SUDDEN UNEXPECTED DEATH IN THE YOUNG Jon Skinner, Jo Duflou & Julie McGaughran TRAGADY (Trans-Tasman Response Against Sudden Death in the Young )

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

RESULTS

~200,000 deaths

25,277 investigated

3,854 aged 5-35

427 natural

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

MYOCARDITIS HYPERTROPHIC CARDIOMYOPATHY

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