Richard Krasuski, MD Duke University

1. Bicuspid Aortic Valve in
Athletes
Richard A. Krasuski, M.D.
Professor of Medicine and Pediatrics
Director of the Adult Congenital Heart Disease Program
Director of Hemodynamic Research
Director, Duke University Collaborative Cardiovascular Society
Duke University Health System

2. Disclosures
• Consultant for Actelion/Janssen Pharmaceuticals, Bayer, Neptune
Medical and Gore Medical
• Research funding from the Adult Congenital Heart Association and
Actelion/Janssen Pharmaceuticals
• Investigator for Edwards Lifesciences, Gradient Denervation
Technologies and Medtronic

3. Flashback to the Last Millenium
• I was on call covering the echo lab at Duke South
• Called to perform emergent echo on Duke BB player with syncope
• After entering locker room following a close game
• Felt warm, flushed and nauseated
• Experienced full syncope/collapse and luckily no trauma
• No prior cardiac or medical history
• ECG: NSR at 96, normal PR/QRS/QTc intervals, no ST or TW
abnormalities
• Exam unremarkable expect for mild tachycardia, systolic ejection
click and +I/IV DM at RUSB

4. The Actual Echo Images
• Is the valve bicuspid?
• If so, what type of BAV?
• How much regurgitation?
• Can the patient continue to
play collegiate BB?
• How frequent should
surveillance be and what?
• What are the risks
longterm?
• What are the options for
future interventions and
how would it impact career
or activity restrictions?

5. Overview
• Demographics and clinical impact of BAV
• Latest nomenclature system for BAVs
• Long-term sequelae – detection, prevention and management
• Clearance for participation and what who needs restrictions

6. • Affects ~1-2% of the general population
• 2-3:1 male predominance
• Usually sporadic, but may be inherited in an autosomal
dominant pattern; has variable penetrance
• Can be seen in syndromes and with other lesions (coarctation)
• Widely varying clinical course based on anatomy and
accompanying pathology
Demographics and Background of BAV

7. Demographics and Background of BAV
• Significant AS occurs in ~50%; Significant AI in 30%
• About 50% develop aortic dilation (20-84% depending on definition
and population studied)
• Should always be assessed for in patients with otherwise
unexplained aortic dilation
• If TTE inconclusive, CMR, cardiac CTA, and TEE can better visualize
aortic valve
• Choice between CT or MRI depends on patient characteristics,
institutional expertise, renal function, affordability, and concerns for
radiation exposure
• BAV seen in up to 10% of patients with HTAD (Loeys-Dietz and other
genetic syndromes) – if dilatation appears excessive, be concerned
D’Ascenzi et al. Scan J Med Sci Sports 2021;31:510-20.

8. International BAV Consensus Statement on
Nomenclature and Classification
Michelena HI et al. J Thorac Cardiovasc Surg 2021;162:781-97.
Ranges from “almost tricuspid” to “perfect bicuspidity”

9. • Younger BAV males
• BAV regurgitation common
• Associated with R-L cusp fusion
• Higher risk for complications
• More genetics dependent
Dilitation Phenotypes of BAV Aortopathy
Michelena HI et al. J Thorac Cardiovasc Surg 2021;162:781-97.; Isselbacher et al. Circulation 2022;146:e334-e482.
• Age dependent, men and women
• BAV stenosis common
• Associated with R-NC cusp fusion
• More hemodynamically dependent
Risk Factors for Dissection
Family history of aortic dissection
Aortic growth rate 0.3 cm/y
Aortic coarctation
“Root phenotype” aortopathy

10. Longer-term Issues for BAV
• Patients with BAV, with or without aortic dilation, require lifelong
surveillance of the aortic root and ascending aorta because of risk of
late aortic growth
• The degree of aortic dilation and the progression of aortopathy may
be greater in patients with aortic root phenotype and those with
predominant AI
• Progressive aortic growth may occur after AVR
• All BAV patients are at increased risk for endocarditis (11x general
population), though prophylaxis is not recommended by guidelines
Pelliccia A et al. Eur Heart J 2021;42:17-96.; Monda et al. Diagnostics 2022;12(1392):1-14.

11. Classification of BAV by Complexity
Michelena HI et al. J Thorac Cardiovasc Surg 2021;162:781-97.

12. Echo is the Cornerstone of Assessment
• Identify anatomy consistent with BAV
• Identify the phenotype
• Assess valve function – stenosis and regurgitation
• Measurement of the ascending aorta
• Assess for coarctation (7-10%) and other congenital lesions
• Ensure absence of complications – dissection (8x general
population) and endocarditis
Michelena HI et al. J Thorac Cardiovasc Surg 2021;162:781-97.

13. Key Aortic Dz Guideline Statements on BAV
Isselbacher et al. Circulation 2022;146:e334-e482.

14. Effect of Physical Activity on BAV
• 292 patients with BAV (210 athletes, 23 ex-athletes, 59 non) – no change in LV
morphology or function over 5 years
• 53 active BAV patients compared to 27 mostly sedentary (11.5 ± 3.4 yo, 77% male) – no
difference in > 10% increase in SOV (13% vs. 8%, p=0.45) or mid-ascending aortic
diameter (9% vs. 13%, p=0.55) at 2 years follow-up
• Meta-analysis - 23 studies of 5580 elite athletes and 727 sedentary controls showed
mean aortic root at SOV 3.2 mm larger (p=0.02) and root size at valve annulus 1.6 mm
greater (p=0.04), but only 1% exceeding normal upper limits and mostly with isometric
exercise
• Cross-sectional study of aortic size in 442 veteran endurance athletes found 21% with
ascending aortas ≥ 40 mm
• 81 Olympic athletes with BAV divided into low and high-risk followed for mean 13 years
showed progression in high-risk group (not in low-risk) even after disqualification
Galanti G et al. Br J Sports Med 2010;44:275-9.; Monda E et al. Pediatric Cardiology 2021;42:1133-40.;
Iskandar A and Thompson PD. Circulation 2013;127(7):791-8.; Churchill TW et al. JAMA Cardiol 2020;5(5):522-31.
Spataro A et al. Int J Sports Med 2008;29:81-5.

15. BAV Sports Participation
• If aorta not dilated, current international guidelines for sports participation in
patients with BAV are identical to guidelines in patients with a trileaflet aortic valve
• With valvular dysfunction or aortic dilitation, reassess every 6 months to 2 years
based on severity and symptomatic status
• Individuals with severe AR may participate in low- and moderate-intensity exercise
if the LV is not dilated, the LVEF remains >50%, the aortic root is within normal
dimensions or is mildly dilated, and exercise stress test results are normal
• Only low intensity exercise is recommended for patients with a dilated left ventricle,
LVEF <50%, significant aortic root dilatation, or exercise-induced arrhythmias
• Aorta >50 mm considered high risk and competitive sports are not recommended
• If mechanical valve replacement performed, individuals should not engage in
contact sports or athletic activities where collision is likely because of the risks of
bleeding risk with anticoagulation
• In many situations, valve-sparing aortic root repair, Wheat, Bentall or Ross
procedures may facilitate normal return to high intensity athletic activity
Pelliccia A et al. Eur Heart J 2021;42:17-96.; Monda et al. Diagnostics 2022;12(1392):1-14.;
Lai AF and Braverman AC et al. JAMA Cardiol 2022:7(7):772-3.

16. ESC Guidelines: Aortic Stenosis
Pelliccia A et al. Eur Heart J 2021;42:17-96.

17. ESC Guidelines: Aortic Insufficiency
Pelliccia A et al. Eur Heart J 2021;42:17-96.

18. ESC Guidelines: Aortic Dilitation
Pelliccia A et al. Eur Heart J 2021;42:17-96.
Rapid aortic root/ascending aortic growth in BAV defined as ≥0.3 cm in 1 year

19. Concerns for Family Members
• Occurrence of BAV is 5-10x higher in first degree relatives
• Most patients with BAV and TAAs who undergo genetic testing will not
be found to have a pathologic genetic variant, even when their
condition is familial
• Nevertheless, when condition is suspected familial, a medical
geneticist or specialist in genetic aortopathy should evaluate, counsel,
and genetically test in BAV and aortopathy
• Genetic testing is recommended for individuals with aortic aneurysm
and syndromic features, family history of dissection, and/or early age of
onset
• Guidelines recommend screening echo in 1st degree relatives of
patients with BAV and aortic aneurysm (strong evidence)
• Guidelines recommend screening echo in 1st degree relatives of
patients with BAV (moderate evidence)
Bravo-James K and Prakash SK. Prog in CV Dis 2020;63(4):398-406.; Isselbacher et al. Circulation 2022;146:e334-e482.

20. Summary
• Bicuspid aortic valve is the most common congenital heart
defect and most have normal lifespan despite risks for stenosis,
regurgitation and ascending aortic dilitation
• If suspected, transthoracic echo provided excellent assessment
and risk stratification for most patients
• Follow-up and repeat imaging based on severity of valve
disease and aortic dilatation as well as symptoms (6 months to
2 years)

21. THANK YOU FOR YOUR ATTENTION
Thank You!!