Heart failure & cardiomyopathies
Prevalence, risk factors, and outcomes of QT prolongation in primary and RASopathy-associated hypertrophic cardiomyopathy
Authors' conclusion
JTc prolongation was independently associated with the risk of SCD events. Including JTc prolongation with SCD risk scores improved 5-year SCD risk prediction for P-HCM. This has implications for closer SCD risk monitoring in patients with P-HCM with JTc prolongation.
Study context
- Population
- Phenotype-positive, pediatric patients with primary hypertrophic cardiomyopathy (P-HCM) and RASopathy hypertrophic cardiomyopathy
- Participants
- 212 vs 55
- Comparison
- Association of JTc prolongation with risk of sudden cardiac death events
- P-HCMn = 212
- RASopathy hypertrophic cardiomyopathyn = 55
- Outcome
- Prolonged JTc
Main finding
As reported in the abstract
Further findings
Sudden cardiac death (SCD) events in P-HCM
| Hazard ratio (CI) | P value | ||
|---|---|---|---|
| JTc prolongationMultivariable analysis | 2.9 (1.2–6.8) | P = 0.016 | |
| 0.10.31310Hazard ratio (log scale) |
Correlation of JTc with severity of left ventricular hypertrophy
- JTc had only a modest correlation with the severity of left ventricular hypertrophy
SCD event-free survival from baseline evaluation in P-HCM · 5-year
- 5-year SCD event-free survival, P-HCM
- 86%
C-statistic for 5-year SCD risk prediction with JTc added as a risk factor
- Precision Medicine in Cardiomyopathy risk scores with JTc
- 0.85
- Precision Medicine in Cardiomyopathy risk scores alone
- 0.73
- Hypertrophic cardiomyopathy Risk-Kids scores with JTc
- 0.73
- Hypertrophic cardiomyopathy Risk-Kids scores alone
- 0.70
Original publication
Prevalence, risk factors, and outcomes of QT prolongation in primary and RASopathy-associated hypertrophic cardiomyopathy. Heart Rhythm. Published 3 June 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →