Sports Participation in Patients with Inherited Diseases of the Aorta Yonatan Buber, MD Adult Congenital Heart Service Leviev Heart Center Safra Childrens Hospital
Disclosures None
Patient Presentation 37 YO M diagnosed with Marfan s Syndrome at 19 years of age Diagnostic criteria included: - Aortic dilatation (z score of 3 at the time) - Systemic features: wrist sign, thumb sign, pectus carinatum deformity, reduced upper segment/lower segment ratio and increased arm span/height Mother and grandfather were also very tall, but were never tested for Marfan s. No family members with a history of heart surgery or premature death due to presumed CV causes
Yesterday Afternoon, Pediatric EP Session Hi Dr. Buber here Wassssaaaaaap? Just wanted to ask for your educated opinion regarding participation in spinning classes for an individual who needs to maintain his figure and has Marfan s, like myself. TODAH!
Aortic Dimensions (cm) 4.5 4 3.5 3 2.5 2 1.5 Aortic root Ascending aorta 1 0.5 0 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015
Aortic Wall Degeneration and Aneurysm Formation Imbalance in the synthesis and degradation of the medial matrix proteins: (imbalance in the medial expression of MMPs, defects in Fibrillin-1, enhanced TGF-beta activity) Dilatation of the ascending aorta Compensatory extracellular matrix formation mediated by smooth muscle cells
Pentachrome staining Black indicates elastin; red/purple, smooth muscle cells; blue, mucopolysaccharides; and yellow, collagens. Emmott A et al. Can J Cardiol 2015
Aneurysm Formation in Bicuspid Aortic Valves Verma S et al. NEJM 2014 Emmott A et al. Can J Cardiol 2015
The Potential Risks Associated with Sports Participation
The concerns The information needed Will the exercise cause a dissection (or even a rupture)? BP response Will the exercise lead to (further) aortic dilatation? Collision/sudden stop risk Type of exercise (isometric/isotonic/contact) The intensity and the frequency of the exercise
estimated percentage of maximal voluntary contraction estimated percentage of maximal oxygen uptake Levine BD et al. A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol 2015
Risk of Dissection Blood Pressure Behavior Isotonic exercise- systemic vascular resistance, systolic BP (less prominent in the young)-> net modest increase in mean BP Isometric exercise- Systolic, diastolic BP and systemic vascular resistance (reflex vasoconstriction to noncontracting muscles). Further increase in all values exists during the strain phase of the Valsalva maneuver Many sports involve a mixture of both types
KoulliasG et al. The Journal of Thoracic and Cardiovascular Surgery 2005
Ex-vivo model Maximal tensile strength before tearing of aortic specimen taken from patients with ascending thoracic aortic aneurysm was 1000 kpa Vorp et al. Ann Thorac Surg 2003
Risk of Aortic Enlargement Aortic size is larger in elite endurance athletes compared to controls (effect is more pronounced than in isometric exercise) Iskandar A, Thompson, PD. Circulation 2013
Sports Participation- Aortopathies Isaiah Austin, NBA 15 th draft 2014
Aortic Diameter is a Surrogate for Wall Stress Estimation T- Wall tension R- radius P-pressure Ō Θ -circumferential stress
The nature of the remodeling might vary significantly depending on the disease process The composition, thickness, and ultimate tensile strength may differ significantly between aortas of the same size
Setting a threshold diameter limit assumes that all thoracic aortas have the same strength, thickness, and blood pressure
While in the real world Nearly 50% of aortic dissections occur below the recommended threshold for intervention
Exercise and Sudden Cardiac Death Related to Rupture Barry J. Maron et al. Circulation. 2009
Classes IA and IIA if none of the following exists: a. Aortic root dilatation: - z score >2 in children or adolescents <15 years old - Aortic diameter >40 mm, b. Moderate to severe mitral regurgitation c. Left ventricular ejection fraction <40% d. Family history of aortic dissection at an aortic diameter <50 mm
After surgical correction due to aneurysm/dissection and no evidence of residual aortic enlargement or dissection: Class IA that do not include the potential for bodily collision
Recreational Sports and Exercise Recommendations
Sports Participation- Bicuspid Aortic Valve
All competitive athletics if: Aortic root and ascending aorta are z score <2, or <40 mm in adult males, <35 mm in females. *The function of the BAV (whether stenotic or regurgitant) is also important in determining participation recommendations.
Aortic z score 2 to 3.5 or aortic root or ascending aortic diameters measuring 40 to 42 mm in men or 36 to 39 mm in women AND NO features of associated connective tissue disorder or familial TAA syndrome:
Athletes with BAV and a severely dilated aorta (score >3.5 to 4 or >43 mm in men or >40 mm in women) should not participate in any competitive sports that involve the potential for bodily collision Athletes with BAV and a markedly dilated aorta (>45 mm) should not participate in any competitive sports
Summary Patients with inherited aortopathies may be at high risk for dissections or gradual dilatation due to medial degeneration and altered hemodynamic forces Although evidence is limited, patients should be counseled to refrain from activities that increase the risk of dissection/rupture/further dilatation, including: - Activities requiring straining/valsalva - Competitive dynamic sports - Burst exercise Until scientific evidence suggest otherwise, patients should be allowed to perform their daily activities (baby lifting, grocery carrying, etc)
Thank You Yonatan.buber@sheba.health.gov.il
1. Athletes with Marfan syndrome should undergo echocardiographic (and in some instances MRA or CT) measurement of the aortic root dimension every 6 to 12 months, depending on aortic size 2. Athletes with unexplained TAA, familial TAA syndrome, or known pathogenic mutation leading to a familial TAA syndrome should undergo echocardiographic and (depending on the diagnosis) MRA or CT surveillance every 6 to 12 months to evaluate for progression of aortic or branch vessel disease
Athletes with BAV and aortic dimensions above the normal range (scores 2 to 3 or aortic diameters measuring 40 to 42 mm in men or 36 to 39 mm in women) should undergo echocardiographic or MRA surveillance of the aorta every 12 months, with more frequent imaging recommended for increasing aortic z score
The no-no s Athletes with Marfan syndrome, familial TAA syndrome, Loeys-Dietz syndrome, unexplained aortic aneurysm, vascular Ehlers-Danlos syndrome, or a related aortic aneurysm disorder should not participate in any competitive sports that involve intense physical exertion or the potential for bodily collision
The no-no s