Key result
Left atrial total emptying function was significantly decreased in both preclinical (64%) and overt hypertrophic cardiomyopathy (59%) sarcomere mutation carriers compared with healthy controls (70%).
Cross-Sectional (n=73)
Yes
Absolute Event Rate: 59% vs 70%
p-value: p=0.005
Left atrial dysfunction is detectable by CMR in preclinical HCM mutation carriers even before the development of left ventricular hypertrophy or left atrial enlargement.
Left atrial dysfunction in mutation carriers is hypothesis-generating; prospective studies needed before considering early intervention.
BACKGROUND: Impaired left atrial (LA) function is an early marker of cardiac dysfunction and predictor of adverse cardiac events. Herein, we assess LA structure and function in hypertrophy in hypertrophic cardiomyopathy (HCM) sarcomere mutation carriers with and without left ventricular hypertrophy (LVH). METHOD: Seventy-three participants of the HCMNet study who underwent cardiovascular magnetic resonance (CMR) imaging were studied, including mutation carriers with overt HCM (n = 34), preclinical mutation carriers without HCM (n = 24) and healthy, familial controls (n = 15). RESULTS: LA volumes were similar between preclinical, control and overt HCM cohorts after covariate adjustment. However, there was evidence of impaired LA function with decreased LA total emptying function in both preclinical (64 ± 8%) and overt HCM (59 ± 10%), compared with controls (70 ± 7%; p = 0.002 and p = 0.005, respectively). LA passive emptying function was also decreased in overt HCM (35 ± 11%) compared with controls (47 ± 10%; p = 0.006). Both LAtotal emptying function and LA passive emptying function were inversely correlated with the extent of late gadolinium enhancement (LGE; p = 0.005 and p < 0.05, respectively), LV mass (p = 0.02 and p < 0.001) and interventricular septal thickness (p < 0.001 for both) and serum NT-proBNP levels (p < 0.001 for both). CONCLUSION: LA dysfunction is detectable by CMR in preclinical HCM mutation carriers despite non-distinguishable LV wall thickness and LA volume. LA function appears most impaired in subjects with overt HCM and a greater extent of LV fibrosis.
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Farhad et al. (2016) conducted a cross-sectional in Hypertrophic cardiomyopathy (HCM) (n=73). Sarcomere mutation (preclinical and overt HCM) vs. Healthy familial controls was evaluated on Left atrial total emptying function (LATEmF) (p=0.005). Left atrial total emptying function was significantly decreased in both preclinical (64%) and overt hypertrophic cardiomyopathy (59%) sarcomere mutation carriers compared with healthy controls (70%).
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