CORRESPONDENCE: LETTER TO THE EDITOR
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Darshan Dalal, MD, PhD, MPH*,
Harikrishna Tandri, MD,
David A. Bluemke, MD, PhD and
Hugh Calkins, MD
* Johns Hopkins Medical Institutions, 600 North Wolfe Street, Carnegie 592, Baltimore, Maryland 21287 (Email: ddalal1{at}jhmi.edu).
We are thankful to Drs. Thomas and Tavares for their interest in our study (1). Affliction of the right ventricular outflow track (RVOT) in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has been well documented since its first description and also in subsequent reports (2–4). In our earlier report, each of the 15 ARVD/C patients presented with left bundle branch block-type ventricular tachycardia and had an established diagnosis of ARVD/C (3). Similarly, Sen-Chowdhry et al. (4) also relied on fulfillment of ARVD/C diagnostic criteria for inclusion in their study. It comes as no surprise that there was a greater proportion of patients with RVOT dilation in these study populations compared with our present report. For identification of early morphologic variants of ARVD/C, our present study was carefully designed to exclude individuals with advanced disease. Exclusion of the probands resulted in a study population that comprised, mostly, of asymptomatic family members who had never sought medical attention for cardiac symptoms (1). None of these patients had a dilated RVOT similar to that described previously (3).
Corrado et al. (5) used electroanatomic voltage mapping to demonstrate that early ARVD/C may manifest as RVOT tachycardia in the absence of right ventricular dilation. Notably, in the same study, there was no significant difference in the RVOT diameter between patients with and without abnormal electroanatomic voltage maps (5). From our investigation of probands in the present study (excluded in the main analysis), it appears that the accordion sign may be a precursor of aneurysmal dilation (1). In considering this, we believe that quantitative evaluation of RVOT does not help quantify the accordion sign itself but the deterioration of right ventricular function in the "triangle of dysplasia" associated with advancing disease.
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Footnotes
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Please note: The first 2 authors contributed equally to this work.
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References
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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. Marcus FI, Fontaine GH, Guiraudon G, et al. Right ventricular dysplasia: a report of 24 adult cases Circulation 1982;65:384-398.[Abstract/Free Full Text] 3. 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] 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] 5. Corrado D, Basso C, Leoni L, et al. Three-dimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia Circulation 2005;111:3042-3050.[Abstract/Free Full Text]
Related Article
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The Right Ventricular Outflow Tract in Arrhythmogenic Right Ventricular Cardiomyopathy
- Boban Thomas and Nuno Jalles Tavares
J. Am. Coll. Cardiol. 2009 54: 1558-1559.
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