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左心室肥厚患者左心房应变特征:法布里病、肌节性肥厚型心肌病和心脏淀粉样变性的研究

Characterization of left atrial strain in left ventricular hypertrophy: A study of Fabry disease, sarcomeric hypertrophic cardiomyopathy and cardiac amyloidosis.

作者信息

Massie Charles, Dubé Frédérique, Sridi-Cheniti Soumaya, Ternacle Julien, Lafitte Stéphane, Réant Patricia

机构信息

Bordeaux University Hospital, 33000 Bordeaux, France; University of Bordeaux, 33000 Bordeaux, France; Hôpital Sacré-Cœur de Montréal, Montreal, QC H4J 1C5, Canada.

Bordeaux University Hospital, 33000 Bordeaux, France; University of Bordeaux, 33000 Bordeaux, France; Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, QC J1J 3H5, Canada.

出版信息

Arch Cardiovasc Dis. 2025 Apr;118(4):231-240. doi: 10.1016/j.acvd.2024.12.013. Epub 2025 Feb 26.

DOI:10.1016/j.acvd.2024.12.013
PMID:40069068
Abstract

BACKGROUND

Patients with left ventricular hypertrophy (LVH) often maintain preserved left ventricular ejection fraction in the early stages of the disease. There is a need to identify simple and reliable variables beyond left ventricular ejection fraction to recognize those at risk of developing adverse clinical outcomes.

AIMS

To examine left atrial (LA) strain in patients with hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Fabry disease (FD), pathologies known to cause LVH, and the relationship between LA strain and adverse clinical outcomes.

METHODS

In this retrospective cohort study, LA strain was measured and compared among patients with HCM, CA and FD. Relationships between LA and left ventricular strain, and LA strain and adverse cardiovascular events were evaluated. The primary outcome was first occurrence of cardiovascular mortality, device implantation, heart failure hospitalization, new-onset atrial fibrillation or stroke.

RESULTS

A total of 191 patients were included (24 with FD, 87 with HCM, 80 with CA). LA reservoir strain was highest in patients with HCM (26%, interquartile range [IQR] 20%, 32%), followed by those with FD (20.5%, IQR: 14%, 27.8%) and CA (11%, IQR: 7%, 18.8%) (P<0.001). LA strain correlated well with left ventricular strain in patients with LVH, with CA showing the best correlation (r=-0.70, 95% confidence interval [95% CI]: -0.80 to -0.56; P<0.001). Multivariable Cox regression analysis showed that LA reservoir strain was significantly associated with the primary outcome in all patients (hazard ratio: 0.91, 95% CI: 0.84 to 0.99; P=0.03) and in those with CA (hazard ratio: 0.90, 95% CI: 0.82 to 0.99; P=0.023).

CONCLUSIONS

LA strain was more reduced in CA than in FD and HCM, probably as a result of atrial wall infiltration, and was associated with adverse clinical outcomes in our heterogenous LVH population and patients with CA.

摘要

背景

左心室肥厚(LVH)患者在疾病早期通常保持左心室射血分数正常。需要识别除左心室射血分数之外简单可靠的变量,以识别有发生不良临床结局风险的患者。

目的

研究肥厚型心肌病(HCM)、心脏淀粉样变性(CA)和法布里病(FD)(已知可导致LVH的疾病)患者的左心房(LA)应变,以及LA应变与不良临床结局之间的关系。

方法

在这项回顾性队列研究中,对HCM、CA和FD患者的LA应变进行测量并比较。评估LA与左心室应变之间的关系,以及LA应变与不良心血管事件之间的关系。主要结局是首次发生心血管死亡、植入装置、心力衰竭住院、新发心房颤动或中风。

结果

共纳入191例患者(24例FD患者、87例HCM患者、80例CA患者)。HCM患者的LA储存应变最高(26%,四分位数间距[IQR]:20%,32%),其次是FD患者(20.5%,IQR:14%,27.8%)和CA患者(11%,IQR:7%,18.8%)(P<0.001)。LVH患者中,LA应变与左心室应变相关性良好,CA患者的相关性最佳(r=-0.70,95%置信区间[95%CI]:-0.80至-0.56;P<0.001)。多变量Cox回归分析显示,LA储存应变在所有患者(风险比:0.91,95%CI:0.84至0.99;P=0.03)和CA患者(风险比:0.90,95%CI:0.82至0.99;P=0.023)中均与主要结局显著相关。

结论

CA患者的LA应变比FD和HCM患者降低更明显,可能是心房壁浸润的结果,并且在我们的异质性LVH人群和CA患者中与不良临床结局相关。

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