Tab. 2.

Key principles of postmortem investigations of sudden unexpected death in the young (adapted from “Post-mortem in sudden unexpected death in the young: guidelines on autopsy practice,”devised by TRAGADY—Trans-Tasman Response Against Sudden Death in the Young—and endorsed bythe Royal College of Pathologists of Australasia [38])

1. All cases of sudden unexpected or unexplained death in the young (age group of 0–40 years) should have an autopsy

2. A full postmortem examination should be completed

3. The investigation, ideally lead by a pathologist, should involve a team approach A person designated to liaise with the family Specialist cardiology involvement with the family when noncardiac causes are excluded Laboratories with molecular genetics, toxicology, and metabolic expertise

4. A detailed antecedent clinical history must be obtained Circumstances of the death—detailed review of the date, time, place, and activity surrounding the death (i.e., at home, work, or on the athletic field, at rest or during exercise or emotional excitement). Was the death witnessed? Document any associated seizures, prodromal symptoms Past medical history—Document the general health status, including previous significant illness or events such as syncope, seizures, epilepsy, palpitations, and respiratory or neurological disease. Retrieve results of any prior investigations (e.g., ECG, EEG, CT, or MRI) Previous surgical procedures or interventions—Document details of current medications, in particular those that are known to be pro-arrhythmic (see: www.qtdrugs.org)

5. A detailed and relevant family history must be obtained Family history—Document any family history of premature death (explained or unexplained death, SIDS, unexplained drowning, or unusual motor vehicle accidents), seizures, or syncope. Be sure to include a detailed description of the date, time, place, and activity surrounding such events, if available Diagnosed disorders—Document any family history of clinical diagnosis of potentially arrhythmic disorders, including LQTS, CPVT, BrS, familial cardiomyopathy, or other cardiac conditions

6. Skilled macroscopic and microscopic examination of the organs is required, particularly of the heart (especially right ventricular muscle) and the brain. This may require some specimens to be examined by other specialists

7. Adequate histological material be obtained for review or, if necessary, referral

8. Tissue or blood suitable for DNA extraction must be obtained

9. Results, including photography, must be documented clearly

10. Results must be described and annotated in a standard fashion which will allow epidemiological data gathering