Conclusions

Based on the available clinical data it is reasonable to conclude that ICD is the preferred therapy for secondary prevention of sudden cardiac arrest in patients with ARVC/D [1, 22, 23]. When considering ICD for primary prevention, it should be kept in mind that predictive markers of SCD in patients with ARVC/D have not yet been defined in large prospective studies focusing on survival. However, based on the best available clinical data, risk factors that have clinical utility in identifying patients with ARVC/D who are at risk for life-threatening ventricular arrhythmias include induction of VT during electro-physiologic testing, detection of nonsustained VT on noninvasive monitoring, male gender, and severe RV dilation or extensive RV involvement. In addition, young age at presentation, LV involvement, prior cardiac arrest, or unexplained syncope serve as markers of risk [8, 9, 10-14]. Patients with genotypes of ARVC/D associated with a high risk for SCD should be considered for ICD therapy [15,17].

While the role of ICD therapy for primary prevention of sudden death in patients with ischemic heart disease and dilated, nonischemic cardiomy-opathy is well established based on multiple clinical trails with a consistent finding of benefit, the data supporting ICD use in ARVC/D patients are less extensive. In the guidelines of the American College of Cardiology and the American Heart Association, ARVC/D is not mentioned specifically as a standard indication for placement of an ICD [22]. However, familial or inherited conditions with a risk of SCD such as hypertrophic cardiomyopathy and long QT syndromes are acknowledged as Class IIA indications for an ICD [22]. The Task Force on SCD of the European Society of Cardiology proposed that an ICD should be implanted in ARVC/D patients with an increased risk for SCD, based on the presence of a previous cardiac arrest, syncope due to VT, evidence for extensive RV disease, LV involvement, and presentation with polymorphic VT and RV apical aneurysm (which is associated with a genetic locus on chromosome 1q42-43) [23].

Most recently, new guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death were published by the AHA, ACC, and ESC [24]. Based on the growing body of evidence resulting from recently published data, this document provides recommendations for the risk stratification and indication of ICD implantation in patients with ARVC/D.

It is evident that there is not yet a clear consensus on the specific risk factors that identify those patients with ARVC/D in whom the probability of SCD is sufficiently high to warrant an ICD for primary prevention. The results of large, prospective registries of ARVC/D patients with rigorous enrollment criteria in whom ICDs have been placed for primary prevention will give insights into the optimal risk stratification techniques for primary prevention. In the meantime, individualized decisions for primary prevention of SCD must be based on experience, judgment, and the available data. In considering this decision, the clinician should be mindful that, in patients with ARVC/D, ICD has proved safe and reliable in sensing and terminating sustained ventricular arrhythmias. Sudden death is rare in the available clinical series while appropriate ICD therapy is common [8, 9,10-12].

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