LAUSR

The interesting manuscript of Bosman et al. challenges the established 2010 arrhythmogenic right ventricular cardiomyopathy (ARVC) Task Force Criteria (TFC). While I agree that the 2010 TFC ought to be improved, I am surprised by some aspects of this work. First, I am surprised about the choice of the gold standard. Instead of using reproducible genetic, phenotypic and clinical criteria, Bosman et al. used expert consensus to diagnose or exclude ARVC. The Supplementary Table 6 displays the challenge of making an ARVC diagnosis. Notably, 4 out of 10 ‘false positive’ patients with the diagnosis ARVC according to 2010 TFC were diagnosed as ‘at risk for ARVC’ by the expert panel. And all as ‘false negative’ declared cases could be diagnosed as borderline or possible ARVC according to the 2010 TFC. Regrettably, the criteria for diagnosis of ARVC by the expert panel are not made available and can therefore not be reproduced. Second, the control group did not consist of healthy controls but of patients in whom ARVC was suspected by referring physicians. Minor family criteria, autopsy diagnosis in a first-degree relative and signal-averaged electrocardiogram (SAECG) did not significantly vary between both groups and are therefore regarded as not useful by the authors. But not having the specifics of the control group plus the small number of patients with a positive minor family criteria limit the statistical power of the comparisons. Additionally, in the 2010 TFC, the SAECG had a sensitivity 74%, with a specificity of 92%. Third, the authors’ statement that ‘ECG and arrhythmia criteria alone can rule out ARVC with high sensitivity’ is overconfident. In cases of early ARVC, ECG and arrhythmia criteria might be insufficient to diagnose the disease, while clinical and family history, magnetic resonance imaging and genetic diagnostic can give important hints to a timely, potentially life-saving diagnosis. The 2010 TFC strive to balance overand underdiagnosis. Attempts to improve diagnostic criteria should maintain and improve this balance.