PDF(Pubmed)
Abstract：
A novel risk calculator based on clinical characteristics and noninvasive tests that predicts the onset of clinical sustained ventricular arrhythmias (VA) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) has been proposed and validated by recent studies. It remains unknown whether programmed ventricular stimulation (PVS) provides additional prognostic value.
All patients with a definite ARVC diagnosis, no history of sustained VAs at diagnosis, and PVS performed at baseline were extracted from 6 international ARVC registries. The calculator-predicted risk for sustained VA (sustained or implantable cardioverter defibrillator treated ventricular tachycardia [VT] or fibrillation, [aborted] sudden cardiac arrest) was assessed in all patients. Independent and combined performance of the risk calculator and PVS on sustained VA were assessed during a 5-year follow-up period.
Two hundred eighty-eight patients (41.0±14.5 years, 55.9% male, right ventricular ejection fraction 42.5±11.1%) were enrolled. At PVS, 137 (47.6%) patients had inducible ventricular tachycardia. During a median of 5.31 [2.89-10.17] years of follow-up, 83 (60.6%) patients with a positive PVS and 37 (24.5%) with a negative PVS experienced sustained VA (P<0.001). Inducible ventricular tachycardia predicted clinical sustained VA during the 5-year follow-up and remained an independent predictor after accounting for the calculator-predicted risk (HR, 2.52 [1.58-4.02]; P<0.001). Compared with ARVC risk calculator predictions in isolation (C-statistic 0.72), addition of PVS inducibility showed improved prediction of VA events (C-statistic 0.75; log-likelihood ratio for nested models, P<0.001). PVS inducibility had a 76% [67-84] sensitivity and 68% [61-74] specificity, corresponding to log-likelihood ratios of 2.3 and 0.36 for inducible (likelihood ratio+) and noninducible (likelihood ratio-) patients, respectively. In patients with a ARVC risk calculator-predicted risk of clinical VA events <25% during 5 years (ie, low/intermediate subgroup), PVS had a 92.6% negative predictive value.
PVS significantly improved risk stratification above and beyond the calculator-predicted risk of VA in a primary prevention cohort of patients with ARVC, mainly for patients considered to be at low and intermediate risk by the clinical risk calculator.
All patients with a definite ARVC diagnosis, no history of sustained VAs at diagnosis, and PVS performed at baseline were extracted from 6 international ARVC registries. The calculator-predicted risk for sustained VA (sustained or implantable cardioverter defibrillator treated ventricular tachycardia [VT] or fibrillation, [aborted] sudden cardiac arrest) was assessed in all patients. Independent and combined performance of the risk calculator and PVS on sustained VA were assessed during a 5-year follow-up period.
Two hundred eighty-eight patients (41.0±14.5 years, 55.9% male, right ventricular ejection fraction 42.5±11.1%) were enrolled. At PVS, 137 (47.6%) patients had inducible ventricular tachycardia. During a median of 5.31 [2.89-10.17] years of follow-up, 83 (60.6%) patients with a positive PVS and 37 (24.5%) with a negative PVS experienced sustained VA (P<0.001). Inducible ventricular tachycardia predicted clinical sustained VA during the 5-year follow-up and remained an independent predictor after accounting for the calculator-predicted risk (HR, 2.52 [1.58-4.02]; P<0.001). Compared with ARVC risk calculator predictions in isolation (C-statistic 0.72), addition of PVS inducibility showed improved prediction of VA events (C-statistic 0.75; log-likelihood ratio for nested models, P<0.001). PVS inducibility had a 76% [67-84] sensitivity and 68% [61-74] specificity, corresponding to log-likelihood ratios of 2.3 and 0.36 for inducible (likelihood ratio+) and noninducible (likelihood ratio-) patients, respectively. In patients with a ARVC risk calculator-predicted risk of clinical VA events <25% during 5 years (ie, low/intermediate subgroup), PVS had a 92.6% negative predictive value.
PVS significantly improved risk stratification above and beyond the calculator-predicted risk of VA in a primary prevention cohort of patients with ARVC, mainly for patients considered to be at low and intermediate risk by the clinical risk calculator.
摘要：
已经提出了一种基于临床特征和无创测试的新型风险计算器，该计算器可以预测致心律失常性右心室心肌病（ARVC）患者的临床持续性室性心律失常（VA）的发作。程控心室刺激(PVS)是否能提供额外的预后价值仍然未知。
所有明确诊断为ARVC的患者，诊断时无持续性VAs病史,基线时的PVS是从6个国际ARVC注册中心中提取的。持续VA(持续或植入式心律转复除颤器治疗室性心动过速[VT]或纤颤,[中止]心脏骤停）在所有患者中进行评估。在5年的随访期间，评估了风险计算器和PVS对持续VA的独立和综合表现。
二百八十八名患者（41.0±14.5年，55.9%男性,纳入右心室射血分数42.5±11.1％）。在PVS,137例（47.6％）患者患有诱导性室性心动过速。在5.31[2.89-10.17]年的中位随访期间，83例（60.6%）PVS阳性患者和37例（24.5%）PVS阴性患者经历了持续性VA（P<0.001）。诱导型室性心动过速在5年的随访中预测了临床持续的VA，并且在考虑了计算器预测的风险（HR，2.52[1.58-4.02];P<0.001)。与孤立的ARVC风险计算器预测(C统计量0.72)相比，增加PVS诱导性显示对VA事件的预测有所改善(C统计量0.75；嵌套模型的对数似然比，P<0.001)。PVS诱导性有76%[67-84]的敏感性和68%[61-74]的特异性,对应于对数似然比2.3和0.36诱导（似然比+）和非诱导（似然比-）患者，分别。在ARVC风险计算器预测的患者中，5年内临床VA事件的风险<25％（即，低/中子群)，PVS的阴性预测值为92.6%。
在ARVC患者的一级预防队列中，PVS显着改善了高于和超出计算器预测的VA风险的风险分层，主要针对临床风险计算器认为处于低风险和中等风险的患者。
所有明确诊断为ARVC的患者，诊断时无持续性VAs病史,基线时的PVS是从6个国际ARVC注册中心中提取的。持续VA(持续或植入式心律转复除颤器治疗室性心动过速[VT]或纤颤,[中止]心脏骤停）在所有患者中进行评估。在5年的随访期间，评估了风险计算器和PVS对持续VA的独立和综合表现。
二百八十八名患者（41.0±14.5年，55.9%男性,纳入右心室射血分数42.5±11.1％）。在PVS,137例（47.6％）患者患有诱导性室性心动过速。在5.31[2.89-10.17]年的中位随访期间，83例（60.6%）PVS阳性患者和37例（24.5%）PVS阴性患者经历了持续性VA（P<0.001）。诱导型室性心动过速在5年的随访中预测了临床持续的VA，并且在考虑了计算器预测的风险（HR，2.52[1.58-4.02];P<0.001)。与孤立的ARVC风险计算器预测(C统计量0.72)相比，增加PVS诱导性显示对VA事件的预测有所改善(C统计量0.75；嵌套模型的对数似然比，P<0.001)。PVS诱导性有76%[67-84]的敏感性和68%[61-74]的特异性,对应于对数似然比2.3和0.36诱导（似然比+）和非诱导（似然比-）患者，分别。在ARVC风险计算器预测的患者中，5年内临床VA事件的风险<25％（即，低/中子群)，PVS的阴性预测值为92.6%。
在ARVC患者的一级预防队列中，PVS显着改善了高于和超出计算器预测的VA风险的风险分层，主要针对临床风险计算器认为处于低风险和中等风险的患者。
