Heart failure & cardiomyopathies
Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry
Authors' conclusion
Patients with childhood-diagnosed HCM have a significantly higher lifetime risk of developing LVSD, and LVSD emerges earlier than for patients with adult-diagnosed HCM. Regardless of age at diagnosis with HCM or LVSD, the prognosis with LVSD is poor
Study context
- Population
- Patients with hypertrophic cardiomyopathy (HCM) in the SHaRe registry
- Participants
- 1,010 vs 6,741
- Exposure
- Childhood-diagnosed HCM vs adult-diagnosed HCM
- Pediatricn = 1,010
- Adultn = 6,741reference
- Outcome
- Prevalence, predictors and prognosis of LVSD (LVEF <50%); prognosis by composite of death, cardiac transplantation and LV assist device implantation
Methodological detailsinternational, multicenter
- International
- Multicenter
Main finding
As reported in the abstract
LVSD prevalence (LVEF <50%)
| Group | Value | Median age at incident left ventricular systolic dysfunction (LVSD) |
|---|---|---|
| Adult | 8.7% | 57.2 years (IQR 47.3-66.5) |
Adult
Value8.7%
Median age at incident left ventricular systolic dysfunction (LVSD)57.2 years (IQR 47.3-66.5)
Observational data: associations, not effects.
Further findings
Predictors of incident LVSD in childhood-diagnosed HCM
| HR (CI) | ||
|---|---|---|
| Age <12 years at HCM diagnosis | 1.72 (1.13–2.62) | |
| Male sex | 3.1 (1.88–5.2) | |
| Pathogenic sarcomere variant | 2.19 (1.08–4.4) | |
| Previous septal reduction therapy | 2.34 (1.42–3.9) | |
| 0.20.5125Hazard ratio (log scale) |
| Outcome | HR (CI) | |
|---|---|---|
| Predictors of incident LVSD in childhood-diagnosed HCMLower initial LVEF · per 5% decrease | 1.53 (1.38–1.69) | |
| 0.512Hazard ratio (log scale) |
Composite outcome (death, transplantation, LV assist device) in childhood-diagnosed HCM with LVSD
| HR (CI) | ||
|---|---|---|
| Female | 2.60 (1.41–4.78) | |
| LVEF <35% | 3.76 (2.16–6.52) | |
| 0.10.31310Hazard ratio (log scale) |
- Composite outcome (death, transplantation, LV assist device) in childhood-diagnosed HCM with LVSD — Patients meeting composite outcome
- 40%
Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.
Original publication
Left Ventricular Systolic Dysfunction in Patients Diagnosed With Hypertrophic Cardiomyopathy During Childhood: Insights From the SHaRe Registry. Circulation. Published 25 May 2023. doi:10.
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