Connective Issues Winter 2023

ACC/AHA Announce New Aortic Disease Management Guidelines

New guidelines for the diagnosis and management of aortic disease were published in the fall in the Journal of the American College of Cardiology and the journal, Circulation . These guidelines, which were created by a team of medical experts on behalf of the American College of Cardiology and American Heart Association, provide recommendations on the diagnosis, evaluation, medical therapy, endovascular and surgical intervention, and long-term surveillance of people with aortic disease based on evidence available in the literature and, in some instances, based on expert consensus opinions. They include specific recommendations for those with syndromic and non-syndromic genetic aortic conditions as well as other types of acute aortic syndromes. They also emphasize the role of shared decision-making. The guidelines provide support for: • Seeking surgical treatment from high-volume centers with experienced surgeons as part of a multi disciplinary team for asymptomatic patients with extensive aortic disease, those who might benefit from complex open and endovascular repairs, and those with multiple co-morbidities. • Genetic testing for people with syndromic features, family history of thoracic aortic disease, or early onset of disease (under the age of 60). • Thoracic aortic imaging of family members of a ff ected individuals to screen for asymptomatic thoracic aortic disease in specific cases. Tips from patient advocate, Maya Zimmerman-Brown, MPH ✓ Come prepared to your appointment with relevant questions. ✓ Bring a copy of the guidelines to your physician. ✓ Listen to your doctor’s rationale and have a discussion. ✓ Look for a second opinion.

If you are going to address an aorta at a smaller size, you really need to have a lot of experience, both in doing the procedure and also in understanding the disease process and risks to the patient undergoing that procedure. ~Dr. Abe DeAnda

• Open surgery is recommended over thoracic endovascular aortic repair in patients with Marfan, Loeys-Dietz, and VEDS who have a descending thoracic aortic aneurysm that meets criteria for intervention and have suitable anatomy. The new guidelines were developed in collaboration with and endorsed by the American Association for Thoracic Surgery, American College of Radiology, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Thoracic Surgeons, and Society for Vascular Surgery. To watch a recording of our webinar with more information about the new guidelines and how they a ff ect people with Marfan, Loeys-Dietz, VEDS, and other genetic aortic and vascular conditions, visit our YouTube Channel. The presenters were members of the ACC/AHA writing committee for the guidelines: Dr. Alan Braverman, cardiologist, Washington University School of Medicine in St. Louis; Dr. Abe DeAnda, cardiothoracic

surgeon, University of Texas Medical Branch (UTMB) in Galveston; and Maya Brown-Zimmerman, MPH, patient advocate. To view specific recommendations for Marfan syndrome, Loeys-Dietz, and pregnancy, scan the QR code.

A multidisciplinary team is critical in shared decision-making as the vast majority of cardiac surgeons perform less than or equal to two aortic root surgeries in a year. It’s not a common surgery for the vast majority of cardiac surgeons. Having complex aortic surgery, especially when very low risk is necessary, it is important to see a surgeon with experience. ~Dr. Alan Braverman

To get assistance finding a surgeon or a coordinated clinic, visit Marfan.org and click on Find a Doctor on the home page to download our institution directory. Or you can connect with our Help & Resource Center at Marfan.org/ask.

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