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

Wall thickness

ni

i thAVol0

𝐿𝑉𝑚𝑎𝑠𝑠 = 𝑉𝑜𝑙𝑒𝑝𝑖𝑐.-𝑉𝑜𝑙𝑒𝑛𝑑𝑜𝑐. ∗ 1.06

Interstudy Reproducibility cMR - Echo

0%

5%

10%

15%

20%

EDVi ESVi EF LV mass

Inte

rstu

dy

CV

Echo

MR

Groethues Am J Cardiol 2002;90:29–34

cMR normal values of indexed LV mass and volumes vs age and sex

78 g/m2

95 g/m2

Maceira J CV Mag Res Img (2006) 8, 417–426

LV mass

EDVi

male female

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

Spiriti Am Heart J 1994 Pellici Prog Cardiovasc Dis 2012;54:387-396

Endurance Sports

Resistance sport

cMR Echo

LV Adaptation to exercise by MRI vs type of training

25 healthy

male voluteers

(27±1 yr)

BL

randomization cMR Echo

6 mo

cMR Echo

Detraining 6 weeks

n=10 6 mo endurance training

n=13 6 mo resistance training end

-10

0

10

20

LVM EDV ESV IVS PWTCh

ange

fro

m

BL

(%)

Endurance Training

Trained

Detrained

* *

-10

0

10

20

LVM EDV ESV IVS PWTCh

ange

fro

m

BL

(%)

Resistance Training

Trained

Detrained

NS NS

NS NS NS

*

*

NS NS

Spence J Physiol 589.22 (2011) pp 5443–5452

Lv and RV mass and EDV by SSFP-MRI in different young male athletes

Speaker

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

Cardiac MRI reference values for athletes and nonathletes corrected for BSA, training itensity and sex

Prakken Eur J Cardiov. Prev & Rehab 2010 17: 198

=

c

+29%

+40%

cMR athletes vs normal values of indexed LV mass and volumes by age and sex

Maceira J CV Mag Res Img (2006) 8, 417–426

LV mass

EDVi

male female

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

Exercise increase LV mass and LV volumes in proportion to physical capacity

Speaker

Scharhag J Am Coll Cardiol 2002;40:1856–63

Franzen Heart Vessels 2012

Exercise increases LV mass and RV mass in direct proportion to LV EDV and RV EDV

Scharf Am Heart J 2010,;159:911-8 Sharf Radiology 257 1:71-79, 2010

29 soccer players

26 triathletes

Athletes present similar hypertrophy of left and right ventricle

Speaker

Scharhag J Am Coll Cardiol 2002;40:1856–63

Differentiation of Athletes heart: Overlap of LV mass: CMH vs other diseases

Normals

Hypertrophy

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

Petersen, JCMRI (2005) 7, 551–558

*: p<.01 vs all other hypertrophy groups

¶: p<.01 between groups

cMR Differentiation of CMH vs Athletes heart by morphology

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

CMH Athletes Heart

Phenotype of hypertrophy in CMH by MRI

a) Focal Sept AS b) Intermediate

2 non contiguous regions

Maron JACC 2009 Vol. 54, No. 3, 220–8

c) Diffuse

Phenotype of CMH by MRI

Maron JACC 2009 Vol. 54, No. 3, 220–8

Phenotype of CMH by MRI

Maron JACC 2009 Vol. 54, No. 3, 220–8

% Segments > 15 mm

DE-cMR detects myocardial fibrosis in CMH

Fibrosis can be present

in mutation carriers

prior to hypertrophy

Ho N Engl J Med 2010;363:552-63

Moon J Am Coll Cardiol 2004;43:2260–4

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

Moon Eur Heart J. 2003 Dec;24(23):2151-5

Anderson Fabry’s Disease

Fiocchi Clinical Imaging 32 (2008) 474–476

Glycogen storage disease

Amyloidosis

Serra Circulation 2003;107;e188-e189

Sarcoidosis

Maceira Circulation. 2005;111:195-202

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

B Gerber UCL Brussels

Indexed Lvmass: 199 g/m2

LV thickness 23 mm

Conclusions Identification of Athletes Heart by cmR

Athletes Heart HCM

LV mass Moderately increased correlated to VO2 max

variable

LV-EDVi Moderately increased correlated to VO2 max

Maintained, reduced

LV mass/EDV Unchanged (eccentric hypertrophy)

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