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Editorial
Sudden Cardiac Death For the families of affected individuals, sudden cardiac death (SCD) comes as a devastating and shocking experience as it often claims the lives of young previously fit adults. Detailed case history, meticulous autopsy examination, and complete toxicological screening are essential to arrive at the underlying cause of death. Most of the cases of sudden death have a cardiac genetic cause, so a detailed autopsy and examination of the heart is essential in all cases.[1] As expected, a UK prospective study in the 1990s showed ischemic heart disease in the majority. This study also highlighted nonischemic heart disease in 7% of cases, particularly hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic cardiomyopathy. In the final 7% of cases, after detailed pathological examination of the whole heart, no cause of death could be established.[2] This study was first greeted with skepticism by the medical profession, particularly cardiologists. Gradually, it became apparent that it was electrical abnormalities that were responsible for these sudden deaths and could be found in their relatives on screening. The term SCD or sudden arrhythmic death (SAD) was introduced, indicating the mode of death as a lethal cardiac arrhythmia. It has also been called as sudden adult death to distinguish it from sudden infant death, but it can occur at any age, not just in adulthood. The incidence of this entity has been unknown and generally underestimated. A further, prospective study showed that SAD occurred predominantly in the young males. The estimated incidence rate of these unexplained cardiac deaths is 1.34/100,000/annum, a potential of over 500 SADs in England per annum. SAD syndrome (SADS) should, therefore, be a certifiable cause of death prompting specialized cardiological evaluation of families.[3] A further study indicated even higher figures with 1.8/100,000/year.[4] Our pathological study of over 400 sudden deaths in England indicated that electrical faults in the hearts are becoming a frequent cause of sudden death.[5] In a further study of families of SADS victims, 53% screened were diagnosed with inheritable heart disease.[6] Other causes of sudden death include mitral valve prolapse, conduction system abnormalities, congenital coronary artery anomalies, myocarditis, aortic rupture, nonatherosclerotic‑acquired coronary artery disease, postoperative congenital heart disease, and aortic stenosis. Thus, a large spectrum of cardiovascular disorders, both congenital and acquired, is responsible for SCD in the young males. Therefore, an accurate cardiac pathological examination is essential.[7] Follow‑up in families with clinical examination is vital for appropriate prophylaxis among relatives, guided and confirmed by genetic analysis.[8]
Mary N. Sheppard CRY Cardiovascular Pathology, Cardiovascular Sciences Research Centre, St. George’s University of London, United Kingdom Address for correspondence: Prof. Mary N. Sheppard, CRY Cardiovascular Pathology, Cardiovascular Sciences Research Centre, St. George’s University of London, United Kingdom. E‑Mail:
[email protected]
References 1. Sheppard MN. Approach to the cardiac autopsy. J Clin Pathol 2012;65:484‑95. 2. Bowker TJ, Wood DA, Davies MJ, Sheppard MN, Cary NR, Burton JD, et al. Sudden, unexpected cardiac or unexplained death in England: A national survey. QJM 2003;96:269‑79. 3. Behr ER, Casey A, Sheppard M, Wright M, Bowker TJ, Davies MJ, et al. Sudden arrhythmic death syndrome: A national survey of sudden unexplained cardiac death. Heart 2007;93:601‑5. 4. Papadakis M, Sharma S, Cox S, Sheppard MN, Panoulas VF, Behr ER. The magnitude of sudden cardiac death in the young: A death certificate‑based review in England and Wales. Europace 2009;11:1353‑8. 5. Fabre A, Sheppard MN. Sudden adult death syndrome and other non‑ischaemic causes of sudden cardiac death. Heart 2006;92:316‑20. 6. Behr ER, Dalageorgou C, Christiansen M, Syrris P, Hughes S, Tome Esteban MT, et al. Sudden arrhythmic death syndrome: Familial evaluation identifies inheritable heart disease in the majority of families. Eur Heart J 2008;29:1670‑80. 7. de Noronha SV, Behr ER, Papadakis M, Ohta‑Ogo K, Banya W, Wells J, et al. The importance of specialist cardiac histopathological examination in the investigation of young sudden cardiac deaths. Europace 2014;16:899‑907. 8. Wilde AA, Behr ER. Genetic testing for inherited cardiac disease. Nat Rev Cardiol 2013;10:571‑83. This is an open access article distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as the author is credited and the new creations are licensed under the identical terms. Access this article online Quick Response Code:
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DOI: 10.4103/2395-5414.166319
How to cite this article: Sheppard MN. Sudden cardiac death. J Pract Cardiovasc Sci 2015;1:103.
© 2015 Journal of the Practice of Cardiovascular Sciences | Published by Wolters Kluwer - Medknow
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