Natural History of Bicuspid and Aneurysm-Associated Aortic Valve Disease

The most common congenital heart defect, congenital BAV, affects 1% of the population (2% in men, 0.5% in women), with a male-to-female ratio of 2:1 to 3:1. It's characterized by diverse phenotypic expressions and outcomes, falling into two prognostic groups:

  • Typical valvulo-aortopathy: Most common, with progressive BAV dysfunction and/or aorta dilatation but long-term survival similar to the general population.
  • Complex valvulo-aortopathy: Associated with significant concomitant disorders and/or accelerated valvulo-aortopathy, leading to inferior long-term survival.

BAV is a lifelong clinical condition with a morbidity burden exceeding 80%. Common complications include progression to ≥ moderate AS or AR, native aortic valve surgery, aortic aneurysm, surgery for aortic aneurysm, surgery for coarctation of the aorta, infective endocarditis, and aortic dissection.

In this interview, Hector I. Michelena, MD, FACC and W. Douglas Weaver MD, MACC discuss the history of and complications associated with bicuspid valvuloaortopathy.

Related References:

  1. Michelena, H. I., Khanna, A. D., Mahoney, D., Margaryan, E., Topilsky, Y., Suri, R. M., Eidem, B., Edwards, W. D., Sundt, T. M., 3rd, & Enriquez-Sarano, M. (2011). Incidence of aortic complications in patients with bicuspid aortic valves. JAMA, 306(10), 1104–1112.
  2. Yang, L. T., Ye, Z., Wajih Ullah, M., Maleszewski, J. J., Scott, C. G., Padang, R., Pislaru, S. V., Nkomo, V. T., Mankad, S. V., Pellikka, P. A., Oh, J. K., Roger, V. L., Enriquez-Sarano, M., & Michelena, H. I. (2023). Bicuspid aortic valve: long-term morbidity and mortality. European heart journal, 44(43), 4549–4562.
  3. Ye, Z, Lane, C, Beachey, J. et al. Clinical Outcomes in Patients With Bicuspid Aortic Valves and Ascending Aorta ≥50 mm Under Surveillance. JACC Adv. 2023 Oct, 2 (8) .

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Valvular Heart Disease

Keywords: ACCELLite

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