Abstract
Introduction: Hypertrophic cardiomyopathy (HCM) due to mutations in sarcomere genes (G+HCM) has been associated with greater risk of adverse outcomes compared to genetic-test negative HCM (G-HCM). HCM morphologic progression across the age spectrum has not been well-defined in the presence or absence of sarcomere mutations. Hypothesis: We hypothesized that G+ HCM is associated with greater adverse remodeling than G- HCM across the age spectrum of adult HCM. Methods: In a prospectively collected HCM cohort, we studied adult HCM probands who had preserved systolic function (EF >50%). To ascertain morphologic differences with age, we compared the continuous variables of left atrial size/body surface area (LA/BSA) and maximum wall thickness (MWT) by echocardiogram, analyzed by age subgroup and by linear regression. Results: Of 500 HCM probands, 241 were G+ and 259 were G-. MWT was larger for all G+ compared to all G- (19.2mm +/- 6.4mm vs. 16.8mm +/- 4.7mm, p<0.001). By age subgroup, MWT was larger for G+ for ages 20-40 (20.1mm vs 15.7mm, p<0.001) and ages 40-60 (19.4mm vs 16.3mm, p<0.001) but not different for 60+ (17.8mm vs 18.1mm, p=0.8). In linear regression by age, there was no significant change in extent of MWT with age among G+ (p=0.7). In contrast, G- patients exhibited a positive correlation of greater MWT with age (p<0.001), which was significantly different than the age relationship for G+ (p=0.008). LA/BSA was larger for all G+ compared to all G- (21.9mm/m 2 +/- 3.9mm/m 2 versus 20.9mm/m 2 +/- 3.8mm/m 2 , p<0.006). By age subgroup, LA/BSA was not significantly different for G+ compared to G- ages 20-40 (20.9mm/m 2 vs 19.9mm/m 2 , p=0.193) but significantly larger for ages 40-60 (21.6mm/m 2 vs 20.0mm/m 2 , p<0.01) and age 60+ (24.5mm/m 2 vs 22.6mm/m 2 , p=0.02). The regression slopes were similar for G+ vs G- (0.07 mm/m 2 /yr versus 0.09mm/m 2 /yr, p=0.36) but the elevations were significantly larger for G+ (p<0.0001), indicating persistently larger LA size in G+ across the age spectrum. Conclusion: LV hypertrophy extent is similar across adult age groups in G+ HCM, while hypertrophy in G- HCM is milder in age groups <60 and similar to G+ HCM only at age >60. Although LA dilation is progressive with age in all HCM, G+ HCM exhibits greater LA dilation across the adult age spectrum.
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