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Published on: July 20, 2022
Left Atrial Strain and Function in Pediatric Hypertrophic Cardiomyopathy
Simone Jhaveri1, Rukmini Komarlu2, Sarah Worley3
1Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, Ohio; Department of Pediatric Cardiology, Cohen Children's Medical Center of New York, Zucker School of Medicine at Hofstra, New Hyde Park, New York.
Insights
Pediatric patients with hypertrophic cardiomyopathy (HCM) and ventricular hypertrophy show reduced left atrial (LA) function. This impaired LA mechanics correlates with diminished exercise capacity, highlighting its clinical significance.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Echocardiography
Background:
- Left atrial (LA) strain and dysfunction are early indicators of diastolic dysfunction and are linked to reduced exercise capacity in adults with hypertrophic cardiomyopathy (HCM).
- Limited research exists on assessing LA mechanics in pediatric HCM patients.
- This study aimed to evaluate LA strain and function in pediatric HCM patients with (P+) and without (G+P-) ventricular hypertrophy and correlate findings with exercise stress test results.
Purpose of the Study:
- To assess left atrial (LA) strain and function in pediatric patients with hypertrophic cardiomyopathy (HCM).
- To compare LA mechanics between pediatric HCM patients with (phenotype positive [P+]) and without (genotype positive, phenotype negative [G+P-]) ventricular hypertrophy.
- To evaluate the correlation between LA function and exercise stress test parameters in pediatric HCM.
Main Methods:
- Retrospective analysis of 78 children (3-25 years) with HCM (46 P+, 32 G+P-) and 20 healthy controls.
- Phasic LA volumetric analysis to compute LA conduit, reservoir, and pump function.
- Speckle-tracking echocardiography for LA reservoir strain (LASr) and LA contractile strain measurement; exercise test data collected within 12 months.
Main Results:
- Patients with P+ HCM exhibited significantly lower LA conduit function (36% vs 48%) and LA reservoir function (137% vs 180%) compared to G+P- patients.
- LA contractile function did not differ significantly between the groups (31% vs 32%).
- P+ HCM patients showed reduced four-chamber LASr (29% vs 41%), two-chamber LASr (30% vs 41%), average LASr (29% vs 42%), and LA contractile strain (9% vs 12%) compared to G+P- patients. LA conduit function weakly correlated with aerobic capacity (r=0.42, P=0.019) in patients who underwent stress testing.
Conclusions:
- Children with P+ HCM demonstrate impaired LA function, detectable through both volumetric and strain analyses.
- Altered LA mechanics in pediatric HCM are associated with reduced exercise capacity.
- This study provides a basis for evaluating novel LA parameters in pediatric HCM and suggests the need for larger longitudinal studies to determine clinical significance.
Background:
Left atrial (LA) strain and dysfunction are early markers of diastolic dysfunction, associated with poor exercise capacity in adults with hypertrophic cardiomyopathy (HCM). Literature on assessment of LA mechanics in pediatric HCM is lacking. The aim of this study was to assess LA strain and LA function in pediatric patients who have HCM with (phenotype positive [P+]) and without (genotype positive, phenotype negative [G+P-]) ventricular hypertrophy and evaluate their correlation with exercise stress test parameters.
Methods:
Seventy-eight children (3-25 years of age) with HCM (P+, n = 46; G+P-, n = 32) and 20 healthy control subjects were retrospectively studied. LA conduit function, reservoir function, and pump function were computed using phasic LA volumetric analysis. LA reservoir strain (LASr) and LA contractile strain were measured using speckle-tracking echocardiography. Exercise test findings within 12 months of echocardiography were recorded.
Results:
LA conduit function (36% vs 48%, P < .001) and LA reservoir function (137% vs 180%, P < .001) were lower in P+ than in G+P- patients. LA contractile function did not differ between the groups (31% vs 32%, P = .87). Compared with patients with G+P- HCM, those with P+HCM had lower four-chamber LASr (29% vs 41%, P < .001), two-chamber LASr (30% vs 41%, P < .001), average LASr (29% vs 42%, P < .001), and LA contractile strain (9% vs 12%, P = .016). In the cohort of patients with HCM who underwent stress testing (n = 35), LA conduit function weakly correlated with aerobic capacity (r = 0.42, P = .019).
Conclusions:
Children with P+HCM have reduced LA function, measurable by both volumetric and strain analysis. Altered LA mechanics are associated with poor exercise capacity. This study lays the foundation for the evaluation of novel LA parameters in pediatric HCM and warrants larger longitudinal studies to assess its clinical significance.
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