关键词: AL, light chain amyloidosis ATTR, transthyretin amyloidosis ATTR-v, variant transthyretin ATTR-wt, wild-type transthyretin AUC, area under the curve BNP, B-type natriuretic peptide CA, cardiac amyloidosis CMR, cardiac magnetic resonance CV, cardiovascular ECG, electrocardiogram LQRSV, low QRS voltages LV, left ventricle LVEDD, left ventricular end-diastolic diameter NAC, National Amyloidosis Centre NT-proBNP, N-terminal pro–B-type natriuretic peptide NYHA, New York Heart Association ROC, receiver-operating characteristic RV, right ventricular TAPSE, tricuspid annulus peak systolic excursion cardiac amyloidosis echocardiography electrocardiography low QRS voltages prognostic significance risk stratification

来  源:   DOI:10.1016/j.jaccao.2022.08.007   PDF(Pubmed)

Abstract:
UNASSIGNED: Low QRS voltages (LQRSVs) are a common electrocardiographic feature in patients with light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR) cardiac amyloidosis (CA).
UNASSIGNED: The aim of this study was to identify clinical and echocardiographic correlates of LQRSV and to investigate their prognostic significance in patients with CA.
UNASSIGNED: This was a multicenter, retrospective study performed in 6 CA referral centers including consecutive patients with AL and ATTR CA. LQRSVs were defined as a QRS amplitude ≤5 mm (0.5 mV) in all peripheral leads. The study outcome was cardiovascular (CV) mortality.
UNASSIGNED: Overall, 411 (AL CA: n = 120, ATTR CA: n = 291) patients were included. LQRSVs were present in 66 (55%) patients with AL CA and 103 (35%) with ATTR CA (P < 0.001). In AL CA, LQRSVs were independently associated with younger age (P = 0.015), higher New York Heart Association functional class (P = 0.016), and natriuretic peptides (P = 0.041); in ATTR CA, LQRSVs were independently associated with pericardial effusion (P = 0.008) and lower tricuspid annulus peak systolic excursion (P = 0.038). During a median follow-up of 33 months (Q1-Q3: 21-46), LQRSVs independently predicted CV death in both AL CA (HR: 1.76; 95% CI: 2.41-10.18; P = 0.031) and ATTR CA (HR: 2.64; 95% CI: 1.82-20.17; P = 0.005). Together with the National Amyloidosis Centre (NAC) staging, LQRSVs provided incremental prognostic value in ATTR CA (AUC for NAC model: 0.83 [95% CI: 0.77-0.89]; AUC for NAC + LQRSV model: 0.87 [95% CI: 0.81-0.93]; P = 0.040).
UNASSIGNED: LQRSVs are common but not ubiquitous in CA; they are more frequent in AL CA than in ATTR CA. LQRSVs reflect an advanced disease stage and independently predict CV death. In ATTR CA, LQRSVs can provide incremental prognostic accuracy over the NAC staging system in patients with intermediate risk.
摘要:
未经证实:低QRS电压(LQRSV)是轻链淀粉样变性(AL)和转甲状腺素蛋白淀粉样变性(ATTR)心脏淀粉样变性(CA)患者的常见心电图特征。
UNASSIGNED:本研究的目的是确定LQRSV的临床和超声心动图相关性,并探讨其在CA患者中的预后意义。
未经评估:这是一个多中心,在6个CA转诊中心进行的回顾性研究,包括连续的AL和ATTRCA患者.LQRSV定义为所有外周导线的QRS振幅≤5mm(0.5mV)。研究结果为心血管(CV)死亡率。
未经评估:总的来说,包括411例(ALCA:n=120,ATTRCA:n=291)患者。66例(55%)ALCA患者和103例(35%)ATTRCA患者存在LQRSV(P<0.001)。在ALCA,LQRSV与年龄无关(P=0.015)。纽约心脏协会功能分级较高(P=0.016),和利钠肽(P=0.041);在ATTRCA中,LQRSV与心包积液(P=0.008)和下三尖瓣环收缩期峰值偏移(P=0.038)独立相关。在33个月的中位随访期间(Q1-Q3:21-46),LQRSV独立预测ALCA(HR:1.76;95%CI:2.41-10.18;P=0.031)和ATTRCA(HR:2.64;95%CI:1.82-20.17;P=0.005)的CV死亡。连同国家淀粉样变性中心(NAC)分期,LQRSV在ATTRCA中提供了递增的预后价值(NAC模型的AUC:0.83[95%CI:0.77-0.89];NAC+LQRSV模型的AUC:0.87[95%CI:0.81-0.93];P=0.040)。
UNASSIGNED:LQRSV在CA中很常见,但并不普遍存在;它们在ALCA中比在ATTRCA中更常见。LQRSV反映晚期疾病阶段并独立预测CV死亡。在ATTRCA,LQRSV可以为中等风险患者的NAC分期系统提供增量的预后准确性。
公众号