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ABSTRACT: Background
Left atrial (LA) enlargement and dysfunction are related to clinical course in patients with hypertrophic cardiomyopathy (HCM). We aimed to investigate genetic contribution to LA structural and functional remodeling.Methods
Two hundred twelve patients were consecutively enrolled, and echocardiography and extensive genetic analysis were performed. Cardiac magnetic resonance (CMR) was performed in 135 patients. Echocardiography was also performed in controls (n?=?30).Results
Patients with HCM had lower late-diastolic mitral annular velocity (a') and higher LA volume index (LAVI) than controls. Patients with pathogenic or likely pathogenic sarcomere gene mutations (PSM, n =?67, 32%) had higher LAVI and lower CMR-derived LA total emptying fraction (37.0?±?18.5 vs. 44.2?±?12.4%, p =?0.025). In patients without AF (n =?187), the PSM had lower a' (6.9?±?2.0 vs. 7.8?±?1.9?cm/s, p =?0.004) than others. The PSM had higher prevalence and amount of late gadolinium enhancement (LGE) in the left ventricle (LV). In multivariate analysis, PSM was significantly related to lower a' independent of E/e', LV mass index, and LAVI. However, the relation significantly attenuated after adjustment for the extent of LGE in the LV, suggesting common myopathy in the LV and LA. In addition, PSM was significantly related to lower LA total emptying fraction independent of age, E/e', s', LV ejection fraction, LV myocardial global longitudinal strain and %LGE mass.Conclusions
PSM was related to LA dysfunction independent of LV filling pressure and LAVI, suggesting its contribution to atrial myopathy in HCM.
SUBMITTER: Chung H
PROVIDER: S-EPMC7789152 | biostudies-literature | 2021 Jan
REPOSITORIES: biostudies-literature
Chung Hyemoon H Kim Yoonjung Y Park Chul Hwan CH Kim In-Soo IS Kim Jong-Youn JY Min Pil-Ki PK Yoon Young Won YW Kim Tae Hoon TH Lee Byoung Kwon BK Hong Bum-Kee BK Rim Se-Joong SJ Kwon Hyuck Moon HM Lee Kyung-A KA Choi Eui-Young EY
Cardiovascular ultrasound 20210106 1
<h4>Background</h4>Left atrial (LA) enlargement and dysfunction are related to clinical course in patients with hypertrophic cardiomyopathy (HCM). We aimed to investigate genetic contribution to LA structural and functional remodeling.<h4>Methods</h4>Two hundred twelve patients were consecutively enrolled, and echocardiography and extensive genetic analysis were performed. Cardiac magnetic resonance (CMR) was performed in 135 patients. Echocardiography was also performed in controls (n = 30).<h4 ...[more]