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
Conflict of Interest statement none B Gerber UCL Brussels
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
cmr to evaluate LV mass, EDV, wall thickness Simpson s method Vol i 0 n A i th LVmass = Vol epic. -Vol endoc. 1.06 Wall thickness
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
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, 417 426
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
Remodeling by Echo vs sport type Endurance Sports Resistance sport Spiriti Am Heart J 1994 Pellici Prog Cardiovasc Dis 2012;54:387-396
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 20 10 0-10 20 10 0-10 Endurance Training * * NS NS LVM EDV ESV IVS PWT * Resistance Training NS NS NS NS NS LVM EDV ESV IVS PWT Spence J Physiol 589.22 (2011) pp 5443 5452 Trained Detrained Trained Detrained
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 2010 29 triathletes 115±12 83±8 124±113 28±2 Scharf Am Heart J 2010 26 soccer pl. 112±12 81±9 118±10 26±2 Perseghing Am H J 2007 14 sprinters 82±8 82±10 100±7 26±4 9 marathon runners 89±16 94±20 95±12 32±5 Steding J CMRI 2010 11 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. 2012 20 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. 2009 Normal vounteers 82±11 95±10 Speaker
Cardiac MRI reference values for athletes and nonathletes corrected for BSA, training itensity and sex +29% = c +40% Prakken Eur J Cardiov. Prev & Rehab 2010 17: 198
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, 417 426
Exercise increase LV mass and LV volumes in proportion to physical capacity Scharhag J Am Coll Cardiol 2002;40:1856 63 Franzen Heart Vessels 2012 Speaker
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
Athletes present similar hypertrophy of left and right ventricle Scharhag J Am Coll Cardiol 2002;40:1856 63 Speaker
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 (40.3 78.9) 75.8 ± 15.5 (55.0 125.7) 85.0 ± 27.3 (48.1 161.3) 75.6 ± 10.1 (51.4 93.6) 93.7 ± 40.1 (46.9 218.2) 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 (9.3-12.6) 12.8±1.8* (9.7-16.6) 21.5±5.9 (14.3-36.5) 17.0±2.6 (13.2-22.4) 19.4±3.8 (13.1-26.6) <.01 Dias. WT/EDVi (mm*m2/ml) 0.14-0.03 (0.1-0.2) 0.13±0.02* (0.1-0.2) 0.29±0.10 (0.2-0.5) 0.23±0.07 (0.2-0.4) 0.28±0.10 (0.1-0.6) <.01 Sys.WT/ESVi (mm*m2/ml) 0.56±0.23 (0.3-1.0) 0.42±0.15)* (0.2-1.0) 0.86±0.32 (0.4-1.9) 0.92±0.36 (0.4-1.7) 1.22±0.82 (0.33-3.3) <.01 *: p<.01 vs all other hypertrophy groups : p<.01 between groups Petersen, JCMRI (2005) 7, 551 558
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% 0.955 diast Wtmax/ diast WT min <1.3 28% 95% 64% 95% 0.862 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% 0.926 Petersen, JCMRI (2005) 7, 551 558
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
Phenotype of CMH by MRI Maron JACC 2009 Vol. 54, No. 3, 220 8
Phenotype of CMH by MRI % Segments > 15 mm Maron JACC 2009 Vol. 54, No. 3, 220 8
DE-cMR detects myocardial fibrosis in CMH Fibrosis can be present in mutation carriers prior to hypertrophy Moon J Am Coll Cardiol 2004;43:2260 4 Ho N Engl J Med 2010;363:552-63
Phenotype of CMH by MRI: Late-enhancement Moon, JACC 2003, 41:1561 7
Phenotype of CMH by MR Rudolph JACC 2009, 53 (3):284 91
Incidence of LGE in HCM by CMR Author Nr of pts % with LGE O Hanlon et al. 2010 JACC 217 63% Bruder et al. 2010 JACC 220 67% Ho et al. 2010 NEJM 28 71% Rubinshtein et al.2010 Circ. Heart Failure 424 56% Kown et al. 2009 JACC 60 63% Rudolph et al.2009 JACC 36 72% Maron et al.2008 Circ. Heart Failure 202 55% Adabag et al.2008 JACC 177 41% Kwon et al. 2008 Int. J. of Cardiov. Imaging 68 57% Abdel Aty et al.2008 J MRI 27 33% Paya et al. 2008 J. of Cardiac Failure 120 69% Melacini et al. 2008 Int. J. Cardiology 44 80% Kim et al. 2008 J MRI 25 84% Debl et al. 2006 Heart 22 73% Soler et al. 2006 J CAT 53 56% Teraoke et al. 2004 MRI 59 76% Bogaert et al. 2003 A. J Roentgol. 11 64% Choudhury et al. 2002 JACC 21 81% All 1814 65% [range, 33-84%] Noureldin et al. J CV MRI 2012,14:17
DE-MR: Prediction of events in CMH O Hanlon J Am Coll Cardiol 2010; 56(11):867-7 Bruder J Am Coll Cardiol 2010;56 875-87
cmr of other infiltrative diseases Anderson Fabry s Disease Amyloidosis Maceira Circulation. 2005;111:195-202 Sarcoidosis Moon Eur Heart J. 2003 Dec;24(23):2151-5 Glycogen storage disease Serra Circulation 2003;107;e188-e189 Fiocchi Clinical Imaging 32 (2008) 474 476
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 110:1622-1626, 2011. 12 young (n=31 years) athletes (Olympic champions running, penathlon) Hansen (Clinical Science 2011 120, 143 152) 28 marathon runners Moussavi (Am J Cardiol 2009;103:1467 1472) 14 marathon runners O Hanlon (J. Cv Magn. Res. 2010, 12:38) 17 recreational athletes Trivax (J Appl Physiol 108: 1148 1153, 2010) 25 marathon runners
Is there LGE in athletes? But it can be observed in some old long-time athletes LaGeche (Eur. Heart J. (2012) 33, 998 1006) 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 (162+26 vs. 144+16%, P ¼ 0.036) Wilson (J Appl Physiol 110:1622-1626, 2011) 50% (6/12) of old (55±6 years) veteran athletes Patterns are however always different from HCM
Role of drugs and doping in LV hypertrophy? Weight-lifter using anabolic steroids Weight-lifter Not using steroids Luycks Int J Cardiol. 2012 Mar 27
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
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
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