Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy

Nava, Andrea; Bauce, Barbara; Basso, Cristina; Muriago, Michela; Rampazzo, Alessandra; Villanova, Carla; Daliento, Luciano; Buja, Gianfranco; Corrado, Domenico; Danieli, Gian Antonio; Thiene, Gaetano

doi:10.1016/s0735-1097(00)00997-9

Cited by 404 publications

(288 citation statements)

References 35 publications

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“…When these modified criteria are used to screen relatives, 39% of them will have signs of the disease, as opposed to 28% if the original criteria were used 36 . A similar percentage (41%) was reported by Nava et al 37 . During a long-term follow-up (mean 8.5 years) of 37 Italian families encompassing 365 subjects, Nava et al…”

Section: Evaluation Of Relativessupporting

confidence: 89%

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Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

2009

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Section: Evaluation Of Relativessupporting

confidence: 89%

“…reported a favorable prognosis even for affected patients (mortality rate of 0.08 patients/year) 37 . Despite this fact, diligent medical care of these patients is of utmost importance and they should avoid strenuous physical activity.…”

Section: Evaluation Of Relativesmentioning

confidence: 99%

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

2009

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“…8,9 Higher numbers, however, are found in specific regions, for example, the Veneto region of Italy. 10 …”

Section: Mutational Spectrummentioning

confidence: 99%

“…5,13,17,18 Index case in that family had not been tested: ARVC is believed to be familial in B30-50% of cases. 1,8,9,20 Therefore, when an index patient has been clinically diagnosed with ARVC, and no genetic test has been performed, the chance for a firstdegree relative to develop ARVC may reach up to 15-25%.…”

Section: Positive Clinical Predictive Valuementioning

confidence: 99%

Clinical utility gene card for: arrhythmogenic right ventricular cardiomyopathy (ARVC)

et al. 2013

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“…However, long-term favourable course of the disease has also been described. The mortality rate in published studies is shown in Table 1 (7,8,(28)(29)(30)(31)(32)(33). Recently, Hulot et al (31), described the natural history of 130 patients with ARVC/D who were referred to a tertiary center and followed for 8.1±7.8 years.…”

Section: Natural History and Mortalitymentioning

confidence: 99%

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Risk Stratification and Therapy

Buja

Estes

Wichter

et al. 2008

Progress in Cardiovascular Diseases

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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited heart muscle disease that occurs primarily in young and middle-age individuals. It is characterized by ventricular arrhythmias (VA), sudden death, and by heart failure occurring later in life (1-5). Ventricular electrical instability may occur at any time during the disease depending upon possible different pathophysiologic mechanisms including: a) Inflammation and apoptosis leading to ventricular fibrillation; or b) Fibro-fatty tissue repair leading to scar-related ventricular tachycardia (VT). Heart failure may occur later in life secondary to slow, progressive loss of right and left ventricular myocardium (1-8). The role of pharmacological therapy in controlling VA and preventing sudden death has been evaluated in single-center studies (9), and the results of catheter ablation have been described in small series of patients (10-15). The efficacy and safety of implantable cardioverter/ defibrillator (ICD) have also been reported in small, single-center studies (16)(17)(18)(19) and recently in larger single and multicenter studies (20)(21)(22)(23)(24)(25)(26)(27). The main questions regarding the risk stratification and the therapeutic strategy in ARVC/D are: 1) differential diagnosis with idiopathic VT (right ventricular outflow tract VT) in an apparently normal heart. The prognosis of this latter condition is usually excellent with rare cases of sudden cardiac death; 2) prognostic and therapeutic significance of noninvasive and invasive investigations including electrophysiologic study (EPS); 3) efficacy of antiarrhythmic drugs (AAD) to prevent VT and sudden cardiac death and the adverse effects of these drugs in this population; 4) indications and results of catheter ablation; 5) identification of patients at high risk of sudden cardiac death who need ICD implantation as well as the complications of ICD in a diseased right ventricular myocardium. It is important to recognize that ARVC/D is a progressive disease and risk factors may change during follow-up requiring periodic revaluation of risk as well as of therapy.With the identification of family members who carry a disease causing gene, the therapeutic dilemma has broadened to include risk stratification in genotype positive family members who may have occasional ventricular ectopy or have no clinical evidence of the disease.Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers

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Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy

Cited by 404 publications

References 35 publications

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

Clinical utility gene card for: arrhythmogenic right ventricular cardiomyopathy (ARVC)

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Risk Stratification and Therapy

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