心房右心功能障碍的患病率及其与心脏淀粉样变患者预后的关系。
Prevalence of Right Atrial Impairment and Association with Outcomes in Cardiac Amyloidosis.
机构信息
Noninvasive Cardiac Imaging Laboratories, Section of Cardiology, Heart and Vascular Center, University of Chicago Medicine, Chicago, Illinois.
Section of Cardiology, MedStar Health, Washington, D.C.
出版信息
J Am Soc Echocardiogr. 2022 Aug;35(8):829-835.e1. doi: 10.1016/j.echo.2022.03.022. Epub 2022 Apr 7.
BACKGROUND
Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy in which abnormally folded proteins deposit within the myocardium and the atrial walls. While left atrial dysfunction has been previously reported, the impact of CA on right atrial (RA) structure and function is unknown.
METHODS
We retrospectively studied 118 patients (67 immunoglobulin light chain [AL-CA], 51 transthyretin [ATTR-CA]; age, 70 ± 12 years; 57% men) who underwent transthoracic echocardiogram in sinus rhythm. Right atrial reservoir, conduit, and booster strain were quantified using speckle-tracking and compared between patients with CA and 50 healthy age-, sex-, and race-matched controls using the chi-squared or Mann-Whitney test. The relationship between RA parameters and mortality was assessed using Cox regression.
RESULTS
Right atrial volume was significantly larger in cases with CA compared with in controls: 29 (22-37) vs 21 (15-25) mL/m, P < .001. Right atrial reservoir (21% [14%-35%] vs 37% [34%-43%], P < .001), conduit 11% [18%-6%] vs 14% [11%-17%], P < .001), and booster (10% [17%-5%] vs 23% [20%-27%], P < .001) strains were all significantly more impaired in the CA group compared with controls. Compared with AL-CA, ATTR-CA patients had significantly larger RA volume (34 [26-44] vs 28 [20-35] mL/m, P = .005) and significantly more impaired RA reservoir (17% [10%-30%] vs 27% [17%-37%], P = .007), conduit (8% [13%-6%] vs 13% [20%-8%], P = .031), and booster (7% [14%-4%] vs 11% [18%-6%], P = .030) strain. Among CA patients, RA reservoir (hazard ratio = 0.97 per %, P = .006) and RA conduit (hazard ratio = 1.05 per %, P = .004) were significantly associated with mortality, while RA volume (P = .362) and RA booster strain (P = .180) were not.
CONCLUSIONS
In CA, abnormalities in RA size and strain are highly prevalent and associated with worse prognosis, suggesting the presence of intrinsic RA atriopathy. Right atrial strain appears to be a potentially useful marker in the diagnosis, subtype differentiation, and risk stratification of CA.
背景
心脏淀粉样变性（CA）是一种浸润性心肌病，其中异常折叠的蛋白质在心肌和心房壁内沉积。虽然左心房功能障碍以前已有报道，但 CA 对右心房（RA）结构和功能的影响尚不清楚。
方法
我们回顾性研究了 118 名（67 名免疫球蛋白轻链[AL-CA]，51 名转甲状腺素[ATTR-CA]；年龄 70±12 岁；57%为男性）窦性心律患者，他们接受了经胸超声心动图检查。使用斑点追踪技术对右心房储备、传导和增强应变进行定量，并使用卡方检验或曼-惠特尼检验比较 CA 患者与 50 名年龄、性别和种族匹配的健康对照者之间的右心房参数。使用 Cox 回归评估 RA 参数与死亡率之间的关系。
结果
与对照组相比，CA 患者的右心房容积明显更大：29（22-37）vs 21（15-25）ml/m，P<0.001。右心房储备（21%[14%-35%] vs 37%[34%-43%]，P<0.001）、传导 11%[18%-6%] vs 14%[11%-17%]，P<0.001）和增强应变（10%[17%-5%] vs 23%[20%-27%]，P<0.001）在 CA 组均显著受损。与 AL-CA 相比，ATTR-CA 患者的右心房容积明显更大（34 [26-44] vs 28 [20-35] ml/m，P=0.005），右心房储备明显受损（17%[10%-30%] vs 27%[17%-37%]，P=0.007）、传导（8%[13%-6%] vs 13%[20%-8%]，P=0.031）和增强应变（7%[14%-4%] vs 11%[18%-6%]，P=0.030）。在 CA 患者中，右心房储备（风险比=每增加 1%，P=0.006）和右心房传导（风险比=每增加 1%，P=0.004）与死亡率显著相关，而右心房容积（P=0.362）和右心房增强应变（P=0.180）与死亡率无关。
结论
在 CA 中，RA 大小和应变的异常非常普遍，并与预后不良相关，提示存在固有 RA 心肌病。右心房应变似乎是诊断、亚型分化和 CA 风险分层的一种潜在有用的标志物。
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