Diagnosis of Arrhythmogenic Right Ventricular Dysplasia: A Review1
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.
- SourceAvailable from: aafp.orgDer 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
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ABSTRACT: The diagnostic dilemma in arrhythmogenic right ventricular dysplasia-cardiomyopathy (ARVD/C) is that a single diagnostic test does not exist and that there is a need for broadening diagnostic criteria. As standard ECG contributes significantly to clinical diagnosis and represents a tool for screening in family studies ECG data should be revisited. In a cohort of 265 patients (159 males, mean age 46.8 years) with ISFC/ESC criteria of ARVD/C ECG features were reevaluated. QRS duration in (V1 + V2 + V3)/(V4 + V5 + V6) > or = 1.2-called localized right precordial QRS prolongation-was present in 261/265 patients (98%) and represents the essential finding. Right precordial epsilon potentials were found in 23% in standard and in 75% in highly amplified and modified recording technique. Right precordial T wave inversions were present in 143 cases (54%) and ST-segment elevation of different types in 66 patients (25%). Localized prolongation of inferior QRS complexes could be found in 58 cases (22%), complete right bundle branch block with T inversions beyond V2 in most cases in 17 patients (6%), incomplete right bundle branch block in 38 cases (14%), pseudo-incomplete right bundle branch block in 8 patients (3%), and right precordial R wave reduction in 14 cases (5%). With regard to sensitivity and already known specificity an ECG score for the diagnosis of ARVD/C was developed with high probability of ARVD/C in cases with > or =4 points, possibly without the need for an additional imaging technique. Standard ECG with additional highly amplified and modified recording technique represents a single diagnostic test with high value in the clinical diagnosis of ARVD/C and should be used as a first line tool in noninvasive family screening.Annals of Noninvasive Electrocardiology 07/2003; 8(3):238-45. DOI:10.1046/j.1542-474X.2003.08312.x · 1.44 Impact Factor