© 2001 by European Society of Cardiology
Copyright © 2001, European Society of Cardiology
Sudden cardiac death in young people with apparently normal heart
Departments of Cardiology and Cardiovascular Pathology, University of Padua Medical School, Padua, Italy
* Corresponding author. Present address: Department of Cardiology, University of Padua Medical School, Via N. Giustiniani 2, 35121 Padua, Italy. Tel.: +39-049-827-2286; fax: +39-049-827-2284 cardpath{at}ux.l.unipd.it
Objective: The aim of the present study was to assess the prevalence of subtle morphologic substrates, clinically unrecognizable, underlying sudden cardiac death (SCD) in young people with apparently normal heart. Methods: In the time interval 1979–1998, 273 consecutive cases of SCD in young people (
35 years) which occurred in the Veneto Region of Italy were prospectively studied. Following exclusion of extracardiac causes of sudden death, the heart was examined according to a detailed morphologic protocol consisting of macroscopic and histologic examination, including study of the specialized conduction system by serial sections. Results: At macroscopic examination, 197 SCD victims (72%) had an overt underlying structural heart disease such as cardiomyopathy in 56, obstructive coronary atherosclerosis in 54, valve disease in 32, non-atherosclerotic coronary artery disease in 28, aortic rupture in 13, postoperative congenital heart disease in five, and other disease in nine. The remaining 76 cases (28%) (50 males and 26 females, aged 4–35 years, mean 23±5 years) had a macroscopically normal heart. A total of 28 of them (37%) had experienced one or more of the following prodroma: syncope, palpitations or both in 20, ECG abnormalities in 18 and arrhythmias in ten. In 79% of them, histologic examination disclosed concealed pathologic substrates consisting of focal myocarditis in 27 cases, regional arrhythmogenic right ventricular cardiomyopathy, mostly localized to RV outflow tract, in nine, and conduction system abnormalities in 24 (leading to ventricular preexcitation in 18 and heart block in six). In 16 hearts (6%) there was no evidence of structural heart disease even after histologic study. Conclusion: Macroscopic heart features were normal in nearly one-third of young SCD victims. In 79% of them, however, histologic study unmasked concealed pathologic substrates such as focal myocarditis or cardiomyopathy and conduction system diseases. A total of 16 victims (6%) had no evidence of structural heart disease and the mechanism of their SCD remained unexplained.
KEYWORDS Arrhythmia (mechanisms); Cardiomyopathy; Conduction system; Histo(patho)logy; Myocarditis; Sudden death
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  I. Ostman-Smith, A. Wisten, E. Nylander, E.-L. Bratt, A. de-Wahl Granelli, A. Oulhaj, and E. Ljungstrom Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy Eur. Heart J., November 5, 2009; (2009) ehp443v1. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. J. Klein, L. J. Gula, A. D. Krahn, A. C. Skanes, and R. Yee WPW Pattern in the Asymptomatic Individual: Has Anything Changed? Circ Arrhythm Electrophysiol, April 1, 2009; 2(2): 97 - 99. [Full Text] [PDF]
|
|
|
|
 |
|
 M. Pieroni, A. Dello Russo, F. Marzo, G. Pelargonio, M. Casella, F. Bellocci, and F. Crea High prevalence of myocarditis mimicking arrhythmogenic right ventricular cardiomyopathy differential diagnosis by electroanatomic mapping-guided endomyocardial biopsy. J. Am. Coll. Cardiol., February 24, 2009; 53(8): 681 - 689. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Haissaguerre, F. Sacher, A. Nogami, N. Komiya, A. Bernard, V. Probst, S. Yli-Mayry, P. Defaye, Y. Aizawa, R. Frank, et al. Characteristics of recurrent ventricular fibrillation associated with inferolateral early repolarization role of drug therapy. J. Am. Coll. Cardiol., February 17, 2009; 53(7): 612 - 619. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Zumhagen, T. Spieker, J. Rolinck, H. A. Baba, G. Breithardt, W. Bocker, L. Eckardt, M. Paul, T. Wichter, and E. Schulze-Bahr Absence of Pathognomonic or Inflammatory Patterns in Cardiac Biopsies From Patients With Brugada Syndrome Circ Arrhythm Electrophysiol, February 1, 2009; 2(1): 16 - 23. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Corrado, C. Basso, A. Pelliccia, and G. Thiene CHAPTER 32 Sports and Heart Disease ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Pieroni, F. Bellocci, F. Crea, I. Nault, M. Wright, and M. Haissaguerre Sudden Cardiac Arrest Associated with Early Repolarization N. Engl. J. Med., August 14, 2008; 359(7): 761 - 762. [Full Text] [PDF]
|
|
|
|
|
|
M. Haissaguerre, N. Derval, F. Sacher, L. Jesel, I. Deisenhofer, L. de Roy, J.-L. Pasquie, A. Nogami, D. Babuty, S. Yli-Mayry, et al. Sudden Cardiac Arrest Associated with Early Repolarization N. Engl. J. Med., May 8, 2008; 358(19): 2016 - 2023. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. Ostman-Smith, G. Wettrell, B. Keeton, D. Holmgren, U. Ergander, S. Gould, C. Bowker, and M. Verdicchio Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy Eur. Heart J., May 1, 2008; 29(9): 1160 - 1167. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Basavarajaiah, M. Wilson, G. Whyte, A. Shah, E. Behr, and S. Sharma Prevalence and significance of an isolated long QT interval in elite athletes Eur. Heart J., December 1, 2007; 28(23): 2944 - 2949. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. W. Van Norstrand, C. R. Valdivia, D. J. Tester, K. Ueda, B. London, J. C. Makielski, and M. J. Ackerman Molecular and Functional Characterization of Novel Glycerol-3-Phosphate Dehydrogenase 1-Like Gene (GPD1-L) Mutations in Sudden Infant Death Syndrome Circulation, November 13, 2007; 116(20): 2253 - 2259. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Struthers and C. Lang The potential to improve primary prevention in the future by using BNP/N-BNP as an indicator of silent 'pancardiac' target organ damage: BNP/N-BNP could become for the heart what microalbuminuria is for the kidney Eur. Heart J., July 2, 2007; 28(14): 1678 - 1682. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 B. A Koplan and W. G Stevenson Sudden arrhythmic death syndrome Heart, May 1, 2007; 93(5): 547 - 548. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 F. De Giorgio, A. Abbate, G. Vetrugno, A. Capelli, and V. Arena Non-atherosclerotic coronary pathology causing sudden death J. Clin. Pathol., January 1, 2007; 60(1): 94 - 97. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Corrado, C. Basso, A. Pavei, P. Michieli, M. Schiavon, and G. Thiene Trends in Sudden Cardiovascular Death in Young Competitive Athletes After Implementation of a Preparticipation Screening Program JAMA, October 4, 2006; 296(13): 1593 - 1601. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A Fabre and M N Sheppard Sudden adult death syndrome and other non-ischaemic causes of sudden cardiac death Heart, March 1, 2006; 92(3): 316 - 320. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. G. Meregalli, A. A.M. Wilde, and H. L. Tan Pathophysiological mechanisms of Brugada syndrome: Depolarization disorder, repolarization disorder, or more? Cardiovasc Res, August 15, 2005; 67(3): 367 - 378. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
S. Nattel and J.-J. Schott Arrhythmogenic right ventricular dysplasia type 1 and mutations in transforming growth factor {beta}3 gene regulatory regions: a breakthrough? Cardiovasc Res, February 1, 2005; 65(2): 302 - 304. [Full Text] [PDF]
|
|
|
|
 |
|
 H. Amital, M. Glikson, M. Burstein, A. Afek, R. Sinnreich, Y. Weiss, and V. Israeli Clinical Characteristics of Unexpected Death Among Young Enlisted Military Personnel: Results of a Three-Decade Retrospective Surveillance Chest, August 1, 2004; 126(2): 528 - 533. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Tabib, R. Loire, L. Chalabreysse, D. Meyronnet, A. Miras, D. Malicier, F. Thivolet, P. Chevalier, and P. Bouvagnet Circumstances of Death and Gross and Microscopic Observations in a Series of 200 Cases of Sudden Death Associated With Arrhythmogenic Right Ventricular Cardiomyopathy and/or Dysplasia Circulation, December 16, 2003; 108(24): 3000 - 3005. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. M Spooner, S. G Priori, and R. J Myerburg Spotlight on sudden cardiac death Cardiovasc Res, May 1, 2001; 50(2): 173 - 176. [Full Text] [PDF]
|
|