THE MARTHA AND RICHARD MELMAN FAMILY BICUSPID AORTIC VALVE (bav) PROGRAM AT NORTHWESTERN MEDICINE BLUHM CARDIOVASCULAR INSTITUTE

August 2022

CARDIOVASCULAR

SURGICAL TREATMENT OF BICUSPID AORTIC VALVE (BAV)

S. Chris Malaisrie, MD, professor of Surgery in the Division of Cardiac Surgery at Northwestern Medicine, discusses some of the procedures performed in the Martha and Richard Melman Family Bicuspid Aortic Valve (BAV) Program at Northwestern Medicine Bluhm Cardiovascular Institute. Dr. Malaisrie also shares surgery options for treatment of BAV, including minimally invasive options.

TRANSFORMING BICUSPID AORTIC VALVE (BAV) CLINICAL CARE

Jyothy J. Puthumana, MD, associate professor of Medicine in the Division of Cardiology at Northwestern Medicine, explains his role in the Martha and Richard Melman Family Bicuspid Aortic Valve (BAV) Program at Northwestern Medicine Bluhm Cardiovascular Institute. Dr. Puthumana also discusses using 3D echo and 4D MRI to better diagnose and treat BAV, the importance of lifelong care for patients with BAV, surgical intervention for BAV and more.

BAV TOP TEN

​We celebrate the following program highlights from the last ten years:

1. Enrolled over 1,000 patients in our longitudinal BAV research registry to track disease progression, treatment outcomes and changes in family history over time. 

2. Coordinated by a designated BAV program nurse, family screening echocardiography of over 250 first-degree relatives – from 130 families – helped identify 16 (6%) new first-degree relatives with BAV, 11 of whom initiated follow up with the Northwestern Medicine BAV program (1).  

3. Performed over 1,100 surgeries and 150 transcatheter valve implantations (TAVI). Although national experience is growing, TAVI is best applied to patients with select BAV anatomy and risk profiles. Decision making is highly individualized, therefore multidisciplinary collaboration and patient education are important in order to reach the best shared decision for each patients’ treatment.

4. Validated contemporary surgical guidelines by demonstrating the safety of ascending aortic resection in patients with BAV aneurysms of 4.5cm or greater when undergoing an aortic valve replacement at high volume centers (2). 

​5. With our colleagues in Northwestern University’s Department of Radiology, we completed 4D flow cardiac magnetic resonance (CMR) scans on 1,280 BAV patients and published 51 related manuscripts. This collaboration between physicians and scientists aims to develop noninvasive biomarkers through the assessment of aortic wall shear stress (WSS) as a potential indicator of aneurysm progression and/or a measurement that could refine the timing of prophylactic aortic resection (3,4).

6. Established an adolescent transition clinic with the Heart Center at Lurie Children’s Hospital to improve continuity of care for adolescent patients transitioning to adult cardiology care teams. This patient population represents the more complex phenotypes of BAV and is at high risk for attrition in early adulthood. By beginning the relationship between these adolescents and our adult care team, we intend to minimize gaps in care for the patient and family with BAV.

7. Implemented a state-of-the-art biobank to store tissue samples for histopathology studies. To-date, we have collected over 875 samples of aortic valve tissue, aorta tissue, and serum plasma from patients with bicuspid and trileaflet aortic valves.

8. Published over 80 BAV research publications cited in medical, surgical, and imaging subspecialty journals.

​9. Found new evidence for a relationship between regions of increased WSS, extracellular matrix dysregulation, and elastic fiber degeneration in the ascending aorta of BAV patients. This association suggests valve-related hemodynamics contribute to the development of BAV aortopathy (4). Furthermore, these findings allowed us to secure $3,000,000.00 in NIH-sponsored funding to continue understanding the potential use of WSS as a marker of aneurysm malignancy and progression.

10. Hosted five patient education, outreach events, both in-person and virtually, reaching over 1,000 attendees in total. These educational events aim to provide an opportunity for patients to learn more about the latest research dialogue and treatment offerings for BAV disease, meet other patients and connect with our team outside of the hospital system.  
 
​Works Cited:
1. Crawford EE, McCarthy PM, Malaisrie SC, Puthumana JJ, Robinson JD, Markl M, et al. Applications of a Specialty Bicuspid Aortic Valve Program: Clinical Continuity and Translational Collaboration. J Clin Med. 2020;9(5).
 
2. Rinewalt D, McCarthy PM, Malaisrie SC, Fedak PW, Andrei AC, Puthumana JJ, et al. Effect of aortic aneurysm replacement on outcomes after bicuspid aortic valve surgery: validation of contemporary guidelines. J Thorac Cardiovasc Surg. 2014;148(5):2060-9.

3. Soulat G, Scott MB, Allen BD, Avery R, Bonow RO, Malaisrie SC, et al. Association of Regional Wall Shear Stress and Progressive Ascending Aorta Dilation in Bicuspid Aortic Valve. JACC Cardiovasc Imaging. 2021.

4. Guzzardi DG, Barker AJ, van Ooij P, Malaisrie SC, Puthumana JJ, Belke DD, et al. Valve-Related Hemodynamics Mediate Human Bicuspid Aortopathy: Insights From Wall Shear Stress Mapping. J Am Coll Cardiol. 2015;66(8):892-900.

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