Bicuspid Aortic Valves

Bicuspid Aortic Valve Occurs in 1-2% of population Associated with : aortic coarctation patent ductus arteriosis abnormal proximal coronary vasculature

Bicuspid Aortic Valve Associated with connective tissue problems of aortic root and aorta Associated with aortic dilation, aneurysm, and dissection Associated with aortic root dilation

Bicuspid Aortic Valve Prone to developing aortic stenosis Aortic root dilation can cause aortic insufficiency

Bicuspid Aortic Valve Schematic presentation (as viewed from the surgeon’s position with the left coronary sinus on the left side) of the classification system of BAVs with one main and two subcategories, including the number of specimens (percent in parenthesis) Hans-H. Sievers, MD, and Claudia Schmidtke, MD, MBA. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thoracic Cardiovasc Surg 2007 133:1226-33.

Bicuspid Aortic Valve

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Bicuspid Aortic Valve

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Bicuspid Aortic Valve

Age distribution of bicuspid and tricuspid aortic valve stenosis.

Age (by decades) versus functional state of 535 congenitally bicuspid aortic valves (data for the 7 normally functioning valves are not shown.) Al=aortic insufficiency (regurgitation); AS=aortic stenosis. (Sabet et al)

Aortic Root and Flow The aortic root anatomy (trileaflet vs bicuspid) and orientation to LV and ascending aorta influences flow dynamics. MRI data has shown laminar, right helical and left helical flow dynamics. Abnormal flow dynamics have been shown to increase root pressure and may contribute to dilation.

Figure 1. Flow patterns in biscuspid aortic valve disease Figure 1. Flow patterns in biscuspid aortic valve disease. (A) Normal flow pattern, (B) right-handed helical flow, and (C) left-handed helical flow. The systolic flow angle(Ɵ) is demonstrated in B-the angle between the aortic midline(dashed) and the instantaneous mean flow vector at peak systole (arrow). Bissell et al Circ Cardiovasc Imaging July 2013

right; RA, right anterior; and RP, right posterior. Figure 3. Wall shear stress (WSS) at all locations in the ascending aorta, in different bicuspid aortic valve flow patterns. (A) Systolic WSS and (B) through-plane systolic WSS. A indicates anterior; L, left; LA, left anterior; LP, left posterior; P, posterior; R, right; RA, right anterior; and RP, right posterior. Bissell et al Circ Cardiovasc Imaging July 2013

2014 AHA/ACC Bicuspid Aortic Valve Diagnostic Testing-Initial Diagnosis: Recommendations Class I 1. An initial TTE is indicated in patients with a known bicuspid aortic valve to evaluate valve morphology, to measure the severity of AS and AR, and to assess the shape and diameter of the aortic sinuses and ascending aorta for prediction of clinical outcome and to determine timing of intervention (256-261). (Level of Evidence: B) 2. Aortic magnetic resonance angiography or CT angiography is indicated in patients with a bicuspid aortic valve when morphology of the aortic sinuses, sinotubular junction, or ascending aorta cannot be assessed accurately or fully by echocardiography. (Level of Evidence: C) Nishimura, RA et al. 2014 AHA/ACC Valvular Heart Disease Guideline

2014 AHA/ACC Bicuspid Aortic Valve Diagnostic Testing- Routine Follow-up: Recommendations Class I 1. Serial evaluation of the size and morphology of the aortic sinuses and ascending aorta by echocardiography, CMR, or CT angiography is recommended in patients with a bicuspid aortic valve and an aortic diameter greater than 4.0 cm, with the examination interval determined by the degree and rate of progression of aortic dilation and by family history. In patients with an aortic diameter greater than 4.5 cm, this evaluation should be performed annually. (Level of Evidence: C) Nishimura, RA et al. 2014 AHA/ACC Valvular Heart Disease Guideline

2014 AHA/ACC Bicuspid Aortic Valve Intervention: Recommendations Class I 1. Operative intervention to repair the aortic sinuses or replace the ascending aorta is indicated in patients with a bicuspid aortic valve if the diameter of the aortic sinuses or ascending aorta is greater than 5.5 cm (113, 268, 269). (Level of Evidence: B) Class IIa 1. Operative intervention to repair the aortic sinuses or replace the ascending aorta is reasonable in patients with bicuspid aortic valves if the diameter of the aortic sinuses or ascending aorta is greater than 5.0 cm and a risk factor for dissection is present (family history of aortic dissection or if the rate of increase in diameter is ≥0.5 cm per year). (Level of Evidence: C) 2. Replacement of the ascending aorta is reasonable in patients with a bicuspid aortic valve who are undergoing aortic valve surgery because of severe AS or AR (Sections 3.2.3 and 4.3.3) if the diameter of the ascending aorta is greater than 4.5 cm. (Level of Evidence: C) Nishimura, RA et al. 2014 AHA/ACC Valvular Heart Disease Guideline

Bicuspid Aortic Valve Dilated Root

Comparison of Aortic Root Measurements in 83 Patients with Bicuspid Aortic Valve and Matched Control Subjects Hahn RT, Roman MJ, Mugtader AH, Devereux RB, Association of Aortic Dilation with Regurgitant, Stenotic, and Functionally Normal Bicuspid Aortic Valves. J Am Coll Cardiol, 1992 Feb; 19(2):283-8

Bicuspid Aortic Valve - References 1) Braverman AC, Hasan G, Beardslee MA, Makan M, Kates, AM, Moon MR. The Bicuspid Aortic Valve. Curr Probl Cardiol 2005 Sep: 471-521. 2) Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally Bicuspid Aortic Valves: A Surgical Pathology Study of 542 Cases (1991 Through 1996) and a Literature Review of 2,715 Additional Cases. Mayo Clin Proc 1999;74:14-26. 3) Borger MA, David TE. Management of the Valve and Ascending Aorta in Adults with Bicuspid Aortic Valve Disease. Semin Thoracic Cardiovasc Surg 2005; 17:143-147. 4) Hans-H. Sievers, MD, and Claudia Schmidtke, MD, MBA. A classification system for the bicuspid aortic valve from 304 surgical specimens. J Thoracic Cardiovasc Surg 2007 133:1226-33. 5) Sabet HY, Edwards WD, Tazelaar HD, Daly RC. Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases. Mayo Clin Proc. 1999;74:14-26. 6) Roberts WC. The congenital bicuspid aortic valve. Am J Cardiol. 1970;26:72-83. 7) Angelini A, Ho SY, Anderson RH, Devine WA, Zuberbuhler JR, Becker AE, et al. The morphology of the normal aortic valve as compared with the aortic valve having two leaflets. J Thorac Cardiovasc Srg. 1989;98:362-7. 8) Hahn RT, Roman MJ, Mugtader AH, Devereux RB, Association of Aortic Dilation with Regurgitant, Stenotic, and Functionally Normal Biscuspid Aortic Valves. J Am Coll Cardiol, 1992 Feb; 19(2):283-8