Magnetic resonance imaging findings in patients meeting Task Force criteria for arrhythmogenic right ventricular dysplasia
ABSTRACT Magnet resonance imaging (MRI) findings in patients meeting Task Force criteria for the diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) have not been systematically described. We report qualitative and quantitative MRI findings in ARVD using state-of-the-art MRI.
MRI was performed on 12 patients with ARVD who were prospectively diagnosed using the Task Force criteria. The imaging protocol included breath-hold double inversion recovery spin-echo and gradient-echo images. Ventricular volumes and dimensions were compared to 10 age- and sex-matched normal volunteers. High intramyocardial T1 signal similar to fat signal was observed in 9 (75%) of the 12 patients and in none of the controls. Right ventricular (RV) hypertrophy was seen in 5 (42%) patients, trabecular disarray in 7 (59%), and wall thinning in 3 (25%). Both the RV end-diastolic diameter and the outflow tract area were significantly higher in ARVD patients compared to controls (51.2 vs 43.2 mm, P < 0.01; and 14.5 vs 9.3 cm2, P < 0.01, respectively). ARVD patients had a higher RV end-diastolic volume index and lower RV ejection fraction compared with controls (127.4 vs 87.5, P < 0.01; and 41.6% vs 57%, P < 0.01, respectively).
High intramyocardial T1 signal indicative of fat is seen in a high percentage (75%) of patients who meet the Task Force criteria for ARVD. Trabecular disarray is seen more frequently than wall thinning and aneurysms. RV dimensions and volumes differ significantly in ARVD compared to controls, indicating a role for quantitative evaluation in the diagnosis of ARVD.
- SourceAvailable from: Shigeo Horinaka
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- "However , the most remarkable feature of this case was the morphological and electrophysiological abnormalities in the RA. There are some reports that the atrial diameter are enlarged in patients with ARVC  . In addition, fibrofatty replacement of the RA with associated SSS in patients ARVC has been demonstrated  . "
ABSTRACT: We describe a 59-year-old woman with sick sinus syndrome (SSS) and arrhythmogenic right ventricular cardiomyopathy (ARVC). Diagnosis of SSS was made because she had frequent episodes of sinus arrest with prolonged ventricular asystole. Cardiac images showed a dilated right atrium (RA) and a right ventricle (RV). Electroanatomical mapping of the RA showed extensive scarring with no recordable electrical potentials. Although she had frequent premature ventricular contractions, neither spontaneous ventricular tachycardia (VT) nor induced VT was observed. Microscopic examination of the RV indicated fibrofatty myocardium. Atrial arrhythmias associated with SSS may be the cause of symptoms in some cases of ARVC.Journal of Cardiology 06/2008; 51(3):205-9. DOI:10.1016/j.jjcc.2008.03.003 · 2.57 Impact Factor
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ABSTRACT: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a genetic cardiomyopathy characterized by ventricular arrhythmias and structural abnormalities of the right ventricle (RV). In ARVD/C there is progressive replacement of right ventricular myocardium with fatty and fibrous tissue. The precise prevalence of ARVD/C has been estimated to vary between 1 in 1000 and 1 in 5000 of the general population. The purpose of this chapter is to review the current understanding of ARVD/C and its management. Particular attention will be focused on some of the recent advances in the understanding of the genetic basis of ARVD/C and how this information provides insight into the mechanism of ventricular arrhythmias that can cause sudden death.