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.
[Show abstract][Hide abstract] ABSTRACT: To identify correlates of impaired quality of life (QOL), anxiety, and depression in patients with an implantable cardioverter-defibrillator (ICD).
Surviving patients (n = 610) who received an ICD in our institution since 1989 completed the Short Form Health Survey (SF-36) and the Hospital Anxiety and Depression Scale. Mean age was 62.4 years with 18% females. In a multivariate logistic regression analysis, symptomatic heart failure was the most important correlate of impaired QOL (SF-36) across all eight subscales [odds ratios (ORs) ranging from 5.21 to 22.53)], whereas psychotropic medication, age, comorbidity, amiodarone, and ICD shocks all correlated to a lesser extent. Symptomatic heart failure was also the most dominant correlate of anxiety [OR 5.15 (3.08-8.63), P < 0.001] and depression [OR 6.82 (3.77-12.39), P < 0.001]. Implantable cardioverter-defibrillator shocks correlated less yet significantly with anxiety [OR 2.21 (1.32-3.72) P < 0.01] and depression [OR 2.00 (1.06-3.80), P < 0.05].
Symptomatic heart failure was the single most important clinical correlate of impaired QOL, anxiety, and depression, with ICD shocks playing only a secondary role. This suggests that comorbidity rather than ICD therapy per se influences patients' device acceptance, supporting the increasing use of prophylactic ICD implantation.
[Show abstract][Hide abstract] ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by the progressive replacement of myocardial cells by fat and fibrous tissue. Here we describe the histopathological features of biopsied myocardium from a patient with ARVC. A large amount of adipose tissue was present in the biopsy specimen, and a group of myocardial cells were isolated as an island-like region in the adipose tissue. Electron microscopic examination of cardiomyocytes revealed a large number of intracellular lipid droplets, including some extremely large droplets. Disruptions of the plasma membrane and dissociation of intercellular junctions were associated with discharge of intracellular lipid droplets into the interstitial space. The high accumulation of intracellular lipid droplets may be involved in the pathogenesis of ARVC and may have played an important role in myocardial cell death and progressive replacement of cardiomyocytes by fatty tissue in the current case.
Heart and Vessels 12/2008; 23(6):440-4. DOI:10.1007/s00380-008-1079-0 · 2.07 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Naxos disease is a recessively inherited stereotype association of arrhythmogenic cardiomyopathy with a cutaneous phenotype, characterized by peculiar woolly hair and palmoplantar keratoderma. The cardiomyopathy clinically manifests by adolescence and the symptomatic presentation is usually with syncope and/or sustained ventricular tachycardia of left bundle branch block configuration. We report the case of a 43-year-old man without any history of heart disease who was admitted to the hospital because of an episode of sustained ventricular tachycardia and troponin I elevation, in the absence of coronary artery disease. Diagnostic workup, including genetic assessment, revealed Naxos disease as the underlying cause. In this case, acute myocarditis seems to be the most plausible explanation for the nonischemic myocardial injury.
Heart and Vessels 02/2009; 24(1):63-5. DOI:10.1007/s00380-008-1082-5 · 2.07 Impact Factor
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