SUDDEN UNEXPECTED DEATH
IN THE YOUNG
Jon Skinner, Jo Duflou & Julie McGaughran
TRAGADY(Trans-Tasman Response Against Sudden
Death in the Young)
BACKGROUND• 400,000 sudden deaths per annum in USA
• Several thousand young adult sudden deaths
• No apparent warning signs in 50% of young SUD
• Distribution of causes of SUD poorly studied
• Significant number have a familial aetiology
METHODS
• All autopsies performed over a 10 yr period at DOFM Glebe were reviewed
• Cases of sudden, unexpected natural death between ages of 5 and 35 selected
• Sudden death defined as death occurring within 24 hours of onset of symptoms
• Review of “difficult” cases by cardiologist and pathologist
DEMOGRAPHICS• Cardiac deaths:
– 241 cases– Mean age 26.8 ± 5.9 yrs– 78.4% male– Mean BMI: 27.1 ± 8.4 kg/m2
• Non-cardiac deaths:– 168 cases– Mean age 25.2 ± 7.6 yrs– 58.9% male– Mean BMI: 26.5 ± 8.4 kg/m2
• Undetermined deaths:– 18 cases
RESULTS - CARDIAC
Other 12%
Myocarditis 12%
HCM 6%
LVH 3%
Athero-sclerosis 25%
Arrhythmia 29%
DCM 5%CHD 3%
Aortic Dissection 5%
PRIMARY ARRHYTHMIA• 70 cases
• Criteria for inclusion:– Documented terminal arrhythmia– Documented collapse with cardiac arrest– Found dead after being asymptomatic in
preceding 24 hours– No or minimal structural pathology
• Confirmed Long QT syndrome in 6 cases
ATHEROSCLEROSIS• 59 cases, 96.6% male• Mean BMI 30
Plaque rupture & thrombus Acute Myocardial infarct
ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA
(ARVD)• Only 4 cases (1%)
• Compared to 13% in Italian study
• Importance of standard diagnostic criteria & appropriate tissue sampling
RESULTS – NON-CARDIAC
ICH 23.8%
Asthma 16.1%
Pulmonary Embolism
12.5%Sepsis 8.9%
Diabetes related 4.2%
Epilepsy 23.8%
Other 10.7%
EPILEPSY
• 40 cases
• Majority classified as SUDEP:– Sudden death
– Typically young adult, post-traumatic
– Apparently during sleep
– No evidence of status epilepticus
– Often subtherapeutic levels of anti-epileptics
PULMONARY THROMBO-EMBOLISM
• 21 cases
• Higher body weight (mean 99 kg) & BMI (mean 34.7) than others
• Infrequent history of long-distance travel
NEGATIVE AUTOPSY DEATHS
• 18 cases
• Criteria for inclusion:– Unknown antemortem
circumstances (15)– Advanced
decomposition (3)
CONCLUSIONS
• The heart was structurally normal in about 30% of SCD cases
• Most likely cause of arrhythmia in structurally normal hearts are genetic syndromes, especially channelopathies
• Early onset atherosclerosis second most frequent COD
• Effective family screening is an important adjunct to the autopsy