Heart failure & cardiomyopathiesCohort study

Countywide burden, pathology, and genetics of lethal hypertrophic cardiomyopathy: from the POST sudden cardiac death (SCD) study

EuropacePublished

  • Prospective cohort study
  • countywide
  • post-mortem

All incident cases 2/1/2011-3/1/2014 (n = 525), then approximately every third day 3/1/2014-9/1/2022 (n = 497)

Authors' conclusion

In this 11-year countywide post-mortem study, HCM meeting pathologic, clinical, or genetic criteria was associated with autopsy-confirmed arrhythmic cause of sudden death, accounting for 2% of SADs up to age 90, highest in cases <35 years old.

Study context

Population
Presumed SCDs (WHO criteria) aged 0–90 years in San Francisco County
Participants
1,022 pSCDs
Exposure
pSCDs with hypertrophic cardiomyopathy (HCM) vs without HCM
  • HCMn = 13
  • Without HCMn = 1,009reference

Main finding

As reported in the abstract

Burden of HCM among pSCDs
pSCDs with HCM
13
Arrhythmic deaths among HCM cases
11
Burden of sudden death (pSCD)
1.3%
Burden of arrhythmic death
2%
Pre-mortem diagnosis of HCM
2 of 13 (15%)
Incidence, persons with HCM 18-35 years, pSCDs/year
0.2%
Incidence, persons with HCM 18-35 years, SADs/year
0.1%

Observational data: associations, not effects.

Further findings

Arrhythmic cause of death

P = 0.03

pSCD burden due to HCM by age

Decreased with age
P = 0.003
Age <35 years: share of pSCD
7.1%
Age <35 years: share of arrhythmic death
9.4%

Genetic testing of 317 consented pSCDs

Pathogenic or likely pathogenic variants
40% (2/5)
  • identified one additional case without clinical phenotype

Original publication

Countywide burden, pathology, and genetics of lethal hypertrophic cardiomyopathy: from the POST SCD study. Europace. Published 18 April 2025. doi:10.1093/europace/euaf088

Summarised from the published abstract; every number is checked against it