CORRESPONDENCE: LETTERS TO THE EDITOR
Echocardiographic Findings in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia
Barbara Bauce, MD, PhD*,
Gianfranco Frigo, MD and
Andrea Nava, MD
* Department of Cardiology, University of Padua Medical School, Via Giustiniani, 2, Padova, 35100, Italy (Email: barbara.bauce{at}unipd.it).
We read with interest the study by Yoerger et al. (1) presenting echocardiographic data of 29 probands affected by arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosed with the International Society and Federation of Cardiology task force criteria. In all cases the echocardiogram showed typical features of the disease. It is well known that ARVC diagnosis is made considering different parameters. The purpose of their study was to assess echocardiographic abnormalities in a series of patients affected by ARVC, in whom the echocardiogram has not been considered for the diagnosis.
Thus, we can assume that all these patients showed a clear form of the disease, fulfilling the diagnostic criteria even in the absence of the echocardiographic tool. However, the real problem is to understand whether the typical ARVC echocardiographic alterations have a diagnostic value per se, even when the task force criteria (2 major, 1 major plus 2 minor, 4 minor) are not satisfied. This point is critical because in the presymptomatic phase of the disease the lack of diagnosis can lead to heavy consequences for the subjects, who could be at risk of life-threatening ventricular arrhythmias and sudden death.
Moreover, genetic studies (2) demonstrated that mutation carriers often do not fulfill the published criteria. This leads to the need to reevaluate the present task force criteria and to reexamine the different echocardiographic abnormalities, trying to assess which of these are specific to make a diagnosis of ARVC in the absence of other codified criteria.
As shown by the investigators, a correct echocardiographic evaluation can lead to the detection of several morphologic and kinetic aspects of the right ventricle. At this point, considering that an echocardiogram is a noninvasive technique that can be repeated in time, can we consider this diagnostic tool as the "gold standard" of ARVC diagnosis in asymptomatic patients or in patients with minor forms of the disease (3)? Hence, the real significance of echocardiographic abnormalities in this group of "uncertain subjects" constitutes the major challenge in echocardiographic evalution of ARVC.
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1. Yoerger DM, Marcus F, Sherrill D, et al. Multidisciplinary Study of Right Ventricular Dysplasia Echocardiographic findings in patients meeting task force criteria for arrhythmogenic right ventricular dysplasianew insights from the Multidisciplinary Study of Right Ventricular Dysplasia. J Am Coll Cardiol 2005;45:860-865.[Abstract/Free Full Text]
2. Rampazzo A, Nava A, Malacrida S, et al. Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy Am J Hum Genet 2002;71:1200-1206.[CrossRef][Web of Science][Medline]
3. Nava A, Bauce B, Basso C, et al. Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy J Am Coll Cardiol 2000;36:2226-2233.[Abstract/Free Full Text]
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