Sudden cardiac arrest in the absence of apparent structural heart disease
- Mark S Link, MD
Mark S Link, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Tufts Medical School
- Section Editors
- Peter J Zimetbaum, MD
Peter J Zimetbaum, MD
- Section Editor — Cardiac Arrhythmias
- Professor of Medicine
- Harvard Medical School
- Scott Manaker, MD, PhD
Scott Manaker, MD, PhD
- Section Editor — Critical Care
- Professor of Medicine
- University of Pennsylvania School of Medicine
Sudden cardiac arrest (SCA) and sudden cardiac death (SCD) refer to the sudden cessation of cardiac activity with hemodynamic collapse due to sustained pulseless ventricular tachycardia/fibrillation, pulseless electrical activity (PEA), or asystole. The event is referred to as SCA (or aborted SCD) if an intervention (eg, defibrillation) restores circulation. The event is called SCD if the patient dies. However, the use of SCD to describe both fatal and nonfatal cardiac arrest persists by convention. (See "Overview of sudden cardiac arrest and sudden cardiac death", section on 'Definitions'.)
SCA and SCD occur most commonly in patients with structural heart disease (including previously undiagnosed heart disease), particularly coronary artery disease. SCD in the apparently normal heart (at autopsy) is less common and is responsible for 10 to 15 percent of cases of SCD [1,2]. SCA with an apparently normal heart is more common in younger arrest victims. The majority of SCD patients without apparent structural heart disease likely do not actually have "normal" hearts, but our diagnostic tools limit identification of structural or functional abnormalities. In the past, the etiology of many of these deaths was unknown and deemed "idiopathic." However, more complete evaluation has identified the cause of death as a primary electrical disorder (ie, long QT, Wolff-Parkinson-White [WPW], catecholaminergic polymorphic ventricular tachycardia [CPVT], and Brugada syndrome) in many of these patients [1-3]. (See "Pathophysiology and etiology of sudden cardiac arrest".)
SCD in the apparently normal heart will be reviewed here. SCD in patients with heart disease, and the evaluation and options for the management of survivors of SCD, are discussed separately. (See "Overview of sudden cardiac arrest and sudden cardiac death", section on 'Etiology' and "Pathophysiology and etiology of sudden cardiac arrest", section on 'Etiology of SCD' and "Incidence of and risk stratification for sudden cardiac death after acute myocardial infarction" and "Hypertrophic cardiomyopathy: Assessment and management of ventricular arrhythmias and sudden cardiac death risk".)
In addition, sudden death may occur from noncardiac causes (eg, trauma, pulmonary embolism seizure), and these topics are discussed separately. (See "Overview of acute pulmonary embolism in adults" and "Sudden unexpected death in epilepsy".)
Based upon a review of death certificates in the United States during 1998 and 1999, sudden cardiac death (SCD) accounted for over 450,000 deaths, which represented 63 percent of cardiac deaths among adults ≥35 years of age . The incidence of SCD is increased six- to ten-fold in the presence of clinically recognized heart disease (figure 1); it also increases with age and is two to three times more common in men than women (figure 2) .
- Chugh SS, Kelly KL, Titus JL. Sudden cardiac death with apparently normal heart. Circulation 2000; 102:649.
- Wever EF, Robles de Medina EO. Sudden death in patients without structural heart disease. J Am Coll Cardiol 2004; 43:1137.
- Myerburg RJ. Sudden cardiac death in persons with normal (or near normal) hearts. Am J Cardiol 1997; 79:3.
- Zheng ZJ, Croft JB, Giles WH, Mensah GA. Sudden cardiac death in the United States, 1989 to 1998. Circulation 2001; 104:2158.
- Kannel WB, Wilson PW, D'Agostino RB, Cobb J. Sudden coronary death in women. Am Heart J 1998; 136:205.
- Stecker EC, Vickers C, Waltz J, et al. Population-based analysis of sudden cardiac death with and without left ventricular systolic dysfunction: two-year findings from the Oregon Sudden Unexpected Death Study. J Am Coll Cardiol 2006; 47:1161.
- Drory Y, Turetz Y, Hiss Y, et al. Sudden unexpected death in persons less than 40 years of age. Am J Cardiol 1991; 68:1388.
- Bowker TJ, Wood DA, Davies MJ, et al. Sudden, unexpected cardiac or unexplained death in England: a national survey. QJM 2003; 96:269.
- Eckart RE, Shry EA, Burke AP, et al. Sudden death in young adults: an autopsy-based series of a population undergoing active surveillance. J Am Coll Cardiol 2011; 58:1254.
- Mellor G, Raju H, de Noronha SV, et al. Clinical characteristics and circumstances of death in the sudden arrhythmic death syndrome. Circ Arrhythm Electrophysiol 2014; 7:1078.
- Chugh SS, Socoteanu C, Reinier K, et al. A community-based evaluation of sudden death associated with therapeutic levels of methadone. Am J Med 2008; 121:66.
- Tester DJ, Spoon DB, Valdivia HH, et al. Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner's cases. Mayo Clin Proc 2004; 79:1380.
- Wang YS, Scheinman MM, Chien WW, et al. Patients with supraventricular tachycardia presenting with aborted sudden death: incidence, mechanism and long-term follow-up. J Am Coll Cardiol 1991; 18:1711.
- Basso C, Corrado D, Rossi L, Thiene G. Ventricular preexcitation in children and young adults: atrial myocarditis as a possible trigger of sudden death. Circulation 2001; 103:269.
- Pappone C, Santinelli V, Rosanio S, et al. Usefulness of invasive electrophysiologic testing to stratify the risk of arrhythmic events in asymptomatic patients with Wolff-Parkinson-White pattern: results from a large prospective long-term follow-up study. J Am Coll Cardiol 2003; 41:239.
- Klein GJ, Bashore TM, Sellers TD, et al. Ventricular fibrillation in the Wolff-Parkinson-White syndrome. N Engl J Med 1979; 301:1080.
- Montoya PT, Brugada P, Smeets J, et al. Ventricular fibrillation in the Wolff-Parkinson-White syndrome. Eur Heart J 1991; 12:144.
- Haïssaguerre M, Derval N, Sacher F, et al. Sudden cardiac arrest associated with early repolarization. N Engl J Med 2008; 358:2016.
- Noseworthy PA, Tikkanen JT, Porthan K, et al. The early repolarization pattern in the general population: clinical correlates and heritability. J Am Coll Cardiol 2011; 57:2284.
- Quattrini FM, Pelliccia A, Assorgi R, et al. Benign clinical significance of J-wave pattern (early repolarization) in highly trained athletes. Heart Rhythm 2014; 11:1974.
- Patton KK, Ellinor PT, Ezekowitz M, et al. Electrocardiographic Early Repolarization: A Scientific Statement From the American Heart Association. Circulation 2016; 133:1520.
- Knecht S, Sacher F, Wright M, et al. Long-term follow-up of idiopathic ventricular fibrillation ablation: a multicenter study. J Am Coll Cardiol 2009; 54:522.
- Santoro F, Di Biase L, Hranitzky P, et al. Ventricular fibrillation triggered by PVCs from papillary muscles: clinical features and ablation. J Cardiovasc Electrophysiol 2014; 25:1158.
- Survivors of out-of-hospital cardiac arrest with apparently normal heart. Need for definition and standardized clinical evaluation. Consensus Statement of the Joint Steering Committees of the Unexplained Cardiac Arrest Registry of Europe and of the Idiopathic Ventricular Fibrillation Registry of the United States. Circulation 1997; 95:265.
- Viskin S, Belhassen B. Idiopathic ventricular fibrillation. Am Heart J 1990; 120:661.
- Ozaydin M, Moazzami K, Kalantarian S, et al. Long-Term Outcome of Patients With Idiopathic Ventricular Fibrillation: A Meta-Analysis. J Cardiovasc Electrophysiol 2015; 26:1095.
- Friedlander Y, Siscovick DS, Weinmann S, et al. Family history as a risk factor for primary cardiac arrest. Circulation 1998; 97:155.
- Jouven X, Desnos M, Guerot C, Ducimetière P. Predicting sudden death in the population: the Paris Prospective Study I. Circulation 1999; 99:1978.
- Behr E, Wood DA, Wright M, et al. Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome. Lancet 2003; 362:1457.
- Wei D, Tao L, Huang M. Genetic variations involved in sudden cardiac death and their associations and interactions. Heart Fail Rev 2016; 21:401.
- Tester DJ, Ackerman MJ. Postmortem long QT syndrome genetic testing for sudden unexplained death in the young. J Am Coll Cardiol 2007; 49:240.
- Tester DJ, Medeiros-Domingo A, Will ML, et al. Cardiac channel molecular autopsy: insights from 173 consecutive cases of autopsy-negative sudden unexplained death referred for postmortem genetic testing. Mayo Clin Proc 2012; 87:524.
- POTENTIAL CAUSES
- Prolonged QT interval
- Polymorphic VT with normal QT interval
- Brugada syndrome
- Commotio cordis
- WPW and other forms of SVT
- Short QT syndrome
- Early repolarization syndrome
- VF secondary to PVCs
- Idiopathic VF
- Familial SCD
- AUTOPSY AND MOLECULAR GENETIC TESTING
- EVALUATION OF SURVIVORS OF SCD
- EVALUATION OF FAMILY MEMBERS
- SUMMARY AND RECOMMENDATIONS