Treatment Options

Concerning the treatment of patients with ARVC/D, most of the data available to date refer to retrospective analyses in single centers with limited numbers of patients. There is limited data on long-term outcomes with different treatment modalities in patients with ARVC/D. Controlled randomized trials have not been performed in ARVC/D and international registries [7,8] are currently enrolling patients but have not yet reported results on treatment.

It is difficult to compare patient cohorts from different centers because of differences in patient selection (i.e., referral bias) and application of diagnostic criteria. In addition, treatment strategies may be different in centers or countries with potential changes over time.

Patients with ARVC/D are usually not limited in exercise capacity and frequently participate in sports including athletic competitions. Particularly in early stages of ARVC/D, ventricular arrhythmias and cardiac arrest frequently occur during or immediately after physical exercise or may be triggered by catecholamines [5, 9]. Therefore, antiadrenergic treatment with ^-blockers has been recommended by most authors. Autonomic dysfunction with abnormal adrenergic stimulation of the myocardium and subsequent reduction of ^-adrenoceptor density was demonstrated by quantitative positron emission tomography [9,10] and provided a pathophysiological rationale for these clinical observations and recommendations. With increasing age and more advanced stages of ARVC/D,VT exercise-dependence appears to diminish and also arrhythmias occur more frequently at rest [11].

In recent years, ARVC/D has been of increasing interest in sports medicine, particularly with regard to sports eligibility, preparticipation screening and follow-up evaluation of athletes [12]. Because of the increased risk of sudden death due to the propensity for ventricular arrhythmias and the potential for accelerated disease progression due to the genetically determined damage of mechanical cell contacts [13-15], patients with ARVC/D should be advised against strenuous exercise and vigorous training and should be excluded from participation in competitive or professional athletic sports [16].

In addition to the general management considerations of antiadrenergic therapy and restriction from strenuous exercise and competitive sports, specific therapeutic options in patients with ARVC/D include antiarrhythmic drug therapy, catheter ablation, and the implantation of a cardioverter-defibrillator (ICD). Map-guided antitachycardia surgery using various techniques (localized incisions to total right ventricular disarticulation procedures) has been abandoned by most centers and has been performed in only very few cases with ARVC/D during the past decade. Frequently, more than one treatment modality may be necessary initially or during the long-term course of ARVC/D. Combinations of antiarrhythmic drug therapy with ICD therapy and/or catheter ablation are possible and may reduce arrhythmia related symptoms and prevent sudden death (Fig. 18.1).

The Münster group summarized the first and secondary allocation to different treatment modalities in 200 consecutive index patients with ARVC/D evaluated for treatment between 1977 and 2000 (Fig. 18.2).Al though these results provide useful information on patient management based on clinical decisions, it is important to realize that treatment algorithms have changed over the past decade with an increase of ICD implantations and decrease in the use of antiarrhythmic drugs and catheter ablation in ventricular arrhythmias in general and in patients with ARVC/D in particular.

Fig. 18.1 • Stepwise treatment for ventricular tachycardia (VT) in patients with ARVC/D. Antitachycardia surgery has been generally abandoned due to improvement in catheter ablation techniques and the introduction of transvenous implantable cardioverter defibrillator (ICD) systems

Fig. 18.2 • Treatment allocation of 200 consecutive patients with ARVC/D investigated by the Münster group between 1977 and 2000. AA drugs, antiar-rhythmic drugs; ICD, implantable cardioverter de-fibrillator; PVS, programmed ventricular stimulation; V7, ventricular tachycardia

For clinical decisions, it is therefore important to identify those patients with high risk of sudden death who would benefit most from ICD implantation and separate them from a low-risk cohort in whom a more conservative approach with antiarrhythmic drug therapy appears feasible, safe, and justified. Clinical risk stratification has therefore gained increasing importance in ARVC/D.

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