Prevention of sudden cardiac death by the implantable cardioverter-defibrillator in adults with inherited heart disease. long term follow-up

Abstract

\n \n \n Type of funding sources: None.\n \n \n \n Inherited heart diseases include all inheritable channelopathies or cardiomyopathies. Sudden cardiac death (SCD) might be the first and last clinical presentation. ICD implantation\xa0 is established as a therapeutic tool according to risk criteria.\n \n \n \n The aim of this study is to analyze the population characteristics in ICD/ CRT-ICD recipients in patients with inherited heart disease and establish possible risk predictor factors of arrhytmogenic events during follow-up.\n MÉTHODS: This is a prospective single-center registry. We included all patients from January1, 2012, to December 31, 2020 who subsequently underwent ICD/ ICD-CRT implantation.\n \n \n \n A total of 172 patients were included with a mean age of 60.47\u2009±\u200913,1 years and a mean follow-up duration of 49.71\u2009±\u200941.8 months. The most frequent underlying cardiac condition was dilated cardiomyopathy (58.1%), followed by hypertrophic cardiomyopathy (15.7%), arrythmogenic cardiomyopathy (2.3%), long QT syndrome (1.7%), Brugada syndrome (1.2%), catecholaminergic (2.9%) , idiopathic (7%) and others (11%). 89 ICDs (51.7%) and 83 ICD-CRTs (48.2%) were implanted for primary prevention of SCD.\n During follow-up, 13.4% of the patients experienced arrhythmogenic events. Only 4.1% of the patients experienced inappropriate shock, secondary to supraventricular arrhythmias all of them. Genetic testing was done in only 14.1% of our patients.\n In the 27 cases of hypertrophic cardiomyopathy, 25 (92.6%) underwent ICD implantation for primary prevention. 14 patients (51.9%) had family history of SCD, 8 (29.6%) had unexplained syncope and 16 (59.3%) had ventricular tachycardia in the Holter monitoring. The mean left atrial size was 44.15\u2009±\u20097.2mm, mean maximum LV-wall thickness 22.85\u2009±\u20095,25mm, and a mean LVOT gradient of 30.56\u2009±\u200931.3mmHg, with a mean actual HCM SCD-score of SCD over 5 years of 5.84\u2009±\u20093.53%. According to traditional parameters, 100% of the HCM sample meet criteria for ICD implantation in primary prevention compared to 66.7% according to the new score.\n During follow-up 2 patients (7.4% ) experienced arrhythmogenic events, and 3 patients (11.1%) had inappropriate shocks secondary to supraventricular arrhythmias.\n Genetic testing was done in 22.2% of the patients and was positive for a known pathogenic mutation in half of the cases.\n None of the parameters evaluated in the current score, a high risk of SCD according to it, having old high risk criteria or implanted of ICD in secondary prevention were related in our study with more arrhytmogenic events.\n \n \n \n ICD implatation for primary prevention is the main indication in our population. Either classic factors or the new HCM SCD-score were no related in our study with the presence of arrhytmogenic events during follow-up.\n