Correspondence
Athletes with Repolarization Abnormalities
N Engl J Med 2008; 358:2296-2298May 22, 2008
- Article
To the Editor:
Pelliccia et al. (Jan. 10 issue)1 report long-term follow-up data on 81 athletes with deeply inverted T waves on electrocardiograms (ECGs), who had no apparent cardiac disease, selected from a cohort of 12,550 trained athletes. Among these 81 athletes, 5 (6%) ultimately proved to have cardiomyopathies, and 3 of the 5 had hypertrophic cardiomyopathy. We would like to reconsider the relation between deeply inverted T waves and hypertrophic cardiomyopathy in apparently healthy young persons, whether or not they are athletes. In our opinion, myocardial hypertrophy does not represent the true hypertrophic cardiomyopathy phenotype. Geisterfer-Lowrance et al.2 have demonstrated that in a mouse model of familial hypertrophic cardiomyopathy, myocyte disarray precedes the development of hypertrophy, whereas ECG changes appear only when the disarray becomes evident. Clinical data support this finding.3 Tissue Doppler and strain-rate imaging may be useful in preclinical diagnosis of the disease.4,5 The presence of deeply inverted T waves on an ECG in an otherwise healthy young person may represent hypertrophic cardiomyopathy, regardless of anatomy, and probably should be considered a reason for disqualification from competitive sports.
5 ReferencesGeorgios K. Efthimiadis, M.D.
Soultana Meditskou, M.D.
Georgios E. Parcharidis, M.D.
Aristotle University of Thessaloniki, 54636 Thessaloniki, Greece
efthymos@med.auth. gr 1
Pelliccia A ,Di Paolo FM ,Quattrini FM , et al. Outcomes in athletes with marked ECG repolarization abnormalities. N Engl J Med 2008;358:152-161
Full Text | Web of Science | Medline2
Geisterfer-Lowrance AA ,Christe M ,Conner DA , et al. A mouse model of familial hypertrophic cardiomyopathy. Science 1996;272:731-734
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McKenna WJ ,Stewart JT ,Nihoyannopoulos P ,McGinty F ,Davies MJ . Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass. Br Heart J 1990;63:287-290
CrossRef | Web of Science | Medline4
Ganame J ,Mertens L ,Eidem BW , et al. Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations. Eur Heart J 2007;28:2886-2894
CrossRef | Web of Science | Medline5
Nagueh SF ,Bachinski LL ,Meyer D , et al. Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy. Circulation 2001;104:128-130
Web of Science | Medline
To the Editor:
Pelliccia et al. report two cardiac arrests, one of which was fatal, among 81 athletes with marked repolarization abnormalities but normal echocardiograms over a mean follow-up period of 9 years. These findings undermine the reassurance provided by a normal echocardiogram in the setting of ECG repolarization abnormalities and the concept of benign “athlete's heart.” Pelliccia and his colleagues have previously advised screening with ECG before participation in athletic events because of the attendant risk of sudden death from cardiac causes in this population (1 in 100,000). Why then do they recommend continued annual surveillance rather than disqualification from competitive sports for this subgroup of athletes who have a risk of sudden death from cardiac causes or aborted cardiac arrest of 1 in 40? Surveillance echocardiography failed to identify the patient who died of right ventricular cardiomyopathy, and the echocardiographic findings were not convincingly abnormal in the patient who received a diagnosis of hypertrophic cardiomyopathy after a cardiac arrest with a 13-mm septum. It appears unlikely that echocardiography will provide adequate surveillance in this subgroup of athletes, but if surveillance rather than disqualification is being considered, other methods such as magnetic resonance imaging may be required.
Joseph M. Galvin, F.R.C.P.I.
Connolly Hospital, Dublin 15, Ireland
joseph.galvin@ireland. com To the Editor:
We commend Pelliccia and colleagues for highlighting the importance of clinical surveillance in athletes with abnormal ECGs. However, the risk of cardiomyopathy in this cohort may be even higher than suggested.
Echocardiography is frequently suboptimal for identifying apical and right ventricular abnormalities; using supplementary techniques may therefore improve the diagnostic yield. Tissue Doppler studies can predict the development of hypertrophic cardiomyopathy in patients with subclinical disease.1 Contrast echocardiography and cardiovascular magnetic resonance (CMR) imaging provide better visualization of apical abnormalities in noncompaction cardiomyopathy and apical hypertrophic cardiomyopathy2 and of right ventricular thickening in hypertrophic cardiomyopathy.3 Accurate identification of right ventricular enlargement and dysfunction is particularly important in arrhythmogenic right ventricular cardiomyopathy, and tissue characterization by CMR imaging may confirm fatty infiltration.4 Contrast-enhanced CMR imaging may provide additional diagnostic and prognostic information in hypertrophic cardiomyopathy and dilated cardiomyopathy.2
Given the inherited component of many cardiomyopathies, a positive family history may also clinch the diagnosis, even when clinical manifestations are absent.
Therefore, family screening and additional imaging are prudent in young persons with ECG abnormalities in whom standard evaluation fails to yield a diagnosis.
4 ReferencesJayanth R. Arnold, B.M., B.Ch.
Theodoros D. Karamitsos, M.D., Ph.D.
Steffen E. Petersen, M.D., D.Phil.
University of Oxford, Oxford OX3 9DU, United Kingdom
ranjitarnold@yahoo.co. uk 1
Nagueh SF ,McFalls J ,Meyer D , et al. Tissue Doppler imaging predicts the development of hypertrophic cardiomyopathy in subjects with subclinical disease. Circulation 2003;108:395-398
CrossRef | Web of Science | Medline2
Assomull RG ,Pennell DJ ,Prasad SK . Cardiovascular magnetic resonance in the evaluation of heart failure. Heart 2007;93:985-992
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Maron MS ,Hauser TH ,Dubrow E , et al. Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol 2007;100:1293-1298
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Tandri H ,Macedo R ,Calkins H , et al. Role of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia: insights from the North American Arrhythmogenic Right Ventricular Dysplasia (ARVD/C) study. Am Heart J 2008;155:147-153
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Author/Editor Response
We disagree with certain inferences that Efthimiadis et al. make from our data. First, there is compelling evidence, assembled over a period of 50 years, that the most consistent manifestation of the hypertrophic cardiomyopathy phenotype is left ventricular hypertrophy.1 To argue otherwise is to unnecessarily inject confusion into the clinical assessment of this disease. However, it is also true, as shown in our study and several others, that other manifestations (including ECG alterations and left ventricular filling abnormalities) can precede the appearance of left ventricular hypertrophy in some young people who inherit a mutant gene for hypertrophic cardiomyopathy.
Second, and more important, it was not our intention to suggest that an abnormal ECG repolarization pattern should itself justify disqualification from competitive sports. This would be inconsistent with both the 36th Bethesda Conference2 and the European Society of Cardiology3 recommendations, given the uncommon occurrence of these ECG patterns in the vast athlete population, as well as the rarity with which these abnormalities predict future cardiac disease and events. However, we do suggest for such athletes a prudent strategy of systematic surveillance with echocardiography and probably CMR imaging.
Galvin and Arnold et al. have raised an issue similar to that raised by Efthimiadis et al., but with additional questions concerning the most effective strategies for long-term assessment of athletes who may have a proclivity for delayed development of the hypertrophic cardiomyopathy phenotype or the arrhythmogenic right ventricular cardiomyopathy phenotype. This is an important point that deserves emphasis. For example, conventional echocardiographic imaging may be incapable of reliably confirming the diagnosis of hypertrophic cardiomyopathy in some patients. Indeed, CMR imaging may identify segmental hypertrophy in either the apex or the anterolateral left ventricular free wall4 that is undetected by echocardiography. Furthermore, as indicated by Arnold et al., tissue characterization by CMR imaging may also identify fatty infiltration of the right ventricle, allowing for the diagnosis of arrhythmogenic right ventricular cardiomyopathy. Therefore, contemporary follow-up in this selected subgroup of trained athletes with marked repolarization changes on ECG should include both echocardiography and CMR imaging.
4 ReferencesAntonio Pelliccia, M.D.
Institute of Sports Medicine and Science, 00197 Rome, Italy
ant.pelliccia@libero. it Barry J. Maron, M.D.
Minneapolis Heart Institute Foundation, Minneapolis, MN 554071
Maron BJ ,McKenna WJ ,Danielson GK , et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. J Am Coll Cardiol 2003;42:1687-1713
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Maron BJ ,Zipes DP . 36th Bethesda Conference: eligibility recommendations for competitive athletes with cardiovascular abnormalities. J Am Coll Cardiol 2005;45:1312-1375
CrossRef3
Pelliccia A ,Fagard B ,Bjornstad HH , et al. Recommendations for competitive sports participation in athletes with cardiovascular disease: a consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. Eur Heart J 2005;26:1422-1445
CrossRef | Web of Science | Medline4
Rickers C ,Wilke NM ,Jerosch-Herold M , et al. Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation 2005;112:855-861
CrossRef | Web of Science | Medline
