Author + information
- Danita M. Yoerger, MD, MMSc⁎ (, )
- Hugh Calkins, MD,
- Frank Marcus, MD and
- Michael H. Picard, MD
- ↵⁎Cardiac Ultrasound Laboratory, Cardiology Division, Massachusetts General Hospital, YAW 5, 55 Fruit Street, Boston, MA 02114
Dr. Bauce and colleagues raise several issues with regard to our investigation of the echocardiographic findings in arrhythmogenic right ventricular dysplasia (ARVD). The first is to indicate that our patients all met the task force criteria for ARVD/C without the echocardiographic-derived data. Therefore, the diagnosis was obvious without the data. This was an intrinsic design of our study (1), as one cannot assess the utility of a diagnostic method without having a firm diagnosis of the disease excluding the test that is being evaluated because there is no “gold standard.”
The purpose of our report was to identify the best quantitative echocardiographic markers of ARVD in individuals meeting task force criteria for ARVD (2). The data presented compare the measurements of different right ventricular (RV) dimensions in ARVD probands versus controls. We found that the right ventricular outflow tract was the most commonly enlarged dimension in ARVD probands. We provided a numerical value that separated the two groups. At present, it is not uncommon for echocardiographic laboratories to report only qualitative RV size. In order to discriminate the normal RV from the abnormal, as is required in the assessment for ARVD, quantitative measurements should be used.
Additionally, Dr. Bauce and colleagues correctly point out the difficulty detecting ARVD in its preclinical or “presymptomatic” phase. We agree that there is an important need to be able to detect minimal structural abnormalities of the RV in patients suspected of ARVD. Upon its completion, the Multidisciplinary Registry of ARVD will have quantitative data from echocardiograms on a well-characterized population of probands meeting ARVD task force criteria and their family members. It is our hope that with these data, the diagnostic utility of these abnormal echocardiographic parameters found in the probands can be tested in family members at risk for ARVD who are asymptomatic and do not yet meet full task force diagnostic criteria.
Currently, there remains no single defined “gold standard” test for ARVD whether symptomatic or asymptomatic, but given its noninvasive nature and ease of repeated performance, even in the presence of implantable cardiovascular-defibrillators, transthoracic echocardiography is a reasonable tool to study progression of this disease.
- American College of Cardiology Foundation
- Marcus F.,
- Towbin J.A.,
- Zareba W.,
- et al.
- Yoerger D.M.,
- Marcus F.,
- Sherrill D.,
- et al.,
- Multidisciplinary Study of Right Ventricular Dysplasia