural fusion. It is exceedingly rare to find the left and non-coronary aortic leaflets fused. Patients with right-left commissural fusion often have more severe problems with enlargement of the aorta into adulthood. This morphology is also the one most commonly associated with coarctation of the aorta, which is a narrowing of the aorta. In contrast, those with with right-non-coronary commissural fusion are at greater life-time risk for progressive narrowing and leaking of the valve.

The most important thing that patients with BAV can do is to lead a healthy life style which includes a heart healthy diet and routine exercise! Exercise is beneficial to both the physical and emotional well-being of people with BAV disease, and regular exercise is an important life-long general wellness recommendation. Most people with BAV can safely exercise without significant restrictions. Strenuous isometric exercise (e.g., weight-lifting, climbing steep inclines, chin-ups), should be avoided if there is severe valve disease, or moderate to severe aortic ectasia.

Management of BAV includes:

  • Education
  • Good dental care and endocarditis prophylaxis, if indicated
  • Serial clinical, echocardiographic and CT/MRI follow up
  • Treatment of high blood pressure and possible medical therapy for associated aortic valve dysfunction and/or aortic dilation
  • Potential physical activity restrictions
  • Cardiac transcatheter interventions and/or surgery for significant aortic stenosis and/or regurgitation
  • Surgery for aortic root aneurysm to prevent life-threatening rupture

The Bicuspid Aortic Valve Program  offers a unique approach to comprehensive care for children and their families affected by BAV. We offer expert clinical care from a multidisciplinary team, advanced diagnostic imaging including 4D flow MRI, family screening, and access to ongoing research initiatives exclusively for BAV patients. We also have a specialized Bridge clinic to care for our young adult patients, to prepare them for transitioning to adult care and to allow them to build a relationship with their adult cardiologist prior to making that transition.