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 1-2% 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.
Learn more about BAV in the video below.
The below 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.
The Lurie Children's Difference
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:
Functional 4D MRI in BAV to determine predictors of aneurysm formation, such as flow mechanics and wall stress
Determination of long-term clinical benefits of medical therapy in patients with BAV and aortic dilation
Assessment of the effects of medical therapy on aortic wall properties in patients with BAV
Determining accuracy of aortic measurements in children using 3D echocardiogram
Determination of biomarkers which may predict aortic growth, aneurysm formation and dissection
Keeping Your Family Informed & Healthy
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:
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
Surgery for aortic valve replacement
Cardiovascular Bridge Program
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.
To schedule an appointment, please contact our clinical coordinator, Becca Kraft, at 312.227.4638.
In addition to in-person visits, we now offer telemedicine visits via video or phone. Some appointments will still require you to be seen in-person, but your physician and care team will let you know if a telemedicine appointment is available.
For families or providers seeking a second opinion, we offer a Priority Second Opinion Clinic.
To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources that may be of use throughout treatment — whether it's getting ready for an inpatient stay or outpatient visit, or learning more about the support services available to patients and families.