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Contribution of sarcomere gene mutations to left atrial function in patients with hypertrophic cardiomyopathy

Authors
 Hyemoon Chung  ;  Yoonjung Kim  ;  Chul Hwan Park  ;  In-Soo Kim  ;  Jong-Youn Kim  ;  Pil-Ki Min  ;  Young Won Yoon  ;  Tae Hoon Kim  ;  Byoung Kwon Lee  ;  Bum-Kee Hong  ;  Se-Joong Rim  ;  Hyuck Moon Kwon  ;  Kyung-A Lee  ;  Eui-Young Choi 
Citation
 CARDIOVASCULAR ULTRASOUND, Vol.19(1) : 4, 2021-01 
Journal Title
CARDIOVASCULAR ULTRASOUND
Issue Date
2021-01
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.
Files in This Item:
T202100178.pdf Download
DOI
10.1186/s12947-020-00233-y
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Laboratory Medicine (진단검사의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Kwon, Hyuck Moon(권혁문) ORCID logo https://orcid.org/0000-0001-9901-5015
Kim, Yoon Jung(김윤정) ORCID logo https://orcid.org/0000-0002-4370-4265
Kim, In-Soo(김인수) ORCID logo https://orcid.org/0000-0003-2801-5514
Kim, Jong Youn(김종윤) ORCID logo https://orcid.org/0000-0001-7040-8771
Kim, Tae Hoon(김태훈) ORCID logo https://orcid.org/0000-0003-3598-2529
Min, Pil Ki(민필기) ORCID logo https://orcid.org/0000-0001-7033-7651
Park, Chul Hwan(박철환) ORCID logo https://orcid.org/0000-0002-0004-9475
Yoon, Young Won(윤영원) ORCID logo https://orcid.org/0000-0002-0907-0350
Lee, Kyung A(이경아) ORCID logo https://orcid.org/0000-0001-5320-6705
Lee, Byoung Kwon(이병권) ORCID logo https://orcid.org/0000-0001-9259-2776
Rim, Se Joong(임세중) ORCID logo https://orcid.org/0000-0002-7631-5581
Choi, Eui Young(최의영) ORCID logo https://orcid.org/0000-0003-3732-0190
Hong, Bum Kee(홍범기) ORCID logo https://orcid.org/0000-0002-6456-0184
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/182005
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