ARVD/C and the athlete s heart: Application of revised Task Force Criteria

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1 ARVD/C and the athlete s heart: Application of revised Task Force Criteria T. Luijkx MD, B.K. Velthuis MD PhD, N.H.J. Prakken MD PhD, M.G.P.J. Cox MD, R.N.W. Hauer MD PhD, M.J.M. Cramer MD PhD Stockholm, 30 August 2010

2 ARVD/C ARVD/C: arrhythmogenic right ventricular dysplasia/cardiomyopathy fibrofatty replacement of myocardium RV dilatation, dysfunction and potentially fatal arrhythmias diagnosis by Task Force Criteria, revised in 2010

3 ARVD/C in athletes ARVD/C important cause of sudden cardiac death (SCD), especially in young athletes SCD can be the first manifestation of ARVD/C many cardiac MRI requests to rule out ARVD/C in athletes physiologic cardiac adaptation overlaps with changes seen in ARVD/C

4 ARVD/C Task Force Criteria (TFC) ARVD/C diagnosed at 4 TFC points or more (2 major / 1 major + 2 minor / 4 minor) I II III IV V VI global or regional dysfunction and structural alterations tissue characterization of wall repolarization abnormalities depolarization/conduction abnormalities arrhythmias family history

5 ARVD/C TFC: cardiac MRI I global or regional dysfunction and structural alterations Major criterion Severe dilatation + reduction of RV EF with no/mild LV impairment Localized RV aneurysms (a-/dyskinetic areas with diastolic bulging) Severe segmental dilatation of the RV Minor criterion Mild global RV dilatation and/or EF reduction with normal LV Mild segmental dilatation of the RV Regional RV hypokinesia

6 ARVD/C revised TFC: cardiac MRI (2010) I global or regional dysfunction and structural alterations Minor Major 1. Regional RV akinesia or dyskinesia or dyssynchronous RV contraction + 1 of the following: 2. RV end-diastolic volume (indexed to BSA, ml/m 2 ) > 90 (F) > 100 (M) > 100 (F) > 110 (M) RV ejection fraction < 45% < 40%

7 Moderator band bulging diastolic systolic

8 Methods Study population: ARVD/C patients (diagnosed with revised TFC) Matched for age and gender to: healthy non-athletes ( 3 hrs/physical training/wk) healthy athletes ( 9 hrs/physical training/wk) Cardiac MRI Analysis: left and right ventricular volumes, wall mass Evaluation of TFC cut-off values and new cut-off values

9 Methods: cardiac MRI analysis Contour tracing of ventricular volumes and wall mass (Prakken 2008)

10 Results: baseline characteristics Non-athletes Athletes ARVD/C patients sex (n) M (42) M (42) M (21) age height weight body surface area 2.08 * *significantly different from all other groups

11 Results: baseline characteristics Non-athletes Athletes ARVD/C patients sex (n) M (42) M (42) M (21) age height weight body surface area 2.08 * *significantly different from all other groups

12 Results: baseline characteristics Non-athletes Athletes ARVD/C patients sex (n) M (42) F (24) M (42) F (24) M (21) F (12) age height weight body surface area 2.08 * *significantly different from all other groups

13 Results: ventricular parameters Non-athletes Athletes ARVD/C patients sex (n) M (42) M (42) M (21) RV EF * RV EDV 106.1* 132.9* 149.0* RV ESV 49.2* 64.8* 100.5* RV EDD 44.8* LV EF * LV EDV * LV ESV 40.8* LV EDD * significantly different from all other groups

14 Results: ventricular parameters Non-athletes Athletes ARVD/C patients sex (n) M (42) F (24) M (42) F (24) M (21) F (12) RV EF * 34.4* 43.4* RV EDV 106.1* 90.9* 132.9* * RV ESV 49.2* 37.9* 64.8* 53.7* 100.5* 67.5* RV EDD 44.8* LV EF * 52.1 LV EDV * LV ESV 40.8* 35.0* LV EDD * significantly different from all other groups

15 Results: revised TFC cut-off values Non-athletes Athletes ARVD/C patients sex (n) M (42) F (24) M (42) F (24) M (21) F (12) RV EF minor (%) * 58* RV EF major (%) * 33* RV EDV minor (%) 62* RV EDV major (%) 50* 13* TFC minor (%) 62* TFC major (%) 50* * significantly different from all other groups

16 Observed patterns RV EF works well, RV EDV does not ARVD/C patients: Athletes: ARVD/C disproportionate RV alterations balanced biventricular alterations healthy athlete RV LV RV LV

17 Possible alternatives to RV measures Ratio measures of LV/RV (EF, EDV, ESV)

18 Results: ratio measures non-athletes athletes ARVD/C patients M F M F M F

19 Results: ratio measures cut-off values Non-athletes Athletes ARVD/C patients sex (n) M (42) F (24) M (42) F (24) M (21) F (12) Ratio EF > 1.20 (%) * 50* Ratio EF > 1.25 (%) * 41* Ratio EDV < 0.85 (%) * 42 Ratio EDV < 0.80 (%) * 33* Altered criteria minor (%) * 50* Altered criteria major (%) * 41* 1 combination of RV EF and Ratio EDV, * significantly different from all other groups

20 Conclusions For distinguishing athlete s heart from ARVD/C: RV EF is suitable RV EDV index is not suitable Good alternatives: Ratio EDV LV/RV Ratio ESV LV/RV RV ESV index No information on length and weight required

sensitivity Preference for Ratio EDV 100 - sensitivity Area under the

21 sensitivity Preference for Ratio EDV sensitivity Area under the curve: RV EF Ratio EDV RV ESV Ratio ESV RV EDV 0.683

22 Discussion No diseased athletes included Beyond the scope of this study: fat infiltration, late gadolinium enhancement, RV free wall thinning Presence of wall motion abnormality prerequisite for minor/major criterion!

23 References Prakken,N., B.Velthuis, E.Vonken, W.Mali, and M.Cramer, 2008, Cardiac MRI: standardized right and left ventricular quantification by briefly coaching inexperienced personnel., The Open Magnetic Resonance Journal 1,

Update on use of cardiac MRI in ARVC/D. Stefan L. Zimmerman, MD Johns Hopkins University Department of Radiology

Update on use of cardiac MRI in ARVC/D Stefan L. Zimmerman, MD Johns Hopkins University Department of Radiology Outline Background Diagnosis Characteristic imaging findings Genetics of ARVC Genotype phenotype