CORRESPONDENCE: LETTER TO THE EDITOR
The Right Ventricular Outflow Tract in Arrhythmogenic Right Ventricular Cardiomyopathy
Boban Thomas, MD* and
Nuno Jalles Tavares, MD
* Caselas MR Centre, Rua Carolina Angelo, Lisbon 1400-045, Portugal (Email: bobantho{at}gmail.com).
Dalal et al. (1) describe the accordion sign in family members of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) probands as a promising tool for the early diagnosis of the disease. Besides this qualitative feature, which also was seen in the right ventricular outflow tract (RVOT)/subtricuspid region, they also mention that quantitative right ventricular parameters tended to be abnormal in those with  1 criteria point in their reclassified approach. However, they do not mention whether the RVOT was enlarged in this cohort, a fact we find surprising because in a previous study (2) from the same group, 66% of patients with ARVD/C had an enlarged RVOT measured on standard axial images.
Given the fact that Corrado et al. (3) have suggested that an early/minor form of ARVD/C may primarily involve the RVOT, mimicking idiopathic RVOT tachycardia, we believe that quantitative assessment of the RVOT should always be part of the evaluation of these patients, especially if we are trying to uncover the early manifestations of the disease. Localized dilation of the RVOT detected by cardiovascular magnetic resonance also was present in another study involving 200 probands and relatives (4). If RVOT dilation is present in the group where the accordion sign was detected, it will be a quantitative parameter of the right ventricle that may also help in the early diagnosis of ARVD/C.
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1. Dalal D, Tandri H, Judge DP, et al. Morphologic variants of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy: a genetics–magnetic resonance imaging correlation study J Am Coll Cardiol 2009;53:1289-1299.[Abstract/Free Full Text]2. Tandri H, Castillo E, Ferrari VA, et al. Magnetic resonance imaging of arrhythmogenic right ventricular dysplasia: sensitivity, specificity, and observer variability of fat detection versus functional analysis of the right ventricle J Am Coll Cardiol 2006;48:2277-2284.[Abstract/Free Full Text] 3. Corrado D, Basso C, Leoni L, et al. Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia J Am Coll Cardiol 2008;51:731-739.[Abstract/Free Full Text] 4. Sen-Chowdhry S, Syrris P, Ward D, et al. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression Circulation 2007;115:1710-1720.[Abstract/Free Full Text]
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