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Published on: October 28, 2020
Patterns of Mechanical Inefficiency in Pediatric Dilated Cardiomyopathy and Their Relation to Left Ventricular
Daniel Forsha1, Cameron Slorach2, Ching Kit Chen3
1Division of Cardiology, Ward Family Heart Center, Children's Mercy Hospital, Kansas City, Missouri.
Insights
Pediatric dilated cardiomyopathy (DCM) shows varied regional mechanical abnormalities, not just global left ventricular (LV) dysfunction. More severe LV discoordination correlates with worse outcomes, suggesting a non-homogeneous disease process.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Echocardiography
Background:
- Pediatric dilated cardiomyopathy (DCM) is a serious condition often leading to heart failure, transplantation, or death.
- Current understanding of DCM primarily focuses on global left ventricular (LV) function, overlooking regional mechanical inefficiencies.
- Regional abnormalities in myocardial contractility and timing may significantly impact LV pumping efficiency and clinical outcomes in pediatric DCM.
Purpose of the Study:
- To define regional strain patterns indicative of mechanical inefficiency in pediatric DCM.
- To categorize these patterns within the context of LV discoordination, encompassing both functional and timing abnormalities.
- To investigate the association between these regional mechanical patterns, LV function, and clinical outcomes in pediatric DCM patients.
Main Methods:
- Two-dimensional longitudinal speckle-tracking strain analysis of LV echocardiographic images from pediatric DCM patients (n=56) and controls (n=20).
- Identification of segmental strain patterns and classification into global contraction groups based on regional contractility and timing.
- Evaluation of clinical outcomes (death/transplantation vs. survival) and echocardiographic parameters, with statistical comparisons between outcome groups.
Main Results:
- 52% of pediatric DCM patients experienced death or transplantation.
- Five distinct segmental strain patterns were identified, defining seven contraction groups characterized by regional discoordination.
- Greater LV discoordination was significantly associated with increased LV dysfunction (P=.0004) and a trend towards higher rates of death or transplantation (P=.069).
- High interreader reproducibility (92%) was achieved for segmental strain pattern analysis.
Conclusions:
- Pediatric DCM exhibits heterogeneous regional wall mechanics leading to inefficient pump function through functional and timing abnormalities.
- These abnormalities can be categorized into distinct subgroups based on regional contractility and timing.
- Increased severity of LV discoordination is linked to more pronounced LV dysfunction and a trend towards poorer clinical outcomes in pediatric DCM.
Background:
Pediatric dilated cardiomyopathy (DCM) is associated with death or transplantation and is typically considered a homogeneous process affecting global left ventricular (LV) function. However, assessment of regional abnormalities that contribute to pumping inefficiencies is lacking. The aim of this study was to define regional strain patterns of mechanical inefficiency in the broader context of LV discoordination (dysfunction and timing abnormalities) and to evaluate their associations with LV function and clinical outcomes.
Methods:
Multiplanar apical LV echocardiographic images from patients with pediatric DCM (n = 56) and control subjects (n = 20) were analyzed by two-dimensional longitudinal speckle-tracking strain analysis to identify segmental strain patterns and global contraction groups. Clinical outcome (death or transplantation vs transplantation-free survival) and echocardiographic data were evaluated. Outcome groups were compared using the Fisher exact test, the χ(2) test, or analysis of variance (with P values ≤ .05 considered to indicate statistical significance).
Results:
Of 56 patients with DCM, 29 (52%) progressed to death or transplantation. Five segmental strain patterns were observed that were used to define seven contraction groups by regional contractility and/or timing discoordination. The group(s) with the most discoordination had the most LV dysfunction (P = .0004) and a trend toward the highest frequency of death or transplantation (P = .069). Interreader reproducibility of segmental strain patterns agreed in 165 or 180 (92%) segments tested (κ = 0.90). Control subjects had normal strain patterns.
Conclusions:
A heterogeneous mixture of abnormalities in the regional wall mechanics that lead to inefficient pump mechanics through functional and timing abnormalities were seen in this cohort and were categorized into natural subgroups. More severe LV discoordination was associated with increased LV dysfunction and a trend toward death or transplantation.
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