6533b858fe1ef96bd12b64d3

RESEARCH PRODUCT

Therapeutic management in Sicilian patients with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy and focus on the role of implantable cardioverter-defibrillator therapy

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description

Arrhythmogenic Right Ventrticular Dysplasia/Cardiomyopathy(ARVD/C) is an inherited cardiomyopathy characterized by right ven-tricularmyocytelosswithfibrofattyreplacement,ahighriskofventric-ular arrhythmias (VA) and sudden cardiac death (SCD) [1]. Preventionof SCD represents the most important management strategy and theachievement of this target can be reached by different therapeuticstrategies including implantable cardioverter-defibrillator (ICD)implantation, pharmacologic therapy, catheter ablation of ventriculartachycardia (VT) and cardiac transplantation [2,3]. The aim of thisstudy is to examine the outcome of the different therapies adopted ina group of affected patients, focusing on the role and predictors of ICDtherapy.We conduced a multicenter study evaluating 28 patients (18 male;age 42 ± 14 years) with definite ARVD/C. Diagnosis of ARVD/C wasbased upon the 2010 revised Task Force Criteria [4] and only patientswith definite diagnosis entered the study to enhance diagnostic speci-ficity. Management therapy was established according to the clinicalfeatures and risk stratification of each patient. All patients were follow-ed up at biannual and yearly intervals and data included invasive andnoninvasive investigation, and device interrogation. The estimate ofthe potential survival benefit of ICD was limited to appropriate ICDshock therapies for episode of VF/VFL, since not any arrhythmic eventsnecessarilycorrespondto thetruearrhythmicriskof death.We studiedour patients over a mean follow-up of 6.0 ± 4.4 years.Antiarrhythmic drugs were used in 26 patients (93%), and in 13 pa-tients (50%) this therapy wasassociated with ICD implantation [Sotalolin11patients(42%)suchasamiodarone;β-blockersin4patient(15%)].During the follow-up 8 patients (61%), that initially received onlyantiarrhythmic drugs, had an ICD implantation cause of the lack ofarrhythmic control. Radiofrequency catheter ablation was performedin 6 patients (21%) and in no cases its efficacy was observed becauseof the recurrence of VT that required ICD implantation in whole cases.Cardiac transplantation was performed as a final therapeutic optiondue to refractory congestive heart failure in 3 patients (10%).At the time of the diagnosis 15 patients (54%) received an ICDimplantation based to the estimated risk of SCD, according with thelast guidelines [5] for management of patients with VA. During thefollow-up 8 patients (61%) received an ICD because of the relapse ofarrhythmic events although they were using antiarrhythmic drugs orunderwent catheter ablation. Twenty patients (86%) had receivedappropriateICDtherapy.AnappropriateICDshockinterventionforven-tricularfibrillation(VF)/ventricular flutter(VFL)wasseenin12patients(52%). Compared with the 100% actual survival rate, VF/VFL-freesurvival rate was 96%, 94% and 51% respectively at 1, 5 and 10 years offollow-up (logrank p b 0.0001) (Fig. 1).The estimated mortality reduc-tion at 1, 5, 10 years of follow-up was 4%, 6% and 49% and the averagerate of ICD interventions for VF/VFL was 5%.Univariatepredictors of ICD shocktherapy werea Holter prematureventricular complex (PVC) count N500/24 h (hazard ratio [HR]: 9.44;95%confidenceinterval[CI]:1.17to15.95;p= 0.03)andagepresenta-tion N44 years (HR: 3.71; 95% CI: 1.04 to 13.19; p = 0.04) (Table 1).Moreover,bothofthemremainedassignificantpredictorsonmultivar-iable analysis. The positive predictive value (PPV) and the negative