Classic-pattern dyssynchrony and electrical activation delays in pediatric dilated cardiomyopathy
Daniel Forsha1, Cameron Slorach2, Ching Kit Chen2
1Division of Pediatric Cardiology, Duke University Medical Center, Durham, North Carolina.
Insights
Classic-pattern dyssynchrony (CPD) was found in 12% of pediatric dilated cardiomyopathy (DCM) patients, indicating potential for cardiac resynchronization therapy (CRT) response. This finding is crucial for identifying pediatric DCM candidates for CRT.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Electrophysiology
Background:
- Pediatric dilated cardiomyopathy (DCM) often leads to heart failure, transplantation, or death, with limited treatment options.
- Cardiac resynchronization therapy (CRT) shows promise for some children with DCM, but predicting response remains a challenge.
- Identifying potential CRT candidates is vital given the high mortality in pediatric DCM.
Purpose of the Study:
- To investigate the presence of classic-pattern dyssynchrony (CPD) in pediatric DCM patients.
- To determine if CPD is associated with electrical activation delays in this population.
- To assess the potential of CPD as a predictor of CRT response in pediatric DCM.
Main Methods:
- Retrospective analysis of echocardiograms from 59 pediatric DCM patients using 2D speckle-tracking strain analysis for CPD.
- Evaluation of electrocardiograms for activation delays, including prolonged QRS duration and left bundle branch block (LBBB).
- Comparison with 40 age-matched control subjects without cardiac disease.
Main Results:
- CPD was identified in 7 out of 59 (12%) pediatric DCM subjects; none were found in controls.
- Prolonged QRS durations were observed in 22% of patients, with 2 meeting strict LBBB criteria.
- Six of the seven CPD subjects exhibited prolonged QRS durations, suggesting an association between mechanical and electrical dyssynchrony.
Conclusions:
- A small subgroup of pediatric DCM patients exhibits CPD, which may indicate potential benefit from CRT.
- CPD is associated with electrical activation delays, though not exclusively with strict LBBB.
- These findings support further prospective evaluation of CPD for guiding CRT in pediatric DCM.
Background:
Progressive heart failure leading to transplantation or death is common in pediatric dilated cardiomyopathy (DCM), and treatment options are limited. Select children with DCM have improved after cardiac resynchronization therapy (CRT), but predicting response is challenging. Nonetheless, considering the frequency of death or transplantation in this population, identifying any candidate would be valuable. Classic-pattern dyssynchrony (CPD) identifies mechanical dyssynchrony patterns consistent with underlying electrical activation delays and strongly predicts CRT response in adult DCM but has not been evaluated in pediatric DCM. The aim of this study was to test the hypothesis that CPD is present in a subgroup of patients with pediatric DCM and is associated with activation delays.
Methods:
Fifty-nine subjects with pediatric DCM (left ventricular end-diastolic diameter Z score > 2 and left ventricular ejection fraction < 40%) who underwent echocardiography with a functional protocol with apical images optimized for two-dimensional speckle-tracking strain analysis (EchoPAC) were retrospectively analyzed for CPD. Electrocardiograms were evaluated for activation delays (prolonged QRS duration and strict criteria for left bundle branch block [LBBB]). Forty control subjects with no cardiac disease and good imaging widows were also analyzed.
Results:
The mean age was 5.4 years (range, 1 day to 20 years); idiopathic DCM was most common (57%). Severe cardiomyopathy was present in 75% (end-diastolic diameter Z score > 4.6 and left ventricular ejection fraction < 32%). CPD was identified in seven subjects (12%), and prolonged QRS durations were present in 13 (22%), but only two subjects met strict criteria for LBBB. Six of seven subjects in the CPD group had prolonged QRS durations, and two of seven had LBBB. No control subjects had CPD. The CPD analysis was highly feasible and reproducible.
Conclusions:
In this severely affected cohort, the small CPD subgroup is potentially important because their progressive disease may respond to CRT. CPD is associated with activation delays, although not necessarily strict LBBB. This has important potential implications for prospective evaluation of CRT in this disease.
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