To the Editor: Pelliccia et al. (Jan. 10 issue)1 report long-termfollow-up data on 81 athletes with deeply inverted T waves onelectrocardiograms (ECGs), who had no apparent cardiac disease,selected from a cohort of 12,550 trained athletes. Among these81 athletes, 5 (6%) ultimately proved to have cardiomyopathies,and 3 of the 5 had hypertrophic cardiomyopathy. We would liketo reconsider the relation between deeply inverted T waves andhypertrophic cardiomyopathy in apparently healthy young persons,whether or not they are athletes. In our opinion, myocardialhypertrophy does not represent the true hypertrophic cardiomyopathyphenotype. Geisterfer-Lowrance et al.2 have demonstrated thatin a mouse model of familial hypertrophic cardiomyopathy, myocytedisarray precedes the development of hypertrophy, whereas ECGchanges appear only when the disarray becomes evident. Clinicaldata support this finding.3 Tissue Doppler and strain-rate imagingmay be useful in preclinical diagnosis of the disease.4,5 Thepresence of deeply inverted T waves on an ECG in an otherwisehealthy young person may represent hypertrophic cardiomyopathy,regardless of anatomy, and probably should be considered a reasonfor disqualification from competitive sports.
Georgios K. Efthimiadis, M.D. Soultana Meditskou, M.D. Georgios E. Parcharidis, M.D. Aristotle University of Thessaloniki 54636 Thessaloniki, Greece efthymos{at}med.auth.gr
References
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. [Free Full Text]
Geisterfer-Lowrance AA, Christe M, Conner DA, et al. A mouse model of familial hypertrophic cardiomyopathy. Science 1996;272:731-734. [Abstract]
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. [Free Full Text]
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. [Free Full Text]
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. [Free Full Text]
To the Editor: Pelliccia et al. report two cardiac arrests,one of which was fatal, among 81 athletes with marked repolarizationabnormalities but normal echocardiograms over a mean follow-upperiod of 9 years. These findings undermine the reassuranceprovided by a normal echocardiogram in the setting of ECG repolarizationabnormalities and the concept of benign "athlete's heart." Pellicciaand his colleagues have previously advised screening with ECGbefore participation in athletic events because of the attendantrisk of sudden death from cardiac causes in this population(1 in 100,000). Why then do they recommend continued annualsurveillance rather than disqualification from competitive sportsfor this subgroup of athletes who have a risk of sudden deathfrom cardiac causes or aborted cardiac arrest of 1 in 40? Surveillanceechocardiography failed to identify the patient who died ofright ventricular cardiomyopathy, and the echocardiographicfindings were not convincingly abnormal in the patient who receiveda diagnosis of hypertrophic cardiomyopathy after a cardiac arrestwith a 13-mm septum. It appears unlikely that echocardiographywill 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{at}ireland.com
To the Editor: We commend Pelliccia and colleagues for highlightingthe importance of clinical surveillance in athletes with abnormalECGs. However, the risk of cardiomyopathy in this cohort maybe even higher than suggested.
Echocardiography is frequently suboptimal for identifying apicaland right ventricular abnormalities; using supplementary techniquesmay therefore improve the diagnostic yield. Tissue Doppler studiescan predict the development of hypertrophic cardiomyopathy inpatients with subclinical disease.1 Contrast echocardiographyand cardiovascular magnetic resonance (CMR) imaging providebetter visualization of apical abnormalities in noncompactioncardiomyopathy and apical hypertrophic cardiomyopathy2 and ofright ventricular thickening in hypertrophic cardiomyopathy.3Accurate identification of right ventricular enlargement anddysfunction is particularly important in arrhythmogenic rightventricular cardiomyopathy, and tissue characterization by CMRimaging may confirm fatty infiltration.4 Contrast-enhanced CMRimaging may provide additional diagnostic and prognostic informationin hypertrophic cardiomyopathy and dilated cardiomyopathy.2
Given the inherited component of many cardiomyopathies, a positivefamily history may also clinch the diagnosis, even when clinicalmanifestations are absent.
Therefore, family screening and additional imaging are prudentin young persons with ECG abnormalities in whom standard evaluationfails to yield a diagnosis.
Jayanth 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{at}yahoo.co.uk
References
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. [Free Full Text]
Assomull RG, Pennell DJ, Prasad SK. Cardiovascular magnetic resonance in the evaluation of heart failure. Heart 2007;93:985-992. [Free Full Text]
Maron MS, Hauser TH, Dubrow E, et al. Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol 2007;100:1293-1298. [CrossRef][Web of Science][Medline]
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. [CrossRef][Web of Science][Medline]
The authors reply: We disagree with certain inferences thatEfthimiadis et al. make from our data. First, there is compellingevidence, assembled over a period of 50 years, that the mostconsistent manifestation of the hypertrophic cardiomyopathyphenotype is left ventricular hypertrophy.1 To argue otherwiseis to unnecessarily inject confusion into the clinical assessmentof this disease. However, it is also true, as shown in our studyand several others, that other manifestations (including ECGalterations and left ventricular filling abnormalities) canprecede the appearance of left ventricular hypertrophy in someyoung people who inherit a mutant gene for hypertrophic cardiomyopathy.
Second, and more important, it was not our intention to suggestthat an abnormal ECG repolarization pattern should itself justifydisqualification from competitive sports. This would be inconsistentwith both the 36th Bethesda Conference2 and the European Societyof Cardiology3 recommendations, given the uncommon occurrenceof these ECG patterns in the vast athlete population, as wellas the rarity with which these abnormalities predict futurecardiac disease and events. However, we do suggest for suchathletes a prudent strategy of systematic surveillance withechocardiography and probably CMR imaging.
Galvin and Arnold et al. have raised an issue similar to thatraised by Efthimiadis et al., but with additional questionsconcerning the most effective strategies for long-term assessmentof athletes who may have a proclivity for delayed developmentof the hypertrophic cardiomyopathy phenotype or the arrhythmogenicright ventricular cardiomyopathy phenotype. This is an importantpoint that deserves emphasis. For example, conventional echocardiographicimaging may be incapable of reliably confirming the diagnosisof hypertrophic cardiomyopathy in some patients. Indeed, CMRimaging may identify segmental hypertrophy in either the apexor the anterolateral left ventricular free wall4 that is undetectedby echocardiography. Furthermore, as indicated by Arnold etal., tissue characterization by CMR imaging may also identifyfatty infiltration of the right ventricle, allowing for thediagnosis of arrhythmogenic right ventricular cardiomyopathy.Therefore, contemporary follow-up in this selected subgroupof trained athletes with marked repolarization changes on ECGshould include both echocardiography and CMR imaging.
Antonio Pelliccia, M.D. Institute of Sports Medicine and Science 00197 Rome, Italy ant.pelliccia{at}libero.it
Barry J. Maron, M.D. Minneapolis Heart Institute Foundation Minneapolis, MN 55407
References
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. [Free Full Text]
Maron BJ, Zipes DP. 36th Bethesda Conference: eligibility recommendations for competitive athletes with cardiovascular abnormalities. J Am Coll Cardiol 2005;45:1312-1375. [Free Full Text]
Pelliccia A, Fagard B, Bjørnstad 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. [Free Full Text]
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. [Free Full Text]
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