Specialties & Conditions Bicuspid Aortic Valve Program
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.
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
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.
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
Associate 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
Assistant 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.
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:
