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Prognostic factors in hypertrophic cardiomyopathy in children: An MRI based study
Lamia Ait Ali1, Chiara Marrone2, Duarte Saraiva Martins3
1Institute of Clinical Physiology CNR, UO Massa and Fondazione G. Monasterio. CNR-Regione Toscana. Italy.
Insights
In children with hypertrophic cardiomyopathy (HCM), cardiac magnetic resonance (CMR) imaging showing late gadolinium enhancement (LGE) and a history of syncope predict adverse cardiovascular events.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- The clinical and prognostic role of cardiac magnetic resonance (CMR) in adults with hypertrophic cardiomyopathy (HCM) is established.
- The predictive value of CMR in pediatric populations with HCM requires further investigation.
Purpose of the Study:
- To assess the role of CMR in predicting cardiovascular events in children diagnosed with HCM.
- To identify specific CMR-derived parameters and clinical factors associated with adverse outcomes in pediatric HCM.
Main Methods:
- A cohort of 116 children with HCM underwent CMR, including cine imaging and late gadolinium enhancement (LGE).
- Patients were followed for hard cardiac events, defined as sustained ventricular tachycardia, cardiac arrest, sudden cardiac death, end-stage heart failure, heart transplant, or appropriate ICD intervention.
- Statistical analysis, including maximal-rank statistic and Harrel's C statistic, was used to identify predictors of events.
Main Results:
- During a median follow-up of 4 years, 21 cardiac events occurred.
- An LGE extent of ≥2% was identified as an optimal cut-point for predicting events.
- Syncope, non-sustained ventricular tachycardia (NSVT), and LGE extent ≥2% were independent predictors of hard cardiac events.
- The combination of LGE extent ≥2% and syncope demonstrated the strongest predictive model, with a hazard ratio of 37.6.
Conclusions:
- Late gadolinium enhancement (LGE) and syncope are significant independent predictors of hard cardiac events in children with hypertrophic cardiomyopathy (HCM).
- CMR, particularly LGE extent, provides valuable prognostic information for risk stratification in pediatric HCM patients.
Background:
Clinical and prognostic role of cardiac magnetic resonance (CMR) in adult population with hypertrophic cardiomyopathy (HCM) have been largely assessed. We sought to investigate the role of CMR for predicting cardiovascular events in children with HCM.
Methods:
CMR was performed in 116 patients with HCM (37 sarcomeric mutations, 31 other mutations, mean age 10.4 ± 4.3 yrs). CMR protocol included cine imaging for evaluation of morphology and function and late gadolinium enhancement (LGE). Hard cardiac events (sustained VT, resuscitated cardiac arrest, sudden cardiac death, end-stage heart failure, heart transplant and appropriate ICD intervention) were recorded through a median follow-up of 4 (1-7) years.
Results:
During follow-up 21 heart cardiac events occurred. At maximal-rank statistic the optimal cut-point for LGE extent for predicting events was ≥2%. Syncope, non-sustained ventricular tachycardia (NSVT) and LGE extent ≥2% were independent predictors of events. At Harrel's C statistic combination of LGE extent ≥2% and syncope was the strongest model for predicting events. HR of patients with LGE extent ≥2% and no history of syncope was 3.6 (1.1-12.2) that increased to 37.6 (5.4-161) in those with LGE extent ≥2% and syncope. The median time dependent AUC of LGE extent (0.88, 95% CI 0.86-0.89) was significantly higher than that of syncope (0.63, 95% CI 0.61-0.66, p < 0.0001) and NSVT (0.52, 95% CI 0.50-0.53, p < 0.0001).
Conclusions:
In children with HCM, LGE and syncope were independent predictors of hard cardiac events at follow-up.
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