Management of Symptomatic Patients

In patients with ARVC/D and no history of syncope or cardiac arrest, the presence of premature ventricular beats, couplets, or short ventricular runs is usually not associated with an increased arrhythmic risk and therefore do not require specific antiarrhythmic treatment. In many cases, reassurance of the patient results in an improvement of symptoms. However, should a patient still have severe symptoms due to palpitations, treatment with conventional ^-blockers or verapamil may be considered. ^-blockers appear to be more effective in patients with exercise-provocable ventricular arrhythmias, whereas verapamil may be more successful for the treatment of arrhythmias that occur at rest and are suppressed during exercise. Specific antiarrhythmic drugs or catheter ablation should be limited to patients with significant symptoms refractory to these measures.

Patients with nonsustained and sustained VT or syncope should undergo a detailed diagnostic evaluation to stratify risk and to assess inducibility of clinical ventricular arrhythmia, both having an impact on the subsequent treatment strategy. In low-risk patients, antiarrhythmic drug therapy (preferentially sotalol) may be considered and should be guided by serial electrophysiologic studies. In our experience, this approach showed favorable long-term results in selected low-risk patients with low rates of VT recurrence and sudden death. Catheter ablation may be an alternative option in patients with localized ARVC/D and a single morphology of a hemody-namically well-tolerated VT refractory to antiarrhythmic drugs. In patients with drug-refractory frequent or incessant VT, catheter ablation may be the only treatment option available, but is usually palliative and not curative.

Although antiarrhythmic drug therapy and catheter ablation may reduce VT recurrences, there is no proof from prospective or randomized studies that they are also effective in the prevention of sudden death. Therefore, more effective protection is required in individuals at a higher risk of sudden death. In patients who have survived cardiac arrest or hemodynamically intolerable fast VT, and those with risk factors such as extensive right ventricular dysfunction, advanced stages of ARVC/D, left ventricular involvement, syncope, pleomorphic VT, and others, ICD implantation is considered the most appropriate therapeutic option to prevent life-threatening VT recurrences and sudden death (Fig. 18.5).

In the future, ongoing multicenter European [8] and North American [7] ARVC/D registries will provide important data on risk stratification and treatment efficacy, which may refine management strategies and thereby further improve long-term prognosis of patients with ARVC/D.

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