Bernhard L. Gerber. Differentiating HCM from athletes heart. Role of contrast enhanced MR.

Transcription

1 Differentiating HCM from athletes heart. Role of contrast enhanced MR. Bernhard L. Gerber MD PhD FACC FESC Division of Cardiology Dpt. of Cardiovascular Diseases Cliniques Universitaires St.Luc UCL, Bruxelles Belgium

2 Conflict of Interest statement none B Gerber UCL Brussels

3 The problem of LVH in athletes Differential diagnosis Athletes heart HCM related to sarcomere mutations Steroid or other drug induced cardiomyopathy Other causes of hypertrophy HTA Aortic stenosis Infiltratative diseases Fabry s, Amyloidosis Tumor Fibroma Myoma

4 cmr to evaluate LV mass, EDV, wall thickness Simpson s method Vol i 0 n A i th LVmass = Vol epic. -Vol endoc Wall thickness

5 Interstudy CV Interstudy Reproducibility cmr - Echo 20% 15% 10% 5% Echo MR 0% EDVi ESVi EF LV mass Groethues Am J Cardiol 2002;90:29 34

6 cmr normal values of indexed LV mass and volumes vs age and sex LV mass male female 95 g/m2 78 g/m2 EDVi Maceira J CV Mag Res Img (2006) 8,

7 LV adaptation in Athletes Morganroth hypothesis endurance athletes (swimmers, long-distance runners) increase LVEDV and LVmass with normal LV wall thickness (eccentric LV hypertrophy) Supposedly from increased preload resistance-trained athletes (wrestlers) increase LV wall thickness and LV mass but not LVEDV (concentric LV hypertrophy). Supposedly from increased afterload

8 Remodeling by Echo vs sport type Endurance Sports Resistance sport Spiriti Am Heart J 1994 Pellici Prog Cardiovasc Dis 2012;54:

9 Change from BL (%) Change from BL (%) LV Adaptation to exercise by MRI vs type of training 25 healthy male voluteers (27±1 yr) cmr Echo BL n=10 6 mo endurance training cmr Detraining cmr randomization Echo 6 weeks Echo n=13 6 mo resistance training 6 mo end Endurance Training * * NS NS LVM EDV ESV IVS PWT * Resistance Training NS NS NS NS NS LVM EDV ESV IVS PWT Spence J Physiol (2011) pp Trained Detrained Trained Detrained

10 Lv and RV mass and EDV by SSFP-MRI in different young male athletes EDVi (ml/m2) Lvmassi (g/m2) RV EDVi (ml/m2) RV mass (g/m2) Scharf Radiol triathletes 115±12 83±8 124±113 28±2 Scharf Am Heart J soccer pl. 112±12 81±9 118±10 26±2 Perseghing Am H J sprinters 82±8 82±10 100±7 26±4 9 marathon runners 89±16 94±20 95±12 32±5 Steding J CMRI handball 121±19 69±10 122±16 18 soccer 122±14 73±13 127±17 12 triathlon 119±14 83±10 127±13 Franzen Heart Vess triathletes 107±4 79±2 125±5 20 marathon runners 99±3 73±2 108±4 Maceira J CMR 2006/Eur Heartj 2006 Normal volunteers 86±9 76±8 91±12 36±7 Hudsmith JCMR 2005 Normal volunteers 82±13 63±9 96±15 41±8 Cain BMC Med. Im Normal vounteers 82±11 95±10 Speaker

11 Cardiac MRI reference values for athletes and nonathletes corrected for BSA, training itensity and sex +29% = c +40% Prakken Eur J Cardiov. Prev & Rehab : 198

12 cmr athletes vs normal values of indexed LV mass and volumes by age and sex LV mass male female EDVi Prakken Eur J Prev Card 2010 Schar Radiol 2010 Schar Am Heart J 2010 Steding J CV MR 2010 Prakken Int J Cardiol 2011 Franzen Heart V 2012 Perseghing Am Heart J 2007 Maceira J CV Mag Res Img (2006) 8,

13 Exercise increase LV mass and LV volumes in proportion to physical capacity Scharhag J Am Coll Cardiol 2002;40: Franzen Heart Vessels 2012 Speaker

14 Exercise increases LV mass and RV mass in direct proportion to LV EDV and RV EDV 29 soccer players 26 triathletes Scharf Am Heart J 2010,;159:911-8 Sharf Radiology 257 1:71-79, 2010

15 Athletes present similar hypertrophy of left and right ventricle Scharhag J Am Coll Cardiol 2002;40: Speaker

16 Differentiation of Athletes heart: Overlap of LV mass: CMH vs other diseases Hypertrophy Normals Athletes Heart CMH HTA Aortic Stenosis p LV mass index (g/m2) 55.6 ± 9.9 ( ) 75.8 ± 15.5 ( ) 85.0 ± 27.3 ( ) 75.6 ± 10.1 ( ) 93.7 ± 40.1 ( ) NS EDVi (ml/m2) 79±12 (63-101) 99±11* (80-115) 77±14 (47-111) 76±12 (58-94) 76±25 (54-90) <.01 Diast Wth (mm) 11.1±1.1 ( ) 12.8±1.8* ( ) 21.5±5.9 ( ) 17.0±2.6 ( ) 19.4±3.8 ( ) <.01 Dias. WT/EDVi (mm*m2/ml) ( ) 0.13±0.02* ( ) 0.29±0.10 ( ) 0.23±0.07 ( ) 0.28±0.10 ( ) <.01 Sys.WT/ESVi (mm*m2/ml) 0.56±0.23 ( ) 0.42±0.15)* ( ) 0.86±0.32 ( ) 0.92±0.36 ( ) 1.22±0.82 ( ) <.01 *: p<.01 vs all other hypertrophy groups : p<.01 between groups Petersen, JCMRI (2005) 7,

17 cmr Differentiation of CMH vs Athletes heart by morphology CMH Athletes Heart Sens Spec PPV NPV AUC Max Diastolic wall thickness <13 mm 40% 100% 100% 84% diast Wtmax/ diast WT min <1.3 28% 95% 64% 95% Diastolic WT/LVEDVI <0.15 mm.m2.ml 80% 99% 95% 94% 0.93 Systolic WT/LVESVI <0.26 mm.m2.m; 4% 100% 100% 76% Petersen, JCMRI (2005) 7,

18 Phenotype of hypertrophy in CMH by MRI a) Focal Sept AS b) Intermediate 2 non contiguous regions c) Diffuse Maron JACC 2009 Vol. 54, No. 3, 220 8

19 Phenotype of CMH by MRI Maron JACC 2009 Vol. 54, No. 3, 220 8

20 Phenotype of CMH by MRI % Segments > 15 mm Maron JACC 2009 Vol. 54, No. 3, 220 8

21 DE-cMR detects myocardial fibrosis in CMH Fibrosis can be present in mutation carriers prior to hypertrophy Moon J Am Coll Cardiol 2004;43: Ho N Engl J Med 2010;363:552-63

22 Phenotype of CMH by MRI: Late-enhancement Moon, JACC 2003, 41:1561 7

23 Phenotype of CMH by MR Rudolph JACC 2009, 53 (3):284 91

24 Incidence of LGE in HCM by CMR Author Nr of pts % with LGE O Hanlon et al JACC % Bruder et al JACC % Ho et al NEJM 28 71% Rubinshtein et al.2010 Circ. Heart Failure % Kown et al JACC 60 63% Rudolph et al.2009 JACC 36 72% Maron et al.2008 Circ. Heart Failure % Adabag et al.2008 JACC % Kwon et al Int. J. of Cardiov. Imaging 68 57% Abdel Aty et al.2008 J MRI 27 33% Paya et al J. of Cardiac Failure % Melacini et al Int. J. Cardiology 44 80% Kim et al J MRI 25 84% Debl et al Heart 22 73% Soler et al J CAT 53 56% Teraoke et al MRI 59 76% Bogaert et al A. J Roentgol % Choudhury et al JACC 21 81% All % [range, 33-84%] Noureldin et al. J CV MRI 2012,14:17

25 DE-MR: Prediction of events in CMH O Hanlon J Am Coll Cardiol 2010; 56(11):867-7 Bruder J Am Coll Cardiol 2010;

26 cmr of other infiltrative diseases Anderson Fabry s Disease Amyloidosis Maceira Circulation. 2005;111: Sarcoidosis Moon Eur Heart J Dec;24(23): Glycogen storage disease Serra Circulation 2003;107;e188-e189 Fiocchi Clinical Imaging 32 (2008)

27 Is there LGE in athletes? NO, typically not (at least in young athletes) Franzen (Heart Vessels 2012) 20 triathletes (38 years) and 20 long distance runners (44 years) Wilson (J Appl Physiol 110: , young (n=31 years) athletes (Olympic champions running, penathlon) Hansen (Clinical Science , ) 28 marathon runners Moussavi (Am J Cardiol 2009;103: ) 14 marathon runners O Hanlon (J. Cv Magn. Res. 2010, 12:38) 17 recreational athletes Trivax (J Appl Physiol 108: , 2010) 25 marathon runners

28 Is there LGE in athletes? But it can be observed in some old long-time athletes LaGeche (Eur. Heart J. (2012) 33, ) 12.5% (5/40) of a sample of athletes (marathon runners, endurance triathlon runners, ultra triathlon, alpine cyclers) Athletes with DE were competing in endurance sports for longer (20±16 vs. 8±6 years) and had higher VO2max for age ( vs %, P ¼ 0.036) Wilson (J Appl Physiol 110: , 2011) 50% (6/12) of old (55±6 years) veteran athletes Patterns are however always different from HCM

29 Role of drugs and doping in LV hypertrophy? Weight-lifter using anabolic steroids Weight-lifter Not using steroids Luycks Int J Cardiol Mar 27

30 Coexistance of pathologies Example 50 yo male Holds a fitness studio Weight lifts 6 h / day Used PO Testosterone IM Growth hormone SC insulin Severe aortic stenosis Indexed Lvmass: 199 g/m2 LV thickness 23 mm B Gerber UCL Brussels

31 Conclusions Identification of Athletes Heart by cmr LV mass LV-EDVi LV mass/edv Athletes Heart Moderately increased correlated to VO2 max Moderately increased correlated to VO2 max Unchanged (eccentric hypertrophy) HCM variable Maintained, reduced Increased (concentric hypertrophy) Wth <13 mm, homogenous >13 mm, heterogeneous WT/EDV ratio < 0.15 mm.m2.ml >0.15 mm.m2/ml RV EDVi Increased in parallel to LV EDVi maintained LGE Typically absent Frequently (65%) present Typical patterns B.Gerber UCL Brussels

32 Conclusions CMR is helpful to distinguish athletes heart from CHM Most athletes present with eccentric hypertrophy, characterized by increased LV-EDVi and RVEDVi, homogeneous wall thickening <13 mm and maintained LVEDV/mass and wall-thickness/edv Young athletes never present with late enhancement. However Late-enhancement by cmr is a typical finding in HCM and other pathologies, and predicts worse outcome in these diseases. Therefore LGE can be an important factor to distinguish athletes heart from HCM Overlap of pathologies may however exist, and there may be questions about the effect of performance enhanced drugs (doping) on LV remodeling in athletes. B Gerber UCL Brussels