Review Article
Volume 4 Issue 4 - 2020
Sudden Cardiac Arrest in a Normal Heart- Approach to Evaluation
Surjya Prasad Upadhyay*
Specialist Anaesthesiology, NMC Royal Hospital DIP, Dubai investment Park, Dubai, UAE
*Corresponding Author: Surjya Prasad Upadhyay, Specialist Anaesthesiology, NMC Royal Hospital DIP, Dubai investment Park, Dubai, UAE.
Received: December 02, 2019; Published: March 30, 2020


The true incidence of cardiac arrest in apparently normal heart is difficult to estimate. Historically 10 - 15% of all cardiac arrest are in apparently normal heart whereas, up to 40% cardiac arrest in young are in apparently normal hearts. Majority of the cardiac arrest in normal heart is due to recurrent ventricular arrhythmia caused by abnormalities in myocardial depolarization and repolarisation, usually due to inherited channelopathy, metabolic or drug induced. Any survivor of sudden cardiac arrest require a comprehensive clinical and in depth sequential testing such as resting and exercise ECG, echocardiography, angiography and various provocative test and genetic testing if needed. General and cardiological evaluation of family member of victim of sudden unexplained cardiac arrest may yield the presence of heritable condition in up to 40% of family members. All first and second degree relatives of sudden cardiac arrest victim in the absence of any overt heart disease should be informed regarding the potentially increased risk of cardiac events and counselled for assessment at a center with experience in the diagnosis and management of inherited cardiac conditions and genetic testing should be undertaken whenever any identifiable condition exist. This review focus on the common conditions leading to cardiac arrest in an individual with apparent normal heart and approach to evaluation and diagnosis of the victim and their close family members.

Keywords: Cardiac Arrest; Normal Heart


  1. Chugh SS., et al. “Sudden cardiac death with apparently normal heart”. Circulation 102.6 (2000): 649.
  2. Wever EF and Robles de Medina EO. “Sudden death in patients without structural heart disease”. Journal of the American College of Cardiology 43.7 (2004): 1137. 
  3. Bagnall RD., et al. “A Prospective Study of Sudden Cardiac Death among Children and Young Adults”. The New England Journal of Medicine 374 (2016): 2441-2452.
  4. Modi S and Krahn AD. “Sudden Cardiac Arrest without Overt heart disease”. Circulation 123 (2011): 2994-3008.
  5. Krahn AD., et al. “Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)”. Circulation 120 (2009): 278-285.
  6. Estes NA. “Sudden cardiac arrest from primary electrical diseases: provoking concealed arrhythmogenic syndromes”. Circulation 112 (2005): 2220-2221.
  7. Krahn AD., et al. “Diagnosis of unexplained cardiac arrest: role of adrenaline and procainamide infusion”. Circulation 112 (2005): 2228-2234.
  8. Tester DJ., 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 Clinic Proceedings 79.11 (2004): 1380. 
  9. Tester DJ and Ackerman MJ. “Postmortem long QT syndrome genetic testing for sudden unexplained death in the young”. Journal of the American College of Cardiology 49.2 (2007): 240.
  10. Tester DJ., et al. “Cardiac channel molecular autopsy: insights from 173 consecutive cases of autopsy-negative sudden unexplained death referred for postmortem genetic testing”. Mayo Clinic Proceedings 87.6 (2012): 524.
  11. Lahrouchi N., et al. “Utility of Post-Mortem Genetic Testing in Cases of Sudden Arrhythmic Death Syndrome”. Journal of the American College of Cardiology 69.17 (2017): 2134.
  12. Ranthe MF., et al. “Risk of cardiovascular disease in family members of young sudden cardiac death victims”. European Heart Journal 34.7 (2013): 503.
  13. Behr E., et al. “Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome”. Lancet 362.9394 (2003): 1457.
  14. Tan HL., et al. “Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives”. Circulation 112.2 (2005): 207.
  15. Giudici V., et al. “Sudden arrhythmic death syndrome: diagnostic yield of comprehensive clinical evaluation of pediatric first-degree relatives”. Pacing and Clinical Electrophysiology 37.12 (2014): 1681-1685.
  16. Wever EF and Robles de Medina EO. “Sudden death in patients without structural heart disease”. Journal of the American College of Cardiology 43.7 (2004): 1137.
  17. Goldenberg I., et al. “Risk factors for aborted cardiac arrest and sudden cardiac death in children with the congenital long-QT syndrome”. Circulation 117 (2008): 2184-2191.
  18. Kaufman ES., et al. “Risk of death in the long QT syndrome when a sibling has died”. Heart Rhythm 5 (2008): 831-836.
  19. Ali RH., et al. “Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with long QT syndrome”. American Journal of Cardiology 85 (2000): 457-461.
  20. Arnestad M., et al. “Prevalence of long-QT syndrome gene variants in sudden infant death syndrome”. Circulation 115 (2007): 361-363.
  21. Johnson JN., et al. “Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy”. Neurology 72 (2009): 224-231.
  22. Priori SG., et al. “Low penetrance in the long-QT syndrome: clinical impact”. Circulation 99 (1999): 529-533.
  23. Leenhardt A., et al. “Catecholaminergic polymorphic ventricular tachycardia in children: a 7-year follow-up of 21 patients”. Circulation 91 (1995): 1512-1519.
  24. Liu N., et al. “Catecholaminergic polymorphic ventricular tachycardia”. Progress in Cardiovascular Diseases 51 (2008): 23-30.
  25. Viitasalo M., et al. “U-waves and T-wave peak to T-wave end intervals in patients with catecholaminergic polymorphic ventricular tachycardia: effects of beta-blockers”. Heart Rhythm 5 (2008): 1382-1388.
  26. Tester DJ., 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 Clinic Proceedings 79.11 (2004): 1380.
  27. Brugada P and Brugada J. “Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome: a multicenter report”. Journal of the American College of Cardiology 20 (1992): 1391-1396.
  28. Antzelevitch C., et al. “Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association”. Circulation 111 (2005): 659-670.
  29. Ikeda T., et al. “The full stomach test as a novel diagnostic technique for identifying patients at risk of Brugada syndrome”. Journal of Cardiovascular Electrophysiology 17 (2006): 602-660.
  30. Morita H., et al. “Atrial fibrillation and atrial vulnerability in patients with Brugada syndrome”. Journal of the American College of Cardiology 40 (2002): 1437-1444.
  31. Brugada R., et al. “Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts”. Circulation 101 (2000): 510-515.
  32. Haissaguerre M., et al. “Sudden cardiac arrest associated with early repolarization”. The New England Journal of Medicine 358 (2008): 2016-2023.
  33. Krahn AD., et al. “Systematic assessment of patients with unexplained cardiac arrest: Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)”. Circulation 120 (2009): 278-285.
  34. Benito B., et al. “Clinical and mechanistic issues in early repolarization of normal variants and lethal arrhythmia syndromes”. Journal of the American College of Cardiology 56 (2010): 1177-1186.
  35. Tikkanen JT., et al. “Long-term outcome associated with early repolarization on electrocardiography”. The New England Journal of Medicine 361 (2009): 2529-2537.
  36. Gaita F., et al. “Short QT syndrome: a familial cause of sudden death”. Circulation 108 (2003): 965-970.
  37. Giustetto C., et al. “Short QT syndrome: clinical findings and diagnostic-therapeutic implications”. European Heart Journal 27 (2006): 2440-2447.
  38. Lobo FV., et al. “Left ventricular involvement in right ventricular dysplasia/cardiomyopathy”. Canadian Journal of Cardiology 15.11 (1999): 1239-1247.
  39. Sen-Chowdhry S., et al. “Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression”. Circulation 115.13 (2007): 1710-1720.
  40. Ferreira AC., et al. “Arrhythmogenic right ventricular dysplasia in the elderly”. Heart disease 5.6 (2003): 393-396.
  41. Marcus FI., et al. “Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force criteria”. Circulation 31 (2010): 806-881.
  42. den Haan AD., et al. “A Comprehensive desmosome mutation analysis in North Americans with arrhythmogenic right ventricular dysplasia/cardiomyopathy”. Circulation Cardiovascular Genetics 2 (2009): 428-435.
  43. “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 795.1 (1997): 265-272.
  44. Ozaydin M., et al. “Long-Term Outcome of Patients with Idiopathic Ventricular Fibrillation: A Meta-Analysis”. Journal of Cardiovascular Electrophysiology 26.10 (2015): 1095-1014.
  45. Maron BJ., et al. “Clinical profile and spectrum of commotio cordis”. JAMA 287.9 (2002): 1142.
  46. Maron BJ and Estes NA. “Commotio cordis”. The New England Journal of Medicine 362.10 (2010): 917.
Citation: Surjya Prasad Upadhyay., et al. “Sudden Cardiac Arrest in a Normal Heart- Approach to Evaluation”. EC Emergency Medicine and Critical Care 4.4 (2020): 51-60.

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