Long-term Follow-up Study Of ICD Implantation In Patients With Hereditary Sudden Death Heart Disease

Objectives: Hereditary sudden death heart disease mainly includes hereditary cardiomyopathy and cardiac ion channel disease,which may lead to malignant arrhythmias and sudden cardiac death(SCD).Implantable cardioverter defibrillator(ICD)is the most effective method to prevent SCD.At present,a large number of clinical studies at home and abroad have proved the safety and effectiveness of implantable ICD for SCD prevention in people with different diseases,including coronary heart disease,myocardial infarction,revascularization,heart failure,nonischemic cardiomyopathy,dilated cardiomyopathy,etc.This study conducted a followup study on patients with hereditary sudden death heart disease who received ICD.Clinical data of patients with hereditary sudden death heart disease who received ICD were collected and long-term follow-up was conducted,including the occurrence of ventricular arrhythmias,treatment of ICD,ICD-related complications,etc.To analyze the prognosis of patients with hereditary sudden death heart disease with ICD implantation for primary or secondary prevention of SCD,and to explore the factors that may influence the prognosis.Methods: This is a single-center,retrospective,observational clinical study.Hereditary sudden death heart disease(Brugada syndrome,congenital Long QT syndrome,arrhythmogenic right ventricular cardiomyopathy,hypertrophic cardiomyopathy,familial dilated cardiomyopathy and other familial sudden death heart diease)that was first implanted with ICD in the Department of Cardiology,the First Affiliated Hospital of Dalian Medical University from February 2010 to October 2022 for primary or secondary prevention of SCD were included Family sudden death heart disease).Baseline data,ICD-related complications,ICD-programmed data(ventricular arrhythmia episode and treatment),cardiovascular rehospitalization,cardiovascular death,and all-cause death were collected.The end points of follow-up were: 1.Ventricular fibrillation(VF)and ventricular tachycardia(VT)correctly identified by ICD;2.All causes of death.The patients were divided into positive event group and negative event group according to the occurrence of endpoint events.Survival analysis was used to analyze the influence of each factor on the prognosis of ICD implantation.Univariate analysis was performed by Kaplan-Meier method(Log-Rank test),and multivariate analysis was performed by Cox regression.Results: A total of 82 patients were initially included in this study,including 6patients with missing follow-up data and 3 patients who were lost to follow-up.Finally,73 patients were followed up from February 11,2010 to December 31,2022.There were 33 cases in the positive event group and 40 cases in the negative event group.There were significant differences in primary prevention(P=0.029),hypertrophic cardiomyopathy(P=0.015),syncope history(P=0.017),cardiac arrest history(P=0.032),VF history(P=0.042),persistent VT history(P=0.009)and QTc(P=0.037)between the two groups(P < 0.05).During follow-up,7 patients(9.6%)developed ICD-related complications,and there was no significant difference in the incidence of ICD-related complications between the two groups(P=0.286).Cardiovascular re-hospitalization occurred in 22 patients(30.1%),and there was no significant difference in the rate of cardiovascular re-hospitalization between the two groups(P=0.292).After KaplanMeier univariate analysis,patients with secondary prevention,arrhythmogenic right ventricular cardiomyopathy,non-hypertrophic cardiomyopathy,cardiac arrest history,VF history,persistent VT history,tachyventricular fibrillation electrical storm history,and prolonged QTc had shorter and faster onset of VT,VF,or death events after ICD implantation.After Cox regression multivariate analysis,VF history(HR=5.253,95%CI 2.118 to 13.031,P < 0.001),prolonged QTc(HR=2.760,95%CI 1.243 to 6.127,P=0.013),arrhythmogenic right ventricular cardiomyopathy(HR=5.065,95%CI 2.127 to 12.064,P < 0.001)were independent influencing factors of VT,VF or death in hereditary sudden death heart disease patients after ICD implantation,and were risk factors,that is,after adjusting for the other two factors,the risk of VT,VF or death in patients with VF history was 5.253 times higher than that in patients without VF history.The risk of VT,VF or death in patients with prolonged QTc was 2.760 times that of patients without prolonged QTc,and the risk of VT,VF or death in patients with ARVC was 5.065 times that of patients without prolonged QTc.Conclusions: In patients with hereditary sudden death heart disease implanted with ICD for SCD prophylaxis,patients with secondary prophylaxis,arrhythmogenic right ventricular cardiomyopathy,non-hypertrophic cardiomyopathy,a history of cardiac arrest,VF,a history of persistent VT,a history of ventricular tachycardia and an electrical storm of ventricular fibrillation,and prolonged QTc had shorter and faster onset of VT,VF,or death.The clinical benefits of implantation of ICD are even greater.VF history,arrhythmogenic right ventricular cardiomyopathy,and prolonged QTc are risk factors for VT,VF,or death after ICD implantation in patients with hereditary sudden death heart disease.Therefore,ICD implantation is more actively recommended to prevent SCD in patients with hereditary sudden death heart disease with VF history,arrhythmogenic right ventricular cardiomyopathy,and prolonged QTc.