Journal of the American Society of Echocardiography
Clinical InvestigationEchocardiography in ChildrenLeft Atrial Strain and Function in Pediatric Hypertrophic Cardiomyopathy
Section snippets
Study Population
A retrospective chart review was performed on all pediatric patients with HCM followed at Cleveland Clinic Children's Hospital between May 2010 and December 2018. Seventy-eight patients between 1 and 25 years of age who were diagnosed with HCM according to the American Heart Association/American College of Cardiology guidelines were included. This included ventricular hypertrophy (maximal wall thickness 1.5 cm or ≥2 SDs above the mean [Z score ≥ 2] for age, sex, or body size) in the absence of
Study Population
The HCM cohort consisted of 78 subjects with a median age of 16 years (range, 3–25 years), of whom 60 (64%) were male (Table 1). Phenotypic criteria for HCM were present in 59% patients (P+ group, n = 46) whereas the remaining carried the genotype but lacked phenotypic characteristics (G+P− group, n = 32). The most common reason for diagnosis was screening because of family history (n = 49 [63%]), with murmur being the second most common presentation (n = 17 [22%]). Among the 58 patients with
Discussion
This is the largest study describing conventional and novel parameters of DD in the pediatric HCM population. This study has several important findings emphasizing the importance of LA assessment in pediatric HCM. LAS and volumetric function parameters were significantly lower in patients with phenotypic (P+) HCM compared with those with a positive genotype but without significant hypertrophy (G+P−). LAS had good interobserver and intraobserver reproducibility in this population. Compared with
Conclusion
Our study sheds light on the role of LA function in children with HCM. This is the largest study on assessment of LA mechanics in the pediatric HCM cohort. We demonstrate that altered atrial mechanics, as measured by volumetric and strain analysis, are present even in the pediatric population with phenotypic HCM. The weak but significant correlation of LA conduit function with aerobic capacity may have prognostic implications, although longitudinal data are warranted to determine its value.
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2022, Journal of the American Society of EchocardiographyCitation Excerpt :While there are published normal data by age groups for all parameters, the normal range is very wide. The assessment of left atrial strain and function as well as stiffness analysis by shear wave imaging show promising results in the pediatric population but require further studies and validation.87,88 Recommendations and Key Points
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Conflicts of Interest: None.