Department of Cardiovascular Medicine, Fukushima Medical University, Fukushima, Japan.
Department of Advanced Cardiac Therapeutics, Fukushima Medical University, Fukushima, Japan.
Ann Noninvasive Electrocardiol. 2020 Jul;25(4):e12749. doi: 10.1111/anec.12749. Epub 2020 Feb 21.
Cardiac amyloidosis (CA) is characterized by left ventricular hypertrophy (LVH) and autonomic nervous imbalance due to amyloid infiltration. However, autonomic dysfunction is often seen in heart failure (HF) with LVH from other etiologies. We aimed to characterize autonomic dysfunction in CA from other etiologies of LVH.
Fifty-five HF patients with LVH (35 males, mean age 65 ± 16 years) were enrolled. LVH was defined as left ventricular mass index measured by echocardiography >95 g/m in women and 115 g/m in men. The etiology was as follows: amyloid light chain (AL)-CA, n = 14; hypertrophic cardiomyopathy, n = 21; and aortic stenosis (AS), n = 20. With the patient in a clinically stable condition, heart rate variability (HRV) and heart rate turbulence (HRT), which reflect autonomic dysfunction, were measured using Holter monitoring and compared among the three groups.
Brain natriuretic peptide levels, LVH severity, left ventricular ejection fraction, and tissue Doppler index E/e' did not differ among the three groups. However, severe abnormalities of HRV and HRT were obtained in AL-CA. In the ROC analysis to identify AL-CA in HF with LVH, the best cutoff value for standard deviation of all R-R intervals, standard deviation of the 5-min mean R-R intervals, turbulence onset, and turbulence slope were 68.5 ms (AUC: 0.865), 58.5 ms (AUC: 0.834), 0.25% (AUC: 0.813), and 1.00 ms/RR (AUC 0.736), respectively.
Autonomic dysfunction is a hallmark of AL-CA, and its noninvasive assessment by Holter monitoring may be a useful tool for differential diagnosis of HF with LVH.
心脏淀粉样变（CA）的特征是由于淀粉样物质浸润导致左心室肥厚（LVH）和自主神经失衡。然而，其他病因引起的心力衰竭（HF）伴 LVH 时也常出现自主神经功能障碍。我们旨在描述其他病因引起的 LVH 合并 CA 中的自主神经功能障碍。
共纳入 55 例 HF 伴 LVH 患者（35 名男性，平均年龄 65±16 岁）。LVH 定义为超声心动图测量的左心室质量指数，女性>95g/m，男性>115g/m。病因如下：AL 轻链（AL）-CA，n=14；肥厚型心肌病，n=21；主动脉瓣狭窄（AS），n=20。在患者临床状况稳定的情况下，通过动态心电图监测测量反映自主神经功能障碍的心率变异性（HRV）和心率震荡（HRT），并在三组之间进行比较。
三组患者的脑钠肽水平、LVH 严重程度、左心室射血分数和组织多普勒指数 E/e' 无差异。然而，AL-CA 患者 HRV 和 HRT 严重异常。在识别 HF 伴 LVH 中 AL-CA 的 ROC 分析中，所有 R-R 间期标准差、5 分钟平均 R-R 间期标准差、震荡起始和震荡斜率的最佳截断值分别为 68.5ms（AUC：0.865）、58.5ms（AUC：0.834）、0.25%（AUC：0.813）和 1.00ms/RR（AUC 0.736）。
自主神经功能障碍是 AL-CA 的标志，通过动态心电图监测对其进行无创评估可能是 HF 伴 LVH 鉴别诊断的有用工具。
