Competetive sports in high risk patients Marfan syndrome with a non (or mildly) dilated aortic root Maarten Groenink Academic Medical Center Amsterdam The Netherlands
Marfan syndrome Autosomal dominant connective tissue disease Mutations in FBN1 gene (>1000) Cardiovascular, skeletal, ocular system Aortic aneurysm formation => dissection => death Great variability in clinical expression
Marfan celebrities Lincoln Pharao Achnaton Paganini Rachmaninoff
Marfan?
MS : specific advantages in sports?
The Marfan aorta
Aortic dissection Ascending aortic dissection : High risk of sudden death Cardiac tamponade Coronary occlusion Massive aortic regurgitation Aortic rupture
Prevention of aortic dissection Prophylactic surgery in dilated aortic segments : - aortic root - other segments (?) Treatment with blood-pressure lowering agents Treatment with anti-inflammatory agents (?) Lifestyle management
Causes of sudden death in athletes Circulation. 2011;123:1594-1600
Causes of SCD in 347 athletes N Engl J Med 2003; 349:1064-1075
Guidelines I ll be happy to give you innovative thinking. What are the guidelines?
Competetive sports in MS? ACC/AHA GUCH guidelines (2008) : ESC GUCH guidelines (2010) : Patients should be advised to avoid exertion at maximal capacity, competitive, contact, and isometric sports. Canadian GUCH guidelines (2010) : Exercise and activity recommendations in patients with Marfan s syndrome should be individualized according to the underlying severity of aortic root dilation and/or a malignant family history of aortic dissection
Canadian guidelines : MS should avoid..: Heavy isometric exercise, such as weight lifting, which can markedly increase peripheral vascular resistance and proximal aortic wall stress. Competitive aerobic sports, which can markedly increase cardiac output and rate of increase in aortic pressure over prolonged periods of time. Activities that involve risk of bodily collisions. Marked changes in ambient air pressure (as in scuba diving or sudden changes in altitude in a nonpressurized aircraft) because they can predispose to pneumothorax.
Forces that act on the aortic root outwardly directed forces Longitudinal forces twisting forces shear stress
Dynamics of the aortic root
Aneurysm formation and dissection in MS
Effects of dynamic and static activity Dynamic Static Dynamic Static 0 3 6 9 12 0.5 1 1.5 2 0 3 6 9 12 0.5 1 1.5 2 Minutes J Am Coll Cardiol. 2005 Apr 19;45(8):1364-7.
Classification J Am Coll Cardiol. 2005 Apr 19;45(8):1364-7.
Weight lifting and aortic dissection Cardiology 2007;107:103 106
Contact sports
Individualized recommendation Dynamic sports up to 40 % VO2max, max HR 100 bpm Echocardiographic check up every 6 months if AR > 40 mm Circulation. 2008;117:2802 13
Classification J Am Coll Cardiol. 2005 Apr 19;45(8):1364-7.
Effect of highly dynamic and static sports on the aorta
Aortic complications in Marfan syndrome
Marfan and sports
Take home messages Almost all competetive sports should be avoided in Marfan syndrome, regardless of aortic root size, especially weight lifting and contact sports Modest levels of dynamic sports can be encouraged with target heart rates < 100 bpm and target VO2 max of 40% Not only aortic root size, but the dimensions of the entire aorta should be considered (MRI) If higher levels of sports are undertaken : echocardiographic control every 6 months
Thank you
SCD by sport, ethnicity, sex (Circulation. 2011;123:1594-1600.)
Pelliccia et al, Eur Heart J 2005 Classification of sports A. Low dynamic B. Moderate dynamic C. High dynamic I. Low static II. Moderate static Bowling Cricket Golf Riflery Billiards Curling Auto racing Diving Equestriana Motorcycling Gymnastics Karate/Judo Sailing Archering Fencing Table tennis Tennis (doubles) Volleyball Baseball/softball Field events (jumping) Figure skating Lacrosse Running (sprint) Badminton Race walking Running (marathon) Cross-country skiing Squash Basketball Biathlon Ice hockey Field hockey Rugby Soccer Cross-country skiing (skating) Running (mid/long) Swimming Tennis (single) Team handball III. High static Bobsledding Field events (throwing) Luge Rock climbing Waterskiing Weight lifting Windsurfing Body building Downhill skiing Wrestling Snow boarding Boxing Canoeing, Kayaking Cycling, Decathlon Rowing Speed skating Triathlon
Marfan without aortic root dilatation A. Low dynamic B. Moderate dynamic C. High dynamic I. Low static II. Moderate static Bowling Cricket Golf Riflery Billiards Curling Auto racing Diving Equestriana Motorcycling Gymnastics Karate/Judo Sailing Archering Fencing Table tennis Tennis (doubles) Volleyball Baseball/softball Field events (jumping) Figure skating Lacrosse Running (sprint) Badminton Race walking Running (marathon) Cross-country skiing Squash Basketball Biathlon Ice hockey Field hockey Rugby Soccer Cross-country skiing (skating) Running (mid/long) Swimming Tennis (single) Team handball III. High static Bobsledding Field events (throwing) Luge Rock climbing Waterskiing Weight lifting Windsurfing Body building Downhill skiing Wrestling Snow boarding Boxing Canoeing, Kayaking Cycling, Decathlon Rowing Speed skating Triathlon