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Modeling the Relationship Between Diastolic Phenotype and Outcomes in Pediatric Hypertrophic Cardiomyopathy
Minh B Nguyen1, Maelys Venet2, Chun-Po Steve Fan3
1Department of Pediatric Cardiology, Baylor College of Medicine, Houston, Texas; Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Insights
Diastolic dysfunction in pediatric hypertrophic cardiomyopathy (HCM) is linked to adverse events. Left atrial size, adjusted for body surface area, independently predicts adverse outcomes in these children.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Genetic Cardiology
Background:
- Pediatric hypertrophic cardiomyopathy (HCM) carries a risk of adverse events.
- The role of diastolic dysfunction in pediatric HCM outcomes is not well understood.
- This study investigates the association between diastolic phenotype and adverse events in pediatric HCM.
Purpose of the Study:
- To explore the association between diastolic dysfunction and major adverse cardiac events (MACE) in pediatric patients with HCM.
- To identify key echocardiographic parameters predictive of MACE in this population.
- To understand the contribution of diastolic function to adverse outcomes in pediatric HCM.
Main Methods:
- Principal component analysis of echocardiographic diastolic function parameters in pediatric HCM patients.
- Regression analysis of principal components against freedom from MACE.
- MACE included ICD insertion, myectomy, aborted SCD, transplantation, mechanical support, or death.
Main Results:
- Left atrial size parameters, adjusted for body surface area, were independently associated with MACE (HR, 0.69; 95% CI, 0.5-0.94).
- Left ventricular filling pressure variables showed an association with MACE, but this was not significant after adjusting for left ventricular thickness and genetic variation.
- The final covariate-adjusted model demonstrated strong predictive performance for MACE (concordance index, 0.82).
Conclusions:
- Diastolic dysfunction parameters, left ventricular hypertrophy severity, and genetic variation are associated with MACE in pediatric HCM.
- Left atrial size adjusted for body surface area is an independent predictor of adverse events.
- Further research on size-adjusted diastolic function parameters may improve adverse event prediction in pediatric HCM.
Background:
Pediatric hypertrophic cardiomyopathy (HCM) is associated with adverse events. The contribution of diastolic dysfunction to adverse events is poorly understood. The aim of this study was to explore the association between diastolic phenotype and outcomes in pediatric patients with HCM.
Methods:
Children <18 years of age with diagnosed with HCM were included. Diastolic function parameters were measured from the first echocardiogram at the time of diagnosis, including Doppler flow velocities, tissue Doppler velocities, and left atrial volume and function. Using principal-component analysis, key features in echocardiographic parameters were identified. The principal components were regressed to freedom from major adverse cardiac events (MACE), defined as implantable cardioverter-defibrillator insertion, myectomy, aborted sudden cardiac death, transplantation, need for mechanical circulatory support, and death.
Results:
Variables that estimate left ventricular filling pressures were highly collinear and associated with MACE (hazard ratio, 0.86; 95% CI, 0.75-1.00), though this was no longer significant after controlling for left ventricular thickness and genetic variation. Left atrial size parameters adjusted for body surface area were independently associated with outcomes in the covariate-adjusted model (hazard ratio, 0.69; 95% CI, 0.5-0.94). The covariate-adjusted model had an Akaike information criterion of 213, an adjusted R2 value of 0.78, and a concordance index of 0.82 for association with MACE.
Conclusion:
Echocardiographic parameters of diastolic dysfunction were associated with MACE in this population study, in combination with the severity of left ventricular hypertrophy and genetic variation. Left atrial size parameters adjusted for body surface area were independently associated with adverse events. Additional study of diastolic function parameters adjusted for patient size could facilitate the prediction of adverse events in pediatric patients with HCM.
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