Competetive sports in high risk patients

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1 Competetive sports in high risk patients Marfan syndrome with a non (or mildly) dilated aortic root Maarten Groenink Academic Medical Center Amsterdam The Netherlands

2 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

3 Marfan celebrities Lincoln Pharao Achnaton Paganini Rachmaninoff

4 Marfan?

5 MS : specific advantages in sports?

6 The Marfan aorta

7 Aortic dissection Ascending aortic dissection : High risk of sudden death Cardiac tamponade Coronary occlusion Massive aortic regurgitation Aortic rupture

8 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

9 Causes of sudden death in athletes Circulation. 2011;123:

10 Causes of SCD in 347 athletes N Engl J Med 2003; 349:

11 Guidelines I ll be happy to give you innovative thinking. What are the guidelines?

12 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

13 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.

14 Forces that act on the aortic root outwardly directed forces Longitudinal forces twisting forces shear stress

15 Dynamics of the aortic root

16 Aneurysm formation and dissection in MS

17 Effects of dynamic and static activity Dynamic Static Dynamic Static Minutes J Am Coll Cardiol Apr 19;45(8):

18 Classification J Am Coll Cardiol Apr 19;45(8):

19 Weight lifting and aortic dissection Cardiology 2007;107:

20 Contact sports

21 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:

22 Classification J Am Coll Cardiol Apr 19;45(8):

23 Effect of highly dynamic and static sports on the aorta

24 Aortic complications in Marfan syndrome

25 Marfan and sports

26 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

27 Thank you

28 SCD by sport, ethnicity, sex (Circulation. 2011;123: )

29 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

30 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

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