Living with Bicuspid Aortic Valve: Tips for a Healthy Lifestyle

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

What is a bicuspid aortic valve?

Bicuspid aortic valve (BAV) is a heart problem present at birth and 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.

Bicuspid Aortic Valve Genetics

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 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 right-non-coronary commissural fusion are at a greater lifetime risk for progressive narrowing and leaking of the valve.

Bicuspid Aortic Valve Lifestyle, Diet & Exercise

The most important thing that patients with BAV can do is to lead a healthy lifestyle 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.

Bicuspid Aortic Valve Management

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.

Frequently Asked Questions

How serious is bicuspid aortic valve?

Bicuspid aortic valve disease can present on a spectrum of disease severity. Some patients may have more valve dysfunction or aortopathy at an earlier age, while others will have minimal disease or effects from their bicuspid aortic valve. However, the vast majority of children have less severity than adults with the disease.

Patients with bicuspid aortic valve disease will experience more valve dysfunction as they age. However, not everyone with bicuspid aortic valve disease will need surgery or a procedure. Patients with bicuspid aortic valve should have regular follow up with their cardiologist to monitor valve dysfunction and aortopathy.

Can you grow out of a bicuspid aortic valve?

Unfortunately, you cannot grow out of your bicuspid aortic valve. It occurs during fetal development and is one of the most common types of congenital heart disease.

What is the life expectancy of someone with a bicuspid aortic valve?

Life expectancy with bicuspid aortic valve disease is normal with routine follow-up appointments with your cardiologist to monitor BAV symptoms and overall health.

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