平板运动试验评价左颈部交感神经节切除术治疗遗传性心律失常的有效性初探

Treatment efficiency evaluation of left cardiac sympathetic denervation for patients with inherited arrhythmia by exercise-stress test

目的分析交感应激诱发恶性室性心律失常的遗传性心律失常患者行左颈部交感神经节切除(LCSD)术前及术后的平板运动试验心电图特点,初步评价LCSD的有效性。方法本研究为观察性研究,回顾性地选取2006年9月至2020年5月在北京清华长庚医院和北京大学人民医院心脏中心行LCSD治疗的儿茶酚胺敏感性多形性室性心动过速(CPVT)和长QT综合征(LQTS)患者,LCSD手术指征为不能耐受β受体阻滞剂或服药后存在心律失常事件。收集患者的临床资料,比较LCSD术前及术后1个月平板运动试验心电图,分析心率及运动耐量、房性及室性心律失常、QTc间期、猝死风险评价指标。收集LCSD术后1、3、6、12个月及此后每年的随访数据,随访内容包括有无心脏事件发生及药物调整。结果共纳入5例遗传性心律失常患者,其中2例为CPVT、1例为LQT1、2例为LQT2。5例患者首发症状均为晕厥,初发症状年龄为12(10,16)岁,行LCSD年龄为21(16,26)岁。5例患者LCSD术后平板运动试验过程中基础心率无明显变化[术前(65.6±6.5)次/min比术后(68.0±11.1)次/min,P=0.57];运动耐量呈下降趋势[术前(12.1±2.8)MET比术后(10.5±2.4)MET,P=0.07]。房性心律失常、室性心律失常的发生均较前减少,最严重室性心律失常评分下降(例序1由术前4分降至术后3分,例序2由术前5分降至3分),3例LQTS患者QTc间期下降[术前基线心率QTc(546.6±72.3)ms比术后(493.0±61.1)ms,P=0.047,术前峰值心率QTc(516.3±73.7)ms比术后(486.7±64.2)ms,P=0.035]。猝死相关指标改善,T波电交替(TWA)呈下降趋势,且恢复阶段1 min内心率变化值(ΔHRR1)由术前(51.5±21.1)次/min降至术后(32.0±13.9)次/min(P=0.035)。随访1(1,4)年,5例患者均规律口服普萘洛尔,剂量调整至(37.0±21.7)mg/d,4例患者无心脏事件,仅1例因情绪激动发生心跳骤停导致心脏性猝死。结论LCSD手术对交感应激引起遗传性心律失常的患者存在一定的治疗作用,且安全性较好。平板运动试验分析初步示LCSD可在不影响心率的前提下,控制恶性心律失常,改善猝死预警指标。

Objective To evaluate the efficiency of left cardiac sympathetic denervation(LCSD)in inherited arrhythmia patients with adrenergic activity-induced malignant ventricular arrhythmia,and observe exercise-stress test features before and after LCSD.Methods This retrospective observational study included catecholaminergic polymorphic ventricular tachycardia(CPVT)and long QT syndromes(LQTS)patients who underwent video-assisted LCSD at Beijing Tsinghua Changgung Hospital and Peking University People's Hospital from September 2006 to May 2020.The indications for LCSD surgery were intolerant or refractory to beta-blocker medication.Clinical and exercise-stress tests data of included patients were collected before and 1 month after LCSD.Heart rate,exercise tolerance,atrial and ventricular arrhythmia,QTc interval and predictors for sudden cardiac death were analyzed.Patents were regularly followed up at 1,3,6,and 12 months after LCSD and then once every year thereafter.Cardiac events and medication adjustment records were collected.Results Five patients(2 CPVT,1 LQT1,and 2 LQT2)were included in the study.All patients experienced syncope as first symptom at the median age of 12(10,16)years,and underwent LCSD at the median age of 21(16,26)years,Baseline heart rate was similar before and after LCSD((65.6±6.5)beats/min vs.(68.0±11.1)beats/min,P=0.57);while maximum workload tended to be lower after LCSD((12.1±2.8)metabolic equivalents(METS)before surgery vs.(10.5±2.4)METS after surgery,P=0.07).Incidence of atrial and ventricular arrhythmia were significantly reduced post LCSD,and the ventricular arrhythmia score was decreased after LCSD in CPVT patients(4 points before LCSD vs.3 points after LCSD in case 1;5 points before LCSD vs.3 points after LCSD in case 2).QTc interval was shortened significantly in three LQTs patients(QTc interval at baseline heart rate:(546.6±72.3)ms before surgery vs.(493±61.1)ms after LCSD,P=0.047;QTc interval at maximal exercise heart rate:(516.3±73.7)ms before surgery vs.(486.7±64.2)ms after LCSD,P=0.035).Additionally,sudden cardiac death risk indicatorΔHRR1(heart rate decreasing value within the first 1 min during recovery phase)decreased from(51.5±21.1)beats/min before surgery to(32.0±13.9)beats/min after surgery(P=0.035).During a median follow-up of 1(1,4)year,all five patients were on low dosage of propranolol(37.0±21.7)mg/d.Cardiac events free survival was achieved in four out of 5 patients(80%)after sympathectomy,while 1 case suffered from sudden cardiac death after emotional stress.Conclusion LCSD surgery can be safely and effectively performed in most hereditary arrhythmia patients with adrenergic activity-induced life-threatening cardiac events.Exercise stress test results show that LCSD could reduce malignant arrhythmias and improve sudden cardiac death risk indicators without decreasing heart rate.