Author + information
- Piotr Szymański, MD, PhD⁎ (, )
- Anna Klisiewicz, MD, PhD and
- Piotr Hoffman, MD, PhD
- ↵⁎Institute of Cardiology, Department of Adult Congenital Heart Diseases, Alpejska 42, 04-628 Warsaw, Poland
In a recent study published in iJACC, Vermes et al. (1) concluded that the revision of the Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) Task Force imaging criteria significantly reduced the overall prevalence of major and minor criteria in cardiac magnetic resonance (CMR) studies (2). The study was accompanied by a very interesting editorial by Bluemke (3), who concluded that the quantitative CMR metrics proposed in the modified task force criteria are useful only “if the CMR laboratory develops its own standards for normal subjects” and if these are the same as previously reported for the MESA (Multi-Ethnic Study of Atherosclerosis) study, which served as a normal reference for comparison with the ARVC/D population.
However, matters become complicated when new echocardiographic ARVC/D Task Force criteria are considered. The new task force recommendations propose as major ARVC/D diagnostic criteria cutoff values of ≥32 mm for diastolic right ventricular outflow tract (RVOT) diameter in the parasternal long-axis (PLAX) view and ≥36 mm for diastolic RVOT diameter in the short-axis view. The respective cutoff values for minor criteria are ≥29 to <32 mm for the diastolic RVOT PLAX view and ≥32 to <36 mm for the diastolic RVOT short-axis view (2). Sensitivity and specificity for the proposed echocardiographic criteria are provided, but contrary to CMR criteria, the task force document does not refer to any specific study (2).
The echocardiographic cutoff values follow neither those proposed by Yoerger et al. (4) nor those proposed by the North American Multidisciplinary Study (5) (both of which are cited in the ARVC/D Task Force document): the RVOT PLAX cutoff value and fractional area change (FAC) proposed by Yoerger et al. (4) are >30 mm and <32%, respectively, and the FAC cutoff value cited in the North American Multidisciplinary Study is ≤26%. FAC cutoff values proposed by the task force are ≤33% and ≤40% (2).
Confusion grows when one refers to the recent American Society of Echocardiography (ASE) guidelines for the echocardiographic assessment of the right heart in adults (6). The cutoff value for the diastolic RVOT PLAX view in these guidelines is ≥32 mm; hence, the revised ARVC/D Task Force minor criteria for the diagnosis of ARVC/D (≥29 to <32 mm) are well within the normal limits recommended by the ASE. To further complicate the situation, the RVOT PLAX measurement techniques proposed by Yoerger et al. (4) and the ASE (6) are not perfectly compatible. Moreover, abnormal FAC is defined as <35% by the ASE and as ≤40% (minor criteria) and <33% (major criteria) by the task force; many normal ASE measurements fulfill the minor ARVC/D diagnostic criteria (2,6).
In conclusion, there is an urgent need to unify the 2 important guideline documents (2,6). Until that happens, because of major discrepancies, we shall not be able to “get along with the guidelines.”
- American College of Cardiology Foundation
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- Bluemke D.A.
- Yoerger D.M.,
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- Rudski L.G.,
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- et al.