Left Atrial Strain and Function in Pediatric Hypertrophic Cardiomyopathy

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.