Diagnosis of arrhythmogenic right ventricular dysplasia: a review.
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: Cardiomyopathies are diseases of the myocardium of unknown etiology associated with cardiac dysfunction. On the grounds of their morphology and pathophysiology, primary or idiopathic cardiomyopathies may be classified into a number of disorders; namely, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, dilated cardiomyopathy, and restrictive cardiomyopathy. The term "secondary cardiomyopathies" is reserved to specific heart muscle diseases clinically very similar to primary cardiomyopathies. Cardiac magnetic resonance imaging has long been used to study cardiac morphology and, more recently, to assess blood flow, perfusion, and contractile function. The emerging role of magnetic resonance imaging for the understanding and treatment of primary cardiomyopathies cannot be underestimated. From a clinical point of view, an examination based on a single, efficient, and noninvasive MR study focusing on the clinically relevant features of cardiomyopathies is an objective and reproducible means for diagnosing and monitoring hypertrophic, arrhythmogenic, dilated, and restrictive cardiomyopathies.Journal of Computer Assisted Tomography 01/2003; 27(5):724-34. DOI:10.1097/00004728-200309000-00009 · 1.60 Impact Factor
- Der Pathologe 06/1992; 13(3):141-5. · 0.64 Impact Factor
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ABSTRACT: To compare right ventricular (RV) volume measurements and their reproducibility between axial and short axis orientation acquisition techniques. Measurements of RV volumes from data sets acquired in axial and short axis orientations were compared in 20 normal subjects. The observer variabilities were assessed and the left ventricle (LV) and RV stroke volumes (SV) were compared. There was a significant and systematic difference in the EDV and ESV volumes between the axial and short axis methods. The latter method resulted in larger volumes (mean bias EDV 7.5 +/- 13.2, 4.7% difference; ESV 7.2 +/- 8.6, 10.7% difference). The axial method had lower intra- and interobserver variability than the short axis method. The standard deviation of the difference (SDD) and the limits of agreement were consistently lower for the axial method. The mean differences between LV and RV stroke volumes expressed as mean +/- 1 SD (r(2) =correlation coefficient) were: axial 7.6 +/- 9.1 (r(2) = 0.93); and short axis 7.4 +/- 10.8 (r(2) = 0.90). There is a significant systematic difference between volumes measured using the two different orientations. The axial orientation resulted in better inter- and intraobserver reproducibility.Journal of Magnetic Resonance Imaging 07/2003; 18(1):25-32. DOI:10.1002/jmri.10329 · 2.79 Impact Factor