Abstract 2828: Application of New Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Dysplasia
Background: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) diagnostic Task Force criteria (TFC) proposed in 1994 are highly specific but lack sensitivity. A new international Task Force will publish modified criteria to improve diagnostic yield. Aim: comparison of diagnosis by 1994 TFC versus newly proposed criteria in 3 patient groups.
Methods: In new TFC, scoring by major and minor criteria is maintained. Structural abnormalities are quantified and TFC highly specific for ARVD/C upgraded to major (see table⇓). Also, new criteria are added: terminal activation duration of QRS = or > 55ms, VT with LBBB morphology and superior axis plus genetic criteria. Three groups were studied:
94 patients with proven ARVD/C according to 1994 TFC (ARVD/C pts),
85 of their family members (Fam) and
34 patients with probable ARVD/C (i.e. 3 points by 1994 TFC; TFC3 pts).
ECGs were scored while off drugs. All pts and Fam were screened for pathogenic mutations in desmosomal genes.
Results: Three ARVD/C pts did not meet the new sharpened criteria on structural abnormalities and thereby did not fulfill new TFC. In 56 ARVD/C pts mutations were found, 53 in Plakophilin2 (PKP2), 2 in Desmoglein2 and 1 in Desmocollin2. Twelve additional Fam (14%) fulfilled new TFC: 11 (94%) females and all carriers of PKP2 mutations. None of Fam lost diagnosis after new scoring. Of TFC3 pts, 20 (59%) fulfilled new TFC: 8 (40%) females and 11 (55%) carrying PKP2 mutations.
Conclusions: In this first study applying new TFC to pts suspected of ARVD/C, 59% of probable ARVD/C pts and 14% of family members were additionally diagnosed. Newly proposed TFC have a major impact in increasing diagnostic yield of ARVD/C.