目的:在轻链心脏淀粉样变性(AL-CA)中通常观察到室性早搏(PVC)和非持续性室性心动过速(NSVT),但其与预后的关系尚不清楚.我们旨在评估中晚期AL-CA患者PVCs和NSVT的预后价值。
方法:我们回顾性纳入了2014年2月至2020年12月间改良的2004年MayoII-IIIb期AL-CA患者。入院时评估24小时Holter记录。结果包括1)新发的不良室性心律失常(VA)或SCD和2)随访期间的心源性死亡。
结果:在研究的143名患者中(60.4±11.1年,男性64.3%),132(92.3%)在Holter上存在PVC,50(35.0%)在Holter上存在NSVT。12例(8.4%)患者在医院死亡,131例患者获得随访(中位数为24.4个月),其中71例患者有心脏死亡,15例患者行不良VA/SCD。NSVT[HR:13.57,95%CI:3.06-60.18,p<0.001],对数转化的PVC计数(HR:1.46,95CI:1.15-1.86,p=0.002)和PVC负荷(HR:1.4395CI:1.14-1.80,p=0.002)可预测不良VA/SCD的新发作。PVC计数的最高三分位数(HR:2.33,95CI:1.27-4.28,p=0.006)和PVC负担(HR:2.58,95CI:1.42-4.69,p=0.002),而不是NSVT(HR:1.16,95CI:0.67-1.98,p=0.603),与心脏死亡有关。较高的PVC计数/负担为预测心脏死亡的2004年Mayo改良阶段提供了增量价值,C指数分别从0.681增加到0.712和0.717(p值<0.05)。
结论:PVC计数,在AL-CA患者随访期间,负荷量和NSVT与不良VA/SCD显著相关。更高的PVC计数/负担增加了预测心脏死亡的增量价值。
OBJECTIVE: Premature ventricular contractions (PVC) and non-sustained ventricular tachycardia (NSVT) are commonly observed in light chain cardiac amyloidosis (AL-CA), but their association with prognosis is still unclear. We aimed to evaluate the prognostic value of PVCs and NSVT in patients with moderate-to-advanced AL-CA.
RESULTS: We retrospectively included patients with AL-CA at modified 2004 Mayo stages II-IIIb between February 2014 and December 2020. Twenty-four-hour Holter recordings were assessed on admission. The outcomes included (i) new onset of adverse ventricular arrhythmia (VA) or sudden cardiac death (SCD) and (ii) cardiac death during follow-up. Of the 143 patients studied (60.41 ± 11.06 years, male 64.34%), 132 (92.31%) had presence of PVC, and 50 (34.97%) had NSVT on Holter. Twelve (8.4%) patients died in hospital and 131 patients were followed up (median 24.4 months), among whom 71 patients had cardiac death, and 15 underwent adverse VA/SCD. NSVT [hazard ratio (HR): 13.57, 95% confidence interval (CI): 3.06-60.18, P < 0.001], log-transformed PVC counts (HR: 1.46, 95%CI: 1.15-1.86, P = 0.002) and PVC burden (HR: 1.43 95%CI:1.14-1.80, P = 0.002) were predictive of new onset of adverse VA/SCD. The highest tertile of PVC counts (HR: 2.33, 95%CI: 1.27-4.28, P = 0.006) and PVC burden (HR: 2.58, 95%CI: 1.42-4.69, P = 0.002), rather than NSVT (HR: 1.16, 95%CI: 0.67-1.98, P = 0.603), was associated with cardiac death. Higher PVC counts/burden provided incremental value on modified 2004 Mayo stage in predicting cardiac death, with C index increasing from 0.681 to 0.712 and 0.717, respectively (P values <0.05).
CONCLUSIONS: PVC count, burden, and NSVT significantly correlated with adverse VA/SCD during follow-up in patients with AL-CA. Higher PVC counts/burdens added incremental value for predicting cardiac death.