Abstract
The comprehensive assessment of patients with hypertrophic cardiomyopathy is a complex process, with each step concurrently focusing on confirmation of the diagnosis, differentiation between sarcomeric and non-sarcomeric disease (phenocopy), and prognostication. Novel modalities such as genetic testing and advanced imaging have allowed for substantial advancements in the understanding of this condition and facilitate patient management. However, their availability is at present not universal, and interpretation requires a high level of expertise. In this setting, electrocardiography, a fast and widely available method, still retains a significant role in everyday clinical assessment of this population. In our review, we follow a stepwise approach for the interpretation of each electrocardiographic segment, discussing clinical implications of electrocardiographic patterns in sarcomeric disease, their value in the differential diagnosis from phenocopies, and impact on patient management. Outlining the substantial amount of information to be obtained from a simple tracing, we exhibit how electrocardiography is likely to remain an integral diagnostic tool in the future as well.
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Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW (2011) 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 124:2761–2796. https://doi.org/10.1161/CIR.0b013e318223e230
Elliott PM, Anastasakis A, Borger MA, Borggrefe M, Cecchi F, Charron P, Hagege AA, Lafont A, Limongelli G, Mahrholdt H, McKenna WJ, Mogensen J, Nihoyannopoulos P, Nistri S, Pieper PG, Pieske B, Rapezzi C, Rutten FH, Tillmanns C, Watkins H (2014) 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 35:2733–2779. https://doi.org/10.1093/eurheartj/ehu284
McLeod CJ, Ackerman MJ, Nishimura RA, Tajik AJ, Gersh BJ, Ommen SR (2009) Outcome of patients with hypertrophic cardiomyopathy and a normal electrocardiogram. J Am Coll Cardiol 54:229–233. https://doi.org/10.1016/j.jacc.2009.02.071
Siontis KC, Geske JB, Ong K, Nishimura RA, Ommen SR, Gersh BJ (2014) Atrial fibrillation in hypertrophic cardiomyopathy: prevalence, clinical correlations, and mortality in a large high-risk population. J Am Heart Assoc 3:e001002. https://doi.org/10.1161/jaha.114.001002
Guttmann OP, Rahman MS, O’Mahony C, Anastasakis A, Elliott PM (2014) Atrial fibrillation and thromboembolism in patients with hypertrophic cardiomyopathy: systematic review. Heart 100:465–472. https://doi.org/10.1136/heartjnl-2013-304276
Vaidya K, Semsarian C, Chan KH (2017) Atrial fibrillation in hypertrophic cardiomyopathy. Heart Lung Circ 26:975–982. https://doi.org/10.1016/j.hlc.2017.05.116
Rowin EJ, Hausvater A, Link MS, Abt P, Gionfriddo W, Wang W, Rastegar H, Estes NAM, Maron MS, Maron BJ (2017) Clinical profile and consequences of atrial fibrillation in hypertrophic cardiomyopathy. Circulation 136:2420–2436. https://doi.org/10.1161/circulationaha.117.029267
Papavassiliu T, Germans T, Flüchter S, Doesch C, Suriyakamar A, Haghi D, Süselbeck T, Wolpert C, Dinter D, Schoenberg SO, van Rossum AC, Borggrefe M (2009) CMR findings in patients with hypertrophic cardiomyopathy and atrial fibrillation. J Cardiovasc Magn Reson 11:34. https://doi.org/10.1186/1532-429x-11-34
Sivalokanathan S, Zghaib T, Greenland GV, Vasquez N, Kudchadkar SM, Kontari E, Lu DY, Dolores-Cerna K, van der Geest RJ, Kamel IR, Olgin JE, Abraham TP, Nazarian S, Zimmerman SL, Abraham MR (2019) Hypertrophic cardiomyopathy patients with paroxysmal atrial fibrillation have a high burden of left atrial fibrosis by cardiac magnetic resonance imaging. JACC Clin Electrophysiol 5:364–375. https://doi.org/10.1016/j.jacep.2018.10.016
Lee SE, Park JK, Uhm JS, Kim JY, Pak HN, Lee MH, Joung B (2017) Impact of atrial fibrillation on the clinical course of apical hypertrophic cardiomyopathy. Heart 103:1496–1501. https://doi.org/10.1136/heartjnl-2016-310720
Olivotto I, Cecchi F, Casey SA, Dolara A, Traverse JH, Maron BJ (2001) Impact of atrial fibrillation on the clinical course of hypertrophic cardiomyopathy. Circulation 104:2517–2524. https://doi.org/10.1161/hc4601.097997
Zegkos T, Efthimiadis GK, Parcharidou DG, Gossios TD, Giannakoulas G, Ntelios D, Ziakas A, Paraskevaidis S, Karvounis HI (2017) Atrial fibrillation in hypertrophic cardiomyopathy: a turning point towards increased morbidity and mortality. Hellenic J Cardiol 58:331–339. https://doi.org/10.1016/j.hjc.2017.01.027
Monserrat L, Elliott PM, Gimeno JR, Sharma S, Penas-Lado M, McKenna WJ (2003) Non-sustained ventricular tachycardia in hypertrophic cardiomyopathy: an independent marker of sudden death risk in young patients. J Am Coll Cardiol 42:873–879. https://doi.org/10.1016/s0735-1097(03)00827-1
Gimeno JR, Tomé-Esteban M, Lofiego C, Hurtado J, Pantazis A, Mist B, Lambiase P, McKenna WJ, Elliott PM (2009) Exercise-induced ventricular arrhythmias and risk of sudden cardiac death in patients with hypertrophic cardiomyopathy. Eur Heart J 30:2599–2605. https://doi.org/10.1093/eurheartj/ehp327
Sedaghat-Hamedani F, Kayvanpour E, Tugrul OF, Lai A, Amr A, Haas J, Proctor T, Ehlermann P, Jensen K, Katus HA, Meder B (2018) Clinical outcomes associated with sarcomere mutations in hypertrophic cardiomyopathy: a meta-analysis on 7675 individuals. Clin Res Cardiol 107:30–41. https://doi.org/10.1007/s00392-017-1155-5
Liebregts M, Vriesendorp PA, Mahmoodi BK, Schinkel AF, Michels M, ten Berg JM (2015) A systematic review and meta-analysis of long-term outcomes after septal reduction therapy in patients with hypertrophic cardiomyopathy. JACC Heart Fail 3:896–905. https://doi.org/10.1016/j.jchf.2015.06.011
Giancaterino S, Urey MA, Darden D, Hsu JC (2020) Management of arrhythmias in cardiac amyloidosis. JACC Clin Electrophysiol 6:351–361. https://doi.org/10.1016/j.jacep.2020.01.004
O’Mahony C, Coats C, Cardona M, Garcia A, Calcagnino M, Murphy E, Lachmann R, Mehta A, Hughes D, Elliott PM (2011) Incidence and predictors of anti-bradycardia pacing in patients with Anderson-Fabry disease. Europace 13:1781–1788. https://doi.org/10.1093/europace/eur267
Gollob MH, Green MS, Tang AS, Roberts R (2002) PRKAG2 cardiac syndrome: familial ventricular preexcitation, conduction system disease, and cardiac hypertrophy. Curr Opin Cardiol 17:229–234. https://doi.org/10.1097/00001573-200205000-00004
Eriksson P, Karp K, Bjerle P, Olofsson BO (1984) Disturbances of cardiac rhythm and conduction in familial amyloidosis with polyneuropathy. Br Heart J 51:658–662. https://doi.org/10.1136/hrt.51.6.658
Efthimiadis GK, Giannakoulas G, Parcharidou DG, Pagourelias ED, Kouidi EJ, Spanos G, Kamperidis V, Gavrielides S, Karvounis H, Styliadis I, Parcharidis GE (2011) Chronotropic incompetence and its relation to exercise intolerance in hypertrophic cardiomyopathy. Int J Cardiol 153:179–184. https://doi.org/10.1016/j.ijcard.2010.08.026
Luo HC, Dimaano VL, Kembro JM, Hilser A, Hurtado-de-Mendoza D, Pozios I, Tomas MS, Yalcin H, Dolores-Cerna K, Mormontoy W, Aon MA, Cameron D, Bluemke DA, Stewart KJ, Russell SD, Cordova JG, Abraham TP, Abraham MR (2015) Exercise heart rates in patients with hypertrophic cardiomyopathy. Am J Cardiol 115:1144–1150. https://doi.org/10.1016/j.amjcard.2015.01.548
Holmqvist F, Platonov PG, Carlson J, Havmöller R, Waktare JE, McKenna WJ, Olsson SB, Meurling CJ (2007) Variable interatrial conduction illustrated in a hypertrophic cardiomyopathy population. Ann Noninvasive Electrocardiol 12:227–236. https://doi.org/10.1111/j.1542-474X.2007.00166.x
Girasis C, Vassilikos V, Efthimiadis GK, Papadopoulou SL, Dakos G, Dalamaga EG, Chouvarda I, Giannakoulas G, Kamperidis V, Paraskevaidis S, Maglaveras N, Karvounis HI, Parcharidis GE, Styliadis IH (2013) Patients with hypertrophic cardiomyopathy at risk for paroxysmal atrial fibrillation: advanced echocardiographic evaluation of the left atrium combined with non-invasive P-wave analysis. Eur Heart J Cardiovasc Imaging 14:425–434. https://doi.org/10.1093/ehjci/jes172
Cecchi F, Montereggi A, Olivotto I, Marconi P, Dolara A, Maron BJ (1997) Risk for atrial fibrillation in patients with hypertrophic cardiomyopathy assessed by signal averaged P wave duration. Heart 78:44–49. https://doi.org/10.1136/hrt.78.1.44
Köse S, Aytemir K, Sade E, Can I, Ozer N, Amasyali B, Aksöyek S, Ovünç K, Ozmen F, Atalar E, Işik E, Kes S, Demirtaş E, Oto A (2003) Detection of patients with hypertrophic cardiomyopathy at risk for paroxysmal atrial fibrillation during sinus rhythm by P-wave dispersion. Clin Cardiol 26:431–434. https://doi.org/10.1002/clc.4960260910
Ozdemir O, Soylu M, Demir AD, Topaloglu S, Alyan O, Turhan H, Bicer A, Kutuk E (2004) P-wave durations as a predictor for atrial fibrillation development in patients with hypertrophic cardiomyopathy. Int J Cardiol 94:163–166. https://doi.org/10.1016/j.ijcard.2003.01.001
Tuluce K, Ozerkan F, Yakar Tuluce S, Yavuzgil O, Gurgun C, Bilgin M, Kahya Eren N, Kocabas U, Nalbantgil S, Soydas Cinar C (2015) Relationships between P wave dispersion, atrial electromechanical delay, left atrial remodeling, and NT-proBNP levels, in patients with hypertrophic cardiomyopathy. Cardiol J 22:94–100. https://doi.org/10.5603/CJ.a2014.0025
Higuchi S, Minami Y, Shoda M, Shirotani S, Saito C, Haruki S, Gotou M, Yagishita D, Ejima K, Hagiwara N (2020) Prognostic implication of first-degree atrioventricular block in patients with hypertrophic cardiomyopathy. J Am Heart Assoc 9:e015064. https://doi.org/10.1161/jaha.119.015064
Patel SI, Ackerman MJ, Shamoun FE, Geske JB, Ommen SR, Love WT, Cha SS, Bos JM, Lester SJ (2019) QT prolongation and sudden cardiac death risk in hypertrophic cardiomyopathy. Acta Cardiol 74:53–58. https://doi.org/10.1080/00015385.2018.1440905
Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RM, Bacchi-Reggiani L, Sangiorgi D, Ferlini A, Cavo M, Zamagni E, Fonte ML, Palladini G, Salinaro F, Musca F, Obici L, Branzi A, Perlini S (2009) Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation 120:1203–1212. https://doi.org/10.1161/circulationaha.108.843334
Ikari Y, Kuwako K, Yamaguchi T (1992) Fabry’s disease with complete atrioventricular block: histological evidence of involvement of the conduction system. Br Heart J 68:323–325. https://doi.org/10.1136/hrt.68.9.323
DiMauro S, Schon EA (2003) Mitochondrial respiratory-chain diseases. N Engl J Med 348:2656–2668. https://doi.org/10.1056/NEJMra022567
Sharma S, Drezner JA, Baggish A, Papadakis M, Wilson MG, Prutkin JM, La Gerche A, Ackerman MJ, Borjesson M, Salerno JC, Asif IM, Owens DS, Chung EH, Emery MS, Froelicher VF, Heidbuchel H, Adamuz C, Asplund CA, Cohen G, Harmon KG, Marek JC, Molossi S, Niebauer J, Pelto HF, Perez MV, Riding NR, Saarel T, Schmied CM, Shipon DM, Stein R, Vetter VL, Pelliccia A, Corrado D (2017) International recommendations for electrocardiographic interpretation in athletes. J Am Coll Cardiol 69:1057–1075. https://doi.org/10.1016/j.jacc.2017.01.015
Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, Ahmad F, Lozado R, Shah G, Fananapazir L, Bachinski LL, Roberts R (2001) Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med 344:1823–1831. https://doi.org/10.1056/nejm200106143442403
Boucek D, Jirikowic J, Taylor M (2011) Natural history of Danon disease. Genet Med 13:563–568. https://doi.org/10.1097/GIM.0b013e31820ad795
Bharati S, Serratto M, DuBrow I, Paul MH, Swiryn S, Miller RA, Rosen K, Lev M (1982) The conduction system in Pompe’s disease. Pediatr Cardiol 2:25–32. https://doi.org/10.1007/bf02265613
López-Sainz Á, Salazar-Mendiguchía J, García-Álvarez A, Campuzano Larrea O, López-Garrido M, García-Guereta L, Fuentes Cañamero ME, Climent Payá V, Peña-Peña ML, Zorio-Grima E, Jordá-Burgos P, Díez-López C, Brugada R, García-Pinilla JM, García-Pavía P (2019) Clinical findings and prognosis of Danon disease. An Analysis of the Spanish Multicenter Danon Registry. Rev Esp Cardiol (Engl Ed) 72:479–486. https://doi.org/10.1016/j.rec.2018.04.035
Konrad T, Sonnenschein S, Schmidt FP, Mollnau H, Bock K, Ocete BQ, Münzel T, Theis C, Rostock T (2017) Cardiac arrhythmias in patients with Danon disease. Europace 19:1204–1210. https://doi.org/10.1093/europace/euw215
Namdar M, Steffel J, Vidovic M, Brunckhorst CB, Holzmeister J, Lüscher TF, Jenni R, Duru F (2011) Electrocardiographic changes in early recognition of Fabry disease. Heart 97:485–490. https://doi.org/10.1136/hrt.2010.211789
Levine JC, Kishnani PS, Chen YT, Herlong JR, Li JS (2008) Cardiac remodeling after enzyme replacement therapy with acid alpha-glucosidase for infants with Pompe disease. Pediatr Cardiol 29:1033–1042. https://doi.org/10.1007/s00246-008-9267-3
Prinz C, Farr M, Hering D, Horstkotte D, Faber L (2010) Reduction in ECG abnormalities and improvement of regional left ventricular function in a patient with Fabry’s disease during enzyme-replacement therapy. Clin Res Cardiol 99:53–55. https://doi.org/10.1007/s00392-009-0084-3
Topilski I, Sherez J, Keren G, Copperman I (2006) Long-term effects of dual-chamber pacing with periodic echocardiographic evaluation of optimal atrioventricular delay in patients with hypertrophic cardiomyopathy >50 years of age. Am J Cardiol 97:1769–1775. https://doi.org/10.1016/j.amjcard.2006.01.040
Montgomery JV, Harris KM, Casey SA, Zenovich AG, Maron BJ (2005) Relation of electrocardiographic patterns to phenotypic expression and clinical outcome in hypertrophic cardiomyopathy. Am J Cardiol 96:270–275. https://doi.org/10.1016/j.amjcard.2005.03.058
Calore C, Zorzi A, Corrado D (2015) Clinical meaning of isolated increase of QRS voltages in hypertrophic cardiomyopathy versus athlete’s heart. J Electrocardiol 48:373–379. https://doi.org/10.1016/j.jelectrocard.2014.12.016
Calore C, Melacini P, Pelliccia A, Cianfrocca C, Schiavon M, Di Paolo FM, Bovolato F, Quattrini FM, Basso C, Thiene G, Iliceto S, Corrado D (2013) Prevalence and clinical meaning of isolated increase of QRS voltages in hypertrophic cardiomyopathy versus athlete’s heart: relevance to athletic screening. Int J Cardiol 168:4494–4497. https://doi.org/10.1016/j.ijcard.2013.06.123
Guerrier K, Anderson JB, Pratt J, King EC, Statile C, Wilmot I, Campbell M, Czosek RJ (2015) Correlation of precordial voltages to left ventricular mass on echocardiogram in adolescent patients with hypertrophic cardiomyopathy compared with that in adolescent athletes. Am J Cardiol 115:956–961. https://doi.org/10.1016/j.amjcard.2015.01.025
Maron BJ, Wolfson JK, Ciró E, Spirito P (1983) Relation of electrocardiographic abnormalities and patterns of left ventricular hypertrophy identified by 2-dimensional echocardiography in patients with hypertrophic cardiomyopathy. Am J Cardiol 51:189–194. https://doi.org/10.1016/s0002-9149(83)80034-4
Chatterjee D, Narayan P (2018) Idiopathic isolated right ventricular apical hypertrophy. Acta Cardiol Sin 34:288–290. https://doi.org/10.6515/acs.201805_34(3).20180122a
Pagourelias ED, Efthimiadis GK, Parcharidou DG, Gossios TD, Kamperidis V, Karoulas T, Karvounis H, Styliadis IH (2011) Prognostic value of right ventricular diastolic function indices in hypertrophic cardiomyopathy. Eur J Echocardiogr 12:809–817. https://doi.org/10.1093/ejechocard/jer126
Maron BJ, Roberts WC, Arad M, Haas TS, Spirito P, Wright GB, Almquist AK, Baffa JM, Saul JP, Ho CY, Seidman J, Seidman CE (2009) Clinical outcome and phenotypic expression in LAMP2 cardiomyopathy. JAMA 301:1253–1259. https://doi.org/10.1001/jama.2009.371
Arad M, Maron BJ, Gorham JM, Johnson WH Jr, Saul JP, Perez-Atayde AR, Spirito P, Wright GB, Kanter RJ, Seidman CE, Seidman JG (2005) Glycogen storage diseases presenting as hypertrophic cardiomyopathy. N Engl J Med 352:362–372. https://doi.org/10.1056/NEJMoa033349
Cyrille NB, Goldsmith J, Alvarez J, Maurer MS (2014) Prevalence and prognostic significance of low QRS voltage among the three main types of cardiac amyloidosis. Am J Cardiol 114:1089–1093. https://doi.org/10.1016/j.amjcard.2014.07.026
Konno T, Nagata Y, Teramoto R, Fujino N, Nomura A, Tada H, Sakata K, Furusho H, Takamura M, Nakamura H, Kawashiri MA, Yamagishi M, Hayashi K (2016) Usefulness of electrocardiographic voltage to determine myocardial fibrosis in hypertrophic cardiomyopathy. Am J Cardiol 117:443–449. https://doi.org/10.1016/j.amjcard.2015.11.015
Biagini E, Pazzi C, Olivotto I, Musumeci B, Limongelli G, Boriani G, Pacileo G, Mastromarino V, Bacchi Reggiani ML, Lorenzini M, Lai F, Berardini A, Mingardi F, Rosmini S, Resciniti E, Borghi C, Autore C, Cecchi F, Rapezzi C (2016) Usefulness of electrocardiographic patterns at presentation to predict long-term risk of cardiac death in patients with hypertrophic cardiomyopathy. Am J Cardiol 118:432–439. https://doi.org/10.1016/j.amjcard.2016.05.023
Koga Y, Yamaga A, Hiyamuta K, Ikeda H, Toshima H (2004) Mechanisms of abnormal Q waves in hypertrophic cardiomyopathy assessed by intracoronary electrocardiography. J Cardiovasc Electrophysiol 15:1402–1408. https://doi.org/10.1046/j.1540-8167.2004.04314.x
Maron BJ, Epstein SE, Roberts WC (1979) Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. Am J Cardiol 43:1086–1102. https://doi.org/10.1016/0002-9149(79)90139-5
Fronza M, Raineri C, Valentini A, Bassi EM, Scelsi L, Buscemi ML, Turco A, Castelli G, Ghio S, Visconti LO (2016) Relationship between electrocardiographic findings and Cardiac Magnetic Resonance phenotypes in patients with Hypertrophic Cardiomyopathy. Int J Cardiol Heart Vasc 11:7–11. https://doi.org/10.1016/j.ijcha.2016.02.001
Dumont CA, Monserrat L, Soler R, Rodríguez E, Fernandez X, Peteiro J, Bouzas A, Bouzas B, Castro-Beiras A (2006) Interpretation of electrocardiographic abnormalities in hypertrophic cardiomyopathy with cardiac magnetic resonance. Eur Heart J 27:1725–1731. https://doi.org/10.1093/eurheartj/ehl101
Lemery R, Kleinebenne A, Nihoyannopoulos P, Aber V, Alfonso F, McKenna WJ (1990) Q waves in hypertrophic cardiomyopathy in relation to the distribution and severity of right and left ventricular hypertrophy. J Am Coll Cardiol 16:368–374. https://doi.org/10.1016/0735-1097(90)90587-f
Ohmoto-Sekine Y, Suzuki J, Shimamoto R, Yamazaki T, Tsuji T, Nagai R, Ohtomo K (2007) Gender-specific clinical characteristics of deep Q waves in hypertrophic cardiomyopathy. Gend Med 4:274–283. https://doi.org/10.1016/s1550-8579(07)80046-5
Konno T, Shimizu M, Ino H, Yamaguchi M, Terai H, Uchiyama K, Oe K, Mabuchi T, Kaneda T, Mabuchi H (2004) Diagnostic value of abnormal Q waves for identification of preclinical carriers of hypertrophic cardiomyopathy based on a molecular genetic diagnosis. Eur Heart J 25:246–251. https://doi.org/10.1016/j.ehj.2003.10.031
Savage DD, Seides SF, Clark CE, Henry WL, Maron BJ, Robinson FC, Epstein SE (1978) Electrocardiographic findings in patients with obstructive and nonobstructive hypertrophic cardiomyopathy. Circulation 58:402–408. https://doi.org/10.1161/01.cir.58.3.402
Nasermoaddeli A, Miura K, Matsumori A, Soyama Y, Morikawa Y, Kitabatake A, Inaba Y, Nakagawa H (2007) Prognosis and prognostic factors in patients with hypertrophic cardiomyopathy in Japan: results from a nationwide study. Heart 93:711–715. https://doi.org/10.1136/hrt.2006.095232
Killu AM, Park JY, Sara JD, Hodge DO, Gersh BJ, Nishimura RA, Asirvatham SJ, McLeod CJ (2018) Cardiac resynchronization therapy in patients with end-stage hypertrophic cardiomyopathy. Europace 20:82–88. https://doi.org/10.1093/europace/euw327
Rogers DP, Marazia S, Chow AW, Lambiase PD, Lowe MD, Frenneaux M, McKenna WJ, Elliott PM (2008) Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy. Eur J Heart Fail 10:507–513. https://doi.org/10.1016/j.ejheart.2008.03.006
Patel V, O’Mahony C, Hughes D, Rahman MS, Coats C, Murphy E, Lachmann R, Mehta A, Elliott PM (2015) Clinical and genetic predictors of major cardiac events in patients with Anderson-Fabry disease. Heart 101:961–966. https://doi.org/10.1136/heartjnl-2014-306782
Raaijmakers R, Noordam C, Noonan JA, Croonen EA, van der Burgt CJ, Draaisma JM (2008) Are ECG abnormalities in Noonan syndrome characteristic for the syndrome? Eur J Pediatr 167:1363–1367. https://doi.org/10.1007/s00431-008-0670-9
Debonnaire P, Katsanos S, Joyce E, OV VDB, Atsma DE, Schalij MJ, Bax JJ, Delgado V, Marsan NA, (2015) QRS fragmentation and QTc duration relate to malignant ventricular tachyarrhythmias and sudden cardiac death in patients with hypertrophic cardiomyopathy. J Cardiovasc Electrophysiol 26:547–555. https://doi.org/10.1111/jce.12629
Femenía F, Arce M, Van Grieken J, Trucco E, Mont L, Abello M, Merino JL, Rivero-Ayerza M, Gorenek B, Rodriguez C, Hopman WM, Baranchuk A (2013) Fragmented QRS as a predictor of arrhythmic events in patients with hypertrophic obstructive cardiomyopathy. J Interv Card Electrophysiol 38:159–165. https://doi.org/10.1007/s10840-013-9829-z
Kang KW, Janardhan AH, Jung KT, Lee HS, Lee MH, Hwang HJ (2014) Fragmented QRS as a candidate marker for high-risk assessment in hypertrophic cardiomyopathy. Heart Rhythm 11:1433–1440. https://doi.org/10.1016/j.hrthm.2014.05.002
Lu X, Wang W, Zhu L, Wang Y, Sun K, Zou Y, Tian T, Hui R, Wang J, Kang L, Song L (2017) Prognostic significance of fragmented QRS in patients with hypertrophic cardiomyopathy. Cardiology 138:26–33. https://doi.org/10.1159/000471845
Nomura A, Konno T, Fujita T, Tanaka Y, Nagata Y, Tsuda T, Hodatsu A, Sakata K, Nakamura H, Kawashiri MA, Fujino N, Yamagishi M, Hayashi K (2015) Fragmented QRS predicts heart failure progression in patients with hypertrophic cardiomyopathy. Circ J 79:136–143. https://doi.org/10.1253/circj.CJ-14-0822
Rattanawong P, Riangwiwat T, Kanitsoraphan C, Chongsathidkiet P, Kanjanahattakij N, Vutthikraivit W, Chung EH (2018) Baseline fragmented QRS increases the risk of major arrhythmic events in hypertrophic cardiomyopathy: systematic review and meta-analysis. Ann Noninvasive Electrocardiol 23:e12533. https://doi.org/10.1111/anec.12533
Haukilahti MA, Eranti A, Kenttä T, Huikuri HV (2016) QRS fragmentation patterns representing myocardial scar need to be separated from benign normal variants: hypotheses and proposal for morphology based classification. Front Physiol 7:653. https://doi.org/10.3389/fphys.2016.00653
Wilson MG, Sharma S, Carré F, Charron P, Richard P, O’Hanlon R, Prasad SK, Heidbuchel H, Brugada J, Salah O, Sheppard M, George KP, Whyte G, Hamilton B, Chalabi H (2012) Significance of deep T-wave inversions in asymptomatic athletes with normal cardiovascular examinations: practical solutions for managing the diagnostic conundrum. Br J Sports Med 46(Suppl 1):i51-58. https://doi.org/10.1136/bjsports-2011-090838
Konno T, Fujino N, Hayashi K, Uchiyama K, Masuta E, Katoh H, Sakamoto Y, Tsubokawa T, Ino H, Yamagishi M (2007) Differences in the diagnostic value of various criteria of negative T waves for hypertrophic cardiomyopathy based on a molecular genetic diagnosis. Clin Sci (Lond) 112:577–582. https://doi.org/10.1042/cs20060293
Lakdawala NK, Thune JJ, Maron BJ, Cirino AL, Havndrup O, Bundgaard H, Christiansen M, Carlsen CM, Dorval JF, Kwong RY, Colan SD, Køber LV, Ho CY (2011) Electrocardiographic features of sarcomere mutation carriers with and without clinically overt hypertrophic cardiomyopathy. Am J Cardiol 108:1606–1613. https://doi.org/10.1016/j.amjcard.2011.07.019
Sharma S, Drezner JA, Baggish A, Papadakis M, Wilson MG, Prutkin JM, La Gerche A, Ackerman MJ, Borjesson M, Salerno JC, Asif IM, Owens DS, Chung EH, Emery MS, Froelicher VF, Heidbuchel H, Adamuz C, Asplund CA, Cohen G, Harmon KG, Marek JC, Molossi S, Niebauer J, Pelto HF, Perez MV, Riding NR, Saarel T, Schmied CM, Shipon DM, Stein R, Vetter VL, Pelliccia A, Corrado D (2018) International recommendations for electrocardiographic interpretation in athletes. Eur Heart J 39:1466–1480. https://doi.org/10.1093/eurheartj/ehw631
Calore C, Zorzi A, Sheikh N, Nese A, Facci M, Malhotra A, Zaidi A, Schiavon M, Pelliccia A, Sharma S, Corrado D (2016) Electrocardiographic anterior T-wave inversion in athletes of different ethnicities: differential diagnosis between athlete’s heart and cardiomyopathy. Eur Heart J 37:2515–2527. https://doi.org/10.1093/eurheartj/ehv591
Flett AS, Maestrini V, Milliken D, Fontana M, Treibel TA, Harb R, Sado DM, Quarta G, Herrey A, Sneddon J, Elliott P, McKenna W, Moon JC (2015) Diagnosis of apical hypertrophic cardiomyopathy: T-wave inversion and relative but not absolute apical left ventricular hypertrophy. Int J Cardiol 183:143–148. https://doi.org/10.1016/j.ijcard.2015.01.054
Pennacchini E, Musumeci MB, Conte MR, Stöllberger C, Formisano F, Bongioanni S, Francia P, Volpe M, Autore C (2015) Electrocardiographic evolution in patients with hypertrophic cardiomyopathy who develop a left ventricular apical aneurysm. J Electrocardiol 48:818–825. https://doi.org/10.1016/j.jelectrocard.2015.06.004
Suwa K, Satoh H, Sano M, Nobuhara M, Saitoh T, Saotome M, Urushida T, Katoh H, Tawarahara K, Ohtani H, Wakabayashi Y, Takase H, Terada H, Takehara Y, Sakahara H, Hayashi H (2014) Functional, morphological and electrocardiographical abnormalities in patients with apical hypertrophic cardiomyopathy and apical aneurysm: correlation with cardiac MR. Open Heart 1:e000124. https://doi.org/10.1136/openhrt-2014-000124
Tangwiwat C, Kaolawanich Y, Krittayaphong R (2019) Electrocardiographic predictors of myocardial fibrosis and apical hypertrophic cardiomyopathy. Ann Noninvasive Electrocardiol 24:e12612. https://doi.org/10.1111/anec.12612
Lambiase PD, Gold MR, Hood M, Boersma L, Theuns D, Burke MC, Weiss R, Russo AM, Kääb S, Knight BP (2016) Evaluation of subcutaneous ICD early performance in hypertrophic cardiomyopathy from the pooled EFFORTLESS and IDE cohorts. Heart Rhythm 13:1066–1074. https://doi.org/10.1016/j.hrthm.2016.01.001
Elliott PM, Kaski JC, Prasad K, Seo H, Slade AK, Goldman JH, McKenna WJ (1996) Chest pain during daily life in patients with hypertrophic cardiomyopathy: an ambulatory electrocardiographic study. Eur Heart J 17:1056–1064. https://doi.org/10.1093/oxfordjournals.eurheartj.a015002
Elliott PM, Rosano GM, Gill JS, Poole-Wilson PA, Kaski JC, McKenna WJ (1996) Changes in coronary sinus pH during dipyridamole stress in patients with hypertrophic cardiomyopathy. Heart 75:179–183. https://doi.org/10.1136/hrt.75.2.179
Lazzeroni E, Picano E, Morozzi L, Maurizio AR, Palma G, Ceriati R, Iori E, Barilli A (1997) Dipyridamole-induced ischemia as a prognostic marker of future adverse cardiac events in adult patients with hypertrophic cardiomyopathy. Echo Persantine Italian Cooperative (EPIC) Study Group. Subproject Hypertrophic Cardiomyopathy Circulation 96:4268–4272. https://doi.org/10.1161/01.cir.96.12.4268
Ogata Y, Hiyamuta K, Terasawa M, Ohkita Y, Bekki H, Koga Y, Toshima H (1986) Relationship of exercise or pacing induced ST segment depression and myocardial lactate metabolism in patients with hypertrophic cardiomyopathy. Jpn Heart J 27:145–158. https://doi.org/10.1536/ihj.27.145
Aquino NJ, Centurión OA, Torales JM, Miño LM, Scavenius KE, Alderete JF, Candia JC, García LB, Cáceres C, Martínez JE, Lovera OA (2019) Association of QRS complex fragmentation with QT interval prolongation in patients with ischemic heart disease. Curr Health Sci J 45:398–404. https://doi.org/10.12865/chsj.45.04.08
Jouven X, Hagege A, Charron P, Carrier L, Dubourg O, Langlard JM, Aliaga S, Bouhour JB, Schwartz K, Desnos M, Komajda M (2002) Relation between QT duration and maximal wall thickness in familial hypertrophic cardiomyopathy. Heart 88:153–157. https://doi.org/10.1136/heart.88.2.153
Johnson JN, Grifoni C, Bos JM, Saber-Ayad M, Ommen SR, Nistri S, Cecchi F, Olivotto I, Ackerman MJ (2011) Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy. Eur Heart J 32:1114–1120. https://doi.org/10.1093/eurheartj/ehr021
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TG and GKE attributed with initial manuscript conception. TG, KS, TZ, and DN performed literature search and data analysis. TG and KS drafted the manuscript. PR, DP, GKE, and HK critically appraised and approved the final manuscript.
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Gossios, T., Savvatis, K., Zegkos, T. et al. Deciphering hypertrophic cardiomyopathy with electrocardiography. Heart Fail Rev 27, 1313–1323 (2022). https://doi.org/10.1007/s10741-021-10147-0
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DOI: https://doi.org/10.1007/s10741-021-10147-0