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Left Atrial Stiffness Is Increased in Pediatric Hypertrophic Cardiomyopathy and Offers Incremental Value in
Ryusuke Numata1, Renzo Calderon-Anyosa2, Sarina Sun1
1Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine Philadelphia PA.
Insights
Left atrial stiffness is a novel echocardiographic marker that predicts major adverse cardiac events (MACE) in children with hypertrophic cardiomyopathy. Increased LA stiffness indicates a higher risk of MACE in this vulnerable pediatric population.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Echocardiography
Background:
- Identifying risk factors for major adverse cardiac events (MACE) is critical in pediatric hypertrophic cardiomyopathy (HCM).
- Left atrial (LA) stiffness shows promise as a surrogate marker in children to assess elevated pulmonary capillary wedge pressure.
Purpose of the Study:
- To evaluate the utility of noninvasive left atrial (LA) stiffness as a predictor of MACE in pediatric patients with hypertrophic cardiomyopathy (HCM).
Main Methods:
- Retrospective analysis of 239 pediatric patients with HCM, divided into MACE (n=79) and non-MACE (n=160) groups.
- Noninvasive LA stiffness calculated as E/e' ratio to peak LA strain via 2D speckle-tracking echocardiography.
- Baseline echocardiograms were used for evaluation before any MACE occurrence.
Main Results:
- The MACE group exhibited significantly reduced peak LA strain and increased LA stiffness compared to the non-MACE group.
- LA stiffness demonstrated superior predictive ability for MACE (C-index, 0.75) and was independently associated with increased MACE risk (HR, 1.12).
- No significant difference in left ventricular ejection fraction was observed between groups.
Conclusions:
- Left atrial (LA) stiffness is a novel echocardiographic index with incremental value in predicting MACE risk in pediatric patients with hypertrophic cardiomyopathy (HCM).
- This finding may aid in risk stratification and management strategies for young patients with HCM.
Background:
Determining risk factors by echocardiography to prevent major adverse cardiac events (MACE) is crucial in pediatric patients with hypertrophic cardiomyopathy. Recent studies have shown that left atrial (LA) stiffness may serve as surrogate marker in children, to differentiate elevated pulmonary capillary wedge pressure from normal.
Methods:
We retrospectively enrolled 239 pediatric patients with hypertrophic cardiomyopathy. They were divided into 2 groups: MACE (n=79) and non-MACE (n=160) groups. Noninvasive LA stiffness was calculated as the ratio of average E/e' to peak LA strain obtained by 2-dimensional speckle-tracking echocardiography. Baseline evaluation was defined as the echocardiogram performed at the time of referral, before the occurrence of a MACE, including appropriate implantable cardioverter-defibrillator intervention, myomectomy, heart transplant, or cardiac death.
Results:
The MACE group showed significantly decreased left ventricular peak longitudinal strain compared with the non-MACE group, with no significant difference in left ventricular ejection fraction. Peak LA strain was significantly reduced (22.5±7.1% versus 30.4±7.3%; P<0.001), whereas LA stiffness was significantly increased (median 0.57 [IQR, 0.37-0.91]%-1 versus 0.31 [IQR, 0.22-0.42]%-1; P<0.001) in the MACE group. LA stiffness was the superior-most index for predicting MACE (C-index, 0.75; cutoff value, 0.31%-1), and even in cross-validation, the C-index consistently demonstrated the highest predictive ability (LA stiffness, 0.746 [95% CI, 0.737-0.753]). Moreover, LA stiffness was independently associated with increased risk of MACE (HR, 1.12 [95% CI, 1.07-1.17]; P<0.001).
Conclusions:
LA stiffness is a newly described index, which may be of incremental value in predicting risk of MACE in pediatric patients with hypertrophic cardiomyopathy.
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