Download sudden unexpected 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
Arrhythmia
29%
Atherosclerosis
25%
Myocarditis
12%
Other 12%
HCM 6%
LVH 3%
CHD 3%
DCM 5%
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
Epilepsy
23.8%
ICH 23.8%
Asthma
16.1%
Other
10.7%
Diabetes
related
4.2%
Sepsis
8.9%
Pulmonary
Embolism
12.5%
EPILEPSY
• 40 cases
• Majority classified as SUDEP:
– Sudden death
– Typically young adult, posttraumatic
– Apparently during sleep
– No evidence of status epilepticus
– Often subtherapeutic levels of antiepileptics
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