Heart failure & cardiomyopathiesCohort study

Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy

JAMA CardiolPublished

  • Retrospective cohort study
  • multicentre
  • masked cardiovascular magnetic resonance (CMR) observers

Authors' conclusion

In this retrospective cohort study, quantitative LGE was a risk factor for SCD in patients younger than 21 years with HCM and improved risk stratification.

Study context

Population
Patients with hypertrophic cardiomyopathy (HCM) younger than 21 years who underwent CMR, sites in the US, Europe and South America, 2015–2022
Participants
700 patients · 37 international centres
Exposure
Late gadolinium enhancement (LGE) vs no LGE on CMR
  • LGEn = 230
Outcome
SCD and surrogate events (resuscitated cardiac arrest, appropriate ICD discharges)
Follow-up
Median 1.9 (0.5-4.1) years

Main finding

SCD or equivalent events

Unadjusted hazard ratio

2.19

95% CI 1.59–3.02

P < 0.001

LGE ≥10% of total myocardium vs No LGE
unadjusted HR 2.19 (95% CI 1.59–3.02); P < 0.001

As reported in the abstract

Sudden cardiac death (SCD) or equivalent events, whole cohort
35 (5.0%)

Observational data: associations, not effects.

Further findings

LGE prevalence and burden
5.9% · mean · SD 7.3% · of LV myocardium
LGE prevalence and burden — LGE present
230 (32.9%)

SCD prediction, HCM Risk-Kids score with LGE burden

Before LGE addition
0.66 (95% CI 0.58-0.75)
After LGE addition
0.73 (95% CI 0.66-0.81)

SCD prediction, Precision Medicine in Cardiomyopathy score with LGE burden

Before LGE addition
0.68 (95% CI 0.49-0.77)
After LGE addition
0.73 (95% CI 0.64-0.82)

Original publication

Myocardial Scarring and Sudden Cardiac Death in Young Patients With Hypertrophic Cardiomyopathy. JAMA Cardiol. Published 1 November 2024. doi:10.1001/jamacardio.2024.2824

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