Diagnosis of arrhythmogenic right ventricular dysplasia: a review.

Department of Radiology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA Leiden, The Netherlands.
Radiographics (Impact Factor: 2.73). 05/2002; 22(3):639-48; discussion 649-50. DOI: 10.1148/radiographics.22.3.g02ma07639
Source: PubMed

ABSTRACT Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disorder of primarily the right ventricle, with unknown cause and prevalence and with a frequent familial occurrence. The typical clinical manifestation consists of ventricular arrhythmias with a left bundle branch block (LBBB) pattern that occur predominantly in young adults. ARVD may result in sudden death. Other manifestations are electrocardiographic repolarization and depolarization changes, structural abnormalities that range from subtle wall aneurysms within the so-called "triangle of dysplasia" to biventricular regional or global dysfunction, and localized or widespread fibrofatty infiltration of the right ventricular myocardium. The diagnosis of ARVD is based on the presence of major and minor criteria encompassing genetic, electrocardiographic, pathophysiologic, and histopathologic factors. The imaging modalities used to evaluate right ventricular abnormalities include conventional angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance (MR) imaging. Among these techniques, MR imaging allows the clearest visualization of the heart. Because MR imaging depicts both functional and structural abnormalities, positive MR imaging findings should be used as important additional criteria in the clinical diagnosis of ARVD. MR imaging appears to be the optimal technique for detection and follow-up of clinically suspected ARVD.

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    ABSTRACT: Right ventricular dysplasia (RVD) is a cardiomyopathy, which is characterized by fatty, or fibro fatty infiltration of myocardium and it is the most common cause of sudden cardiac death in the young. It may be manifested clinically as ventricular arrhythmias with left bundle branch block (LBBB) that may lead to cardiac arrest. So, this condition is also known as Arrhythmogenic right ventricular dysplasia (ARVD).The diagnosis of ARVD is based on the presence of major and minor criteria encompassing genetic, electrocardiographic, pathophysiologic, histopathological factors and imaging modalities. An early and accurate diagnosis followed by appropriate therapy for this condition is increasingly important for it may prevent lethal arrhythmias. Here we are reporting a case of young army man who was died suddenly of RVD along with brief discussion about the Pathophysiology, clinical features, and criteria for diagnosis and differential diagnoses of the condition.
    J Indian Acad Forensic Med. 12/2012; 35(1):79.
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    ABSTRACT: To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) and compare cardiac MR (cMR) findings against clinical diagnosis.
    World journal of cardiology. 07/2014; 6(7):675-81.
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    ABSTRACT: PURPOSE Fibrofatty degeneration of myocardium in ARVC causes detectable wall motion abnormalities. The aim of this study was to examine whether cardiac magnetic resonance (CMR) based strain analysis using feature tracking (FT) can serve as an observer-independent and quantifiable measure to confirm global and regional ventricular dysfunction in ARVC patients and support the detection of early forms of ARVC. METHOD AND MATERIALS We enrolled 20 patients diagnosed with ARVC, 30 with boderline-ARVC and 22 subjects with a positive family history but no signs of a manifest ARVC. 10 healthy volunteers (HV) served as controls. 15 ARVC patients received genotyping for placophilin-2 mutation (PKP-2), of which 8 were found to be positive. Cine MR datasets of all subjects were assessed for myocardial strain using FT (TomTec Diogenes Software). Global and segmental strain in radial, circumferential and longitudinal mode were assessed. RESULTS RV global longitudinal strain in ARVC (-12.7±7.3%) and boderline-ARVC (13.5±6.9%) was significantly reduced (p≤0.05) in comparison with HV (-19.3±6.0%) and subjects with positive family history (-20.3±4.7%). Furthermore, in ARVC patients RV global circumferential strain values were significantly reduced compared with HV at the midventricular (-5.0±3.1 vs. -8.0±2.8%) and basal level (-5.1±2.7 vs. -9.2±3.6%). Even for patients with ARVC or borderline-ARVC and normal RV ejection fraction (n=17) global longitudinal strain proved to be significantly reduced compared with HV (-14.2±6.9 vs. -19.3±6.0%; p≤0.05). In ARVC patients with PKP-2 mutation there was a clear trend towards a more pronounced impairment in RV global longitudinal strain. On ROC analysis RV global longitudinal strain rate and circumferential strain rate at the basal level proved to be the best discriminators between ARVC patients and HV (AUC: 0.9 and 0.92, respectively). CONCLUSION CMR based strain analysis using FT is an objective and useful measure for quantification of wall motion abnormalities in ARVC. It allows differentiation between manifest or boderline-ARVC and HV, even if ejection fraction is still normal. CLINICAL RELEVANCE/APPLICATION Importance of quantitative parameters in ARVC diagnosis is underlined by the modification of the Task Force Criteria 2010 and CMR-based strain promises to be a powerful measure to objectify diagnosis.
    Radiological Society of North America 2013 Scientific Assembly and Annual Meeting; 12/2013