背景:心脏淀粉样变性(CA)是心力衰竭的公认原因。左心房(LA)肌病导致心力衰竭的预后较差,并且是转甲状腺素蛋白(ATTR)和轻链(AL)CA的特征。LA机械离散度(LA-MD)是与LA肌病和房颤(AF)的未来发展有关的心房内不同步的新型标志物。
目的:本研究旨在通过全面的LA超声心动图评估,确定ATTR和AL心肌病中LA肌病的特征和预后价值。
方法:将ATTR(n=86)和AL(n=86)CA患者与高血压性心脏病(HHT)患者(n=58)进行比较。经胸超声心动图测量,包括LA应变和LA-MD,并对患者进行死亡率随访。
结果:ATTR和AL患者的中位随访时间为66个月,26例死亡事件。左心室(LV)质量,舒张功能(平均e'和E/e'),低压全球纵向应变,与AL相比,ATTR组的LA体积和功能(LA功能指数和应变)受损更多;与HHT患者相比,两个淀粉样蛋白组的这些超声心动图参数受损更多(P<0.05)。ATTR与AL的LA-MD增加[中位数72.2(四分位数间范围55-88.9)与54(43.5-64.2),分别,P<0.001]。多变量逻辑回归调整年龄,AF的存在,低压质量,全球和基础菌株,E/e表明LA-MD是ATTRCA的独立决定因素(P=0.014)。在多变量分析中,在CA组中,LA储层应变与心力衰竭的存在独立相关(P<0.001)。LA最小体积(截止值≥18mL/m2)是ALCA死亡率的决定因素[Cox比例风险比(HR)1.042(1.003-1.082),P=0.034和Kaplan-Meier分析,P=0.016]。
结论:表征LA肌病在CA中具有重要的诊断和预后价值。ATTR患者心房不同步增加,这可能会对房颤的发展产生影响。LA储层劳损与CA的心力衰竭有关,而LA最小体积是ALCA死亡率的预测因子。
BACKGROUND: Cardiac amyloidosis (CA) is an under-recognized cause of heart failure. Left atrial (LA) myopathy contributes to a worse prognosis in heart failure and is a feature of transthyretin (ATTR) and light-chain (AL) CA. LA mechanical dispersion (LA-MD) is a novel marker of intra-atrial dyssynchrony implicated in LA myopathy and the future development of atrial fibrillation (AF).
OBJECTIVE: This study aimed to determine the characteristics and prognostic value of LA myopathy in ATTR and AL cardiomyopathy through a comprehensive LA echocardiographic evaluation.
METHODS: ATTR (n = 86) and AL (n = 86) CA patients were compared with hypertensive heart disease (HHT) patients (n = 58). Transthoracic echocardiographic measurements including LA strain and LA-MD were obtained with patient follow-up for mortality.
RESULTS: ATTR and AL patients had a median follow-up of 66 months, with 26 mortality events. Left ventricular (LV) mass, diastolic function (average-e\' and E/e\'), LV global longitudinal strain, and LA volume and function (LA function index and strain) were more impaired in ATTR versus AL; these echocardiographic parameters were more impaired in both amyloid groups compared to HHT patients (P < 0.05). LA-MD was increased in ATTR versus AL [median 72.2 (inter-quartile range 55-88.9) vs. 54 (43.5-64.2), respectively, P < 0.001]. Multivariable logistic regression adjusted for age, presence of AF, LV mass, global and basal strain, and E/e\' demonstrated that LA-MD was an independent determinant of ATTR CA (P = 0.014). On multivariable analysis, LA reservoir strain was independently associated with the presence of heart failure in the CA group (P < 0.001). LA minimum volume (cut-off ≥18 mL/m2) was a determinant of mortality in AL CA [Cox proportional hazard ratio (HR) 1.042 (1.003-1.082), P = 0.034 and Kaplan-Meier analysis, P = 0.016].
CONCLUSIONS: Characterizing LA myopathy has significant diagnostic and prognostic utility in CA. ATTR patients have increased atrial dyssynchrony, which may have implications for AF development. LA reservoir strain was associated with heart failure in CA, whilst LA minimum volume was a predictor of mortality in AL CA.