The Heart Center at Lurie Children’s is dedicated to monitoring and caring for more than 1,000 infants, children and teenagers with bicuspid aortic valve (BAV) disease. BAV is the most common form of congenital heart disease, affecting between 0.5-2% of the general population.
A normal aortic valve has three leaflets that open symmetrically. Individuals with a BAV are born with an aortic valve that either has two leaflets or fusion between two of the leaflets so that the valve opens abnormally and over time can become obstructed (stenosis) or leak (regurgitation). Patients with BAV are also at increased risk for progressive enlargement of the aorta, which can lead to the development of aortic aneurysms, aortic dissection (a tear in the aorta) and/or rupture.
The above illustration shows a 4-D flow MRI scan. The image on the right demonstrates flow disturbances in a BAV patient with aortic stenosis, compared to a control subject on the left with a normal tri-leaflet aortic valve. The healthy patient's aorta shows a cohesive forward flow pattern. In the BAV patient, blood flow is directed at an abnormal angle along the front and rightward side of the aorta, which places increased stress on the aortic wall.
Source: Michael Markl, MD, Associate Professor of Radiology & Biomedical Engineering, Director of Cardiovascular MR Research, and Alex Barker PhD, Assistant Research professor in Radiology, Northwestern University.
We provide a multidisciplinary team of experts to ensure that your child receives the most comprehensive care possible. This team meets weekly to discuss and decide on a care plan for each patient, which allows multiple heart doctors to weigh in on treatment options for your child.
Current research initiatives include:
Bicuspid aortic valve may be an inherited condition, occurring in up to 9% of first-degree relatives. Recent studies have shown that first-degree relatives may also have aortic enlargement in the absence of a BAV. Screening is recommended for all first-degree relatives (such as parents and siblings) of a person diagnosed with BAV. Early diagnosis can lead to the proper surveillance, management and improved long-term outcomes.
The easiest way to detect BAV and check for disease progression is with an ultrasound of the heart, called an echocardiogram. We perform this painless, non-invasive test in our accredited echocardiography laboratory. If more information is needed, we offer additional cardiac imaging techniques, such as computerized tomography (CT) and magnetic resonance imaging (MRI). Our specialists can provide longitudinal surveillance and care for your child, or provide a second opinion regarding diagnosis and treatment.
Management of the patient with BAV includes:
Specialists from Lurie Children’s Heart Center and Bluhm Cardiovascular Institute at Northwestern Medicine (NM) now meet regularly with BAV patients between the ages of 16-26 in one clinic setting. This integrated care occurs at a critical time in a patient’s life – during the transition to adult care. Our partnership with the Bluhm Cardiovascular Institute creates a continuity of care that patients benefit from throughout their lifetimes; they don’t have gaps in care while they find adult providers, and they have a continuous medical history that can help doctors predict complications before they arise.
The Cardiovascular Bridge Program also manages a centralized database that collects clinical information regarding BAV over time. This information will allow us to learn more about the natural history, physiologic and genetic mechanisms of this disease so that we can improve long-term outcomes.
The BAV team of experts includes:
To schedule an appointment, please contact our nurse practitioner, Elizabeth Cappella at 312.227.4641 or e-mail ecappella@luriechildrens.org.
