Reducing sudden death in young people in Australia and  New Zealand: the TRAGADY initiative

Skinner, Jon R; Duflou, Johan A; Semsarian, Christopher

doi:10.5694/j.1326-5377.2008.tb02173.x

Topics

Best-practice guidelines mandate a full postmortem examination in these deaths to identify genetic causes and allow potentially life-saving interventions in the victim’s relatives

The sudden and unexpected death of an apparently fit, healthy young person has a devastating effect on the family and community. If there is no adequate explanation for the death, the desperate sadness is usually compounded by frustration and fear that another family member may be struck. Yet, there is no uniform process in place in Australia and New Zealand to obtain a proper medical history of the victim, or to examine and investigate the victim’s family. Such a process could provide clues to the diagnosis and also identify previously undiagnosed genetic diseases.

1 Green Lane Paediatric and Congenital Cardiac Services, Starship Children’s Hospital, Auckland, New Zealand.
2 Department of Forensic Medicine, Sydney, NSW.
3 Department of Pathology, University of Sydney, Sydney, NSW.
4 Royal Prince Alfred Hospital, and Faculty of Medicine, University of Sydney, Sydney, NSW.
5 Agnes Ginges Centre for Molecular Cardiology, Centenary Institute, Sydney, NSW.

Correspondence: jskinner@adhb.govt.nz

Acknowledgements:

We thank the wider membership of TRAGADY for donating their time and expertise. We are also grateful for the seeding grant provided by the Australia and New Zealand Children’s Heart Research Centre.

1. Doolan A, Langlois N, Semsarian C. Causes of sudden cardiac death in young Australians. Med J Aust 2004; 180: 110-112. <MJA full text>
2. Puranik R, Chow CK, Duflou JA, et al. Sudden death in the young. Heart Rhythm 2005; 2: 1277-1282.
3. Mohamed U, Napolitano C, Priori SG. Molecular and electrophysio-logical bases of catecholaminergic polymorphic ventricular tachycardia. J Cardiovasc Electrophysiol 2007; 18: 791-797.
4. Chung SK, MacCormick JM, McCulley CH, et al. Long QT and Brugada syndrome gene mutations in New Zealand. Heart Rhythm 2007; 4: 1306-1314.
5. Maron BJ, Spirito P, Shen WK, et al. Prevention of sudden cardiac death and selection of patients for implantable cardioverter-defibrillators in hypertrophic cardiomyopathy. JAMA 2007; 298: 405-412.
6. Tan HL, Hofman N, van Langen IM, et al. Sudden unexplained death: heritability and diagnostic yield of cardiological and genetic examination in surviving relatives. Circulation 2005; 112: 207-213.
7. Tester DJ, Ackerman MJ. Postmortem long QT syndrome genetic testing for sudden unexplained death in the young. J Am Coll Cardiol 2007; 49: 240-246.
8. Ingles J, Semsarian C. Sudden cardiac death in the young: a clinical genetic approach. Intern Med J 2007; 37: 32-37.
9. TRAGADY mission statement. http://www.cidg.org/webcontent/cidg/Home/TRAGADY/tabid/161/Default.aspx (accessed Nov 2005).
10. Trans-Tasman Response Against Sudden Death in the Young (TRAGADY). Post-mortem in sudden unexpected death in the young: guidelines on autopsy practice. Sydney: Royal College of Pathologists of Australasia, 2008. http://www.rcpa.edu.au/applications/DocumentLibraryManager2/upload/SUDYbestpracticedocumentendorsedMay 2708%20(2).pdf (accessed May 2008).

Online responses are no longer available. Please refer to our instructions for authors page for more information.