Sudden cardiac death in the young: the molecular autopsy and a practical approach to surviving relatives

European Heart Journal
Christopher SemsarianArthur A M Wilde

Abstract

The sudden death of a young, apparently fit and healthy person is amongst the most challenging scenarios in clinical medicine. Sudden cardiac death (SCD) is a devastating and tragic outcome of a number of underlying cardiovascular diseases. While coronary artery disease and acute myocardial infarction are the most common causes of SCD in older populations, genetic (inherited) cardiac disorders comprise a substantial proportion of SCD cases aged 40 years and less. This includes the primary arrhythmogenic disorders such as long QT syndromes and inherited cardiomyopathies, namely hypertrophic cardiomyopathy. In up to 30% of young SCD, no cause of death is identified at postmortem, so-called autopsy-negative or sudden arrhythmic death syndrome (SADS). Management of families following SCD begins with a concerted effort to identify the cause of death in the decedent, based on either premorbid clinical details or the pathological findings at postmortem. Where no cause of death is identified, genetic testing of deoxyribonucleic acid extracted from postmortem blood (the molecular autopsy) may identify a cause of death in up to 30% of SADS cases. Irrespective of the genetic testing considerations, all families in which a sudden unexplain...Continue Reading

References

Jul 15, 1995·The American Journal of Cardiology·W K ShenB J Gersh
Apr 18, 1996·The New England Journal of Medicine·R R Liberthson
Oct 26, 1996·Lancet·J A BrookesW R Lees
Nov 15, 1997·Journal of the American College of Cardiology·J J de Vreede-SwagemakersH J Wellens
Nov 24, 1998·Circulation·D P Zipes, H J Wellens
Jul 27, 2000·The New England Journal of Medicine·P J SchwartzR Bloise
May 4, 2001·Cardiovascular Research·D CorradoG Thiene
Oct 31, 2001·Lancet·P J SchwartzJ Nastoli
Nov 17, 2001·JAMA : the Journal of the American Medical Association·M J AckermanJ A Towbin
Sep 12, 2003·The New England Journal of Medicine·Barry J Maron
Nov 7, 2003·Lancet·E BehrUNKNOWN Sudden Arrhythmic Death Syndrome Steering Group
Jan 30, 2004·The Medical Journal of Australia·Alessandra DoolanChristopher Semsarian
May 4, 2004·Journal of the American College of Cardiology·Sumeet S ChughSusan J Hayflick
Dec 8, 2004·Annals of Internal Medicine·Robert E EckartRenu Virmani
May 13, 2005·Mayo Clinic Proceedings·David J TesterMichael J Ackerman
Dec 20, 2005·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Rajesh PuranikMark A McGuire
Jan 11, 2007·Circulation·Marianne ArnestadPeter J Schwartz
Jan 16, 2007·Journal of the American College of Cardiology·David J Tester, Michael J Ackerman
May 1, 2007·Lancet·Julian L Burton, James Underwood
Jun 5, 2007·International Journal of Cardiology·Alessandra DoolanChristopher Semsarian
Jun 9, 2007·Heart Rhythm : the Official Journal of the Heart Rhythm Society·David J TesterMichael J Ackerman
Feb 19, 2008·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Jodie InglesChristopher Semsarian
Nov 18, 2008·The Medical Journal of Australia·Jon R SkinnerChristopher Semsarian
Feb 10, 2010·Nature Reviews. Cardiology·A Selcuk AdabagBernard J Gersh
Sep 25, 2010·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Ray E HershbergerJill D Siegfried
Nov 13, 2010·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Jonathan R SkinnerUNKNOWN Cardiac Inherited Disease Group New Zealand
Dec 7, 2010·European Heart Journal·Bo Gregers WinkelJacob Tfelt-Hansen
Apr 12, 2011·European Heart Journal·Marina Cerrone, Silvia G Priori
Jul 20, 2011·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Jodie InglesChristopher Semsarian
Jul 30, 2011·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Ronan MargeyJoseph Galvin
Aug 6, 2011·Heart Rhythm : the Official Journal of the Heart Rhythm Society·Christopher Semsarian, Robert M Hamilton
Sep 10, 2011·Journal of the American College of Cardiology·Robert E EckartUNKNOWN Department of Defense Cardiovascular Death Registry Group
Feb 7, 2012·Pediatric Cardiology·David J Tester, Michael J Ackerman
Aug 14, 2012·Journal of Cardiovascular Electrophysiology·Bo Gregers WinkelJytte Banner
Apr 10, 2013·European Journal of Cardiovascular Nursing : Journal of the Working Group on Cardiovascular Nursing of the European Society of Cardiology·Laura YeatesJodie Ingles
May 15, 2013·Circulation. Arrhythmia and Electrophysiology·Michael PapadakisSanjay Sharma
Jun 6, 2013·European Journal of Human Genetics : EJHG·Christian van der WerfEllen M A Smets
Aug 1, 2013·Nature Reviews. Cardiology·Arthur A M Wilde, Elijah R Behr

❮ Previous
Next ❯

Citations

Jun 26, 2015·Journal of Human Genetics·Yukiko Nakano, Wataru Shimizu
Aug 1, 2015·International Journal of Legal Medicine·Eva-Lena StattinAase Wisten
Oct 21, 2016·Journal of Arrhythmia·Christopher Semsarian, Jodie Ingles
Jul 28, 2015·Journal of cardiology·Yasunori SuematsuUNKNOWN Japanese Circulation Society Resuscitation Science Study (JCS-ReSS) Group
Jul 16, 2015·European Heart Journal·Thomas F Lüscher
Jul 15, 2015·European Heart Journal·Thomas F Lüscher
Jun 3, 2015·European Heart Journal·Thomas F Lüscher
Sep 26, 2016·Trends in Cardiovascular Medicine·Christian SteinbergAndrew D Krahn
Jun 16, 2016·Frontiers in Cardiovascular Medicine·Najim LahrouchiConnie R Bezzina
Jun 23, 2016·The New England Journal of Medicine·Richard D BagnallChristopher Semsarian
Oct 4, 2016·Cardiology Clinics·Joanna Sweeting, Christopher Semsarian
Sep 13, 2016·Cardiovascular Pathology : the Official Journal of the Society for Cardiovascular Pathology·Giulia Ottaviani, L Maximilian Buja
Mar 24, 2017·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Richard D BagnallChristopher Semsarian
Mar 30, 2017·Forensic Science, Medicine, and Pathology·Michael BurkeAdam Murdoch
May 5, 2017·Nature Reviews. Cardiology·Pietro Enea LazzeriniMohamed Boutjdir
Mar 4, 2017·Sports Medicine·Oscar CampuzanoRamon Brugada
Sep 11, 2017·Virchows Archiv : an International Journal of Pathology·Cristina BassoUNKNOWN Association for European Cardiovascular Pathology
Jun 25, 2016·The Journal of Law, Medicine & Ethics : a Journal of the American Society of Law, Medicine & Ethics·Quianta L MooreAmy L McGuire
Jul 1, 2017·Journal of Clinical Pathology·Chantal Van NiekerkLorraine du Toit-Prinsloo
Jun 11, 2017·Circulation. Cardiovascular Genetics·Christopher Semsarian, Arthur A M Wilde
Sep 23, 2018·Heart·Chrishan J NalliahPrashanthan Sanders
Jul 22, 2018·Archivos de cardiología de México·Juan Carlos BonillaJosep Brugada
Jul 12, 2019·Internal Medicine Journal·Julia Isbister, Christopher Semsarian
Aug 15, 2019·Pacing and Clinical Electrophysiology : PACE·Bishoy Deif, Jason D Roberts
Apr 28, 2018·Clinical Medicine : Journal of the Royal College of Physicians of London·Gaetano Thiene
Oct 7, 2017·Current Opinion in Cardiology·Christian Steinberg
Jun 2, 2020·Circulation·Laura A Hancock, Graham Nichol
Aug 12, 2020·Netherlands Heart Journal : Monthly Journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation·A A M WildeC van der Werf
Sep 25, 2019·Cold Spring Harbor Perspectives in Medicine·Jodie Ingles
Dec 21, 2019·The Clinical Biochemist. Reviews·Han-Chih Hencher Lee, Chor-Kwan Ching
Feb 23, 2020·Open Heart·Elizabeth Davida ParatzUNKNOWN Australian UCDP Registry
Jul 15, 2016·Genome Research·Amy L McGuireUNKNOWN Molecular Autopsy Consortium of Houston (MATCH)
Nov 29, 2017·Frontiers in Cardiovascular Medicine·Manuel RuedaAli Torkamani
Jun 17, 2018·Forensic Science, Medicine, and Pathology·Barbara Ströh van DeventerChantal van Niekerk
Sep 15, 2018·Netherlands Heart Journal : Monthly Journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation·B van DrielJ van der Velden

❮ Previous
Next ❯

Related Concepts

Related Feeds

Cardiology Journals

Discover the latest cardiology research in this collection of the top cardiology journals.

Cardiomyopathy

Cardiomyopathy is a disease of the heart muscle, that can lead to muscular or electrical dysfunction of the heart. It is often an irreversible disease that is associated with a poor prognosis. There are different causes and classifications of cardiomyopathies. Here are the latest discoveries pertaining to this disease.

Arrhythmia

Arrhythmias are abnormalities in heart rhythms, which can be either too fast or too slow. They can result from abnormalities of the initiation of an impulse or impulse conduction or a combination of both. Here is the latest research on arrhythmias.

Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia is a congenital cardiomyopathy that is characterized by infiltration of adipose and fibrous tissue into the right ventricle wall and loss of myocardial cells. Primary injuries usually are at the free wall of the right ventricular and right atria resulting in ventricular and supraventricular arrhythmias. Discover the latest research on arrhythmogenic right ventricular dysplasia here.

Related Papers

Heart Rhythm : the Official Journal of the Heart Rhythm Society
Christopher Semsarian, Robert M Hamilton
Internal Medicine Journal
Jodie Ingles, Christopher Semsarian
Therapeutic Advances in Cardiovascular Disease
Rhian Shephard, Christopher Semsarian
© 2021 Meta ULC. All rights reserved