Outcome of cardioverter-defibrillator implant in patients with arrhythmogenic right ventricular cardiomyopathy.
ABSTRACT The aim of the present study was to investigate outcomes of implantable cardioverter-defibrillator (ICD) treatment in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). We reviewed baseline/follow-up data of 15 consecutive ARVC patients (mean age 55 +/- 15 years) and 30 randomly drawn patients with coronary artery disease (CAD) (mean age 60 +/- 10 years) with matching durations of follow-up (all implanted with ICDs for primary/secondary prevention of sudden death). At implant, appropriate placement of the RV lead was more difficult in ARVC patients. During follow-up (median 41 months), appropriate interventions for any ventricular tachyarrhythmias occurred in 8 (53%) ARVC patients and 17 (57%) CAD patients, but the occurrence of high rate (>240 beats/min) ventricular tachyarrhythmias was higher in ARVC patients. Inappropriate ICD interventions occurred in 5 (33%) ARVC patients and 10 (33%) CAD patients. Lead-related adverse events requiring surgical revision occurred in 7 (47%) ARVC patients as compared with 4 (13%) CAD patients (P = 0.0004). While ICD implantation is highly effective for prevention of sudden death in ARVC, it does carry elevated burdens of long-term lead-related adverse events. These findings underline the need of careful follow-up in ARVC aimed at early recognition of complications that can impair ICD function.
Article: Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare "disease of the desmosome" with multiple clinical presentations.[show abstract] [hide abstract]
ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare but increasingly recognized form of a cardiomyopathy, involving primarily the right ventricle. Mutations in seven candidate genes coding for five desmosomal proteins (plakoglobin, plakophilin-2, desmoplakin, desmoglein-2, desmocollin-2), for the cardiac ryanodine receptor-2, for the transforming growth factor beta-3, and for the transmembrane protein 43, respectively, are pathogenetically important. A typical feature of the disease is the replacement of the right ventricular myocardium by fibrofatty infiltrates, leading to electrical instability including ventricular arrhythmias in the early stages, and reduced contractility and heart failure later on. The left ventricle may also be involved. Unfortunately, the disease is often diagnosed post mortem only, especially in young adults dying suddenly during exercise. Since the disease is inherited in up to 50% of cases, the screening of relatives is important. The implantable cardioverter defibrillator is an important therapeutic tool. Nevertheless, the mortality of the disease remains to be 2%-4% per year. Several clinical, electrocardiographic, and imaging parameters were identified as risk predictors for an adverse outcome. In this paper, we describe distinct clinical presentations of ARVC/D, review the genetic background of the disease, and discuss its diagnosis and treatment.Clinical Research in Cardiology 03/2009; 98(3):141-58. · 2.95 Impact Factor