Bicuspid Aortic Valve (BAV) Program

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.

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Bicuspid Aortic Valve Diagram

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.

Getting Screened

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:

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
Surgery for aortic valve replacement

Learn more about BAV screening

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.

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Diagnosed with Bicuspid Aortic Valve as a toddler, Gabe has been a patient with Lurie Children's Bicuspid Aortic Valve Program since the age of 3. Now, as a young adult, he's a part of the Bicuspid Aortic Valve Bridge Program, a joint clinic with Northwestern Medicine Bluhm Cardiovascular Institute, to help with his transition from pediatric to adult care.

Our Specialists

The BAV team of experts includes the following members.

Pediatric Cardiologist

Joshua D. Robinson, MD

Attending Physician, Cardiology; Section Head, Cardiac Imaging; Director, Bicuspid Aortic Valve Program

Professor of Pediatrics (Cardiology) and Radiology, Northwestern University Feinberg School of Medicine

Cardiology Advanced Practice & Registered Nursing Team

Elizabeth S. Cappella, APRN-NP

Advanced Practice Provider, Outpatient Cardiology

Kali Hensley, RN

Registered Nurse

Pediatric Heart Surgeons

Osama M. Eltayeb, MD

Director, Vascular Rings Program; Director, Tracheal Reconstruction Program; Attending Physician, Cardiovascular-Thoracic Surgery; Member, Lurie Children's Surgical Foundation

Associate Professor of Surgery (Cardiac Surgery), Northwestern University Feinberg School of Medicine

Michael C. Mongé, MD

Surgical Director, Heart Failure/Heart Transplant Program; Attending Physician, Cardiovascular-Thoracic Surgery; Member, Lurie Children's Surgical Foundation

Associate Professor of Surgery (Cardiac Surgery), Northwestern University Feinberg School of Medicine

Genetics

Barbara K. Burton, MD

Attending Physician, Genetics, Genomics and Metabolism

Professor of Pediatrics (Genetics, Genomics and Metabolism), Northwestern University Feinberg School of Medicine

Our adult Cardiology team from Bluhm Cardiovascular Institute at Northwestern Medicine includes:

Jyothy Puthumana, MD
Erin Crawford, RN, BSN

Make an Appointment

To schedule an appointment, please call 312.227.4100.

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.

Second Opinions

For families or providers seeking a second opinion, we offer a Priority Second Opinion Clinic.

REQUEST A SECOND OPINION

Heart Center Family Resource Guide

To help prepare families for their care with Lurie Children's Heart Center, we have compiled a list of resources about treatment and recovery. Learn how to get ready for an inpatient stay or outpatient visit, and read about our support services for patients and families.

Additional Resources

For more information regarding BAV and associated conditions, please view the following resources:

Related Specialties

Heart Center