Long-term follow-up analysis of a highly characterized arrhythmogenic cardiomyopathy cohort with classical and non-classical phenotypes–a real-world assessment of a novel prediction model: does the subtype really matter. Issue 5 (13th January 2020)

• Record Type: Journal Article
• Authors: Casella, Michela; Gasperetti, Alessio; Gaetano, Fassini; Busana, Mattia; Sommariva, Elena; Catto, Valentina; Sicuso, Rita; Rizzo, Stefania; Conte, Edoardo; Mushtaq, Saima; Andreini, Daniele; Di Biase, Luigi; Carbucicchio, Corrado; Natale, Andrea; Basso, Cristina; Tondo, Claudio; Dello Russo, Antonio
• Is Part Of: Europace. Volume 22:Issue 5(2020)
• Page Start: 797
• Page End: 805
• Publication Date: 2020-01-13
• Subjects: Arrhythmogenic cardiomyopathy -- Arrhythmogenic right ventricular dysplasia/cardiomyopathy -- Left dominant -- Ventricular arrhythmias -- Implantable cardioverter-defibrillator -- Sudden death risk; Arrhythmia -- Treatment -- Periodicals; Cardiac pacing -- Periodicals; Catheter ablation -- Periodicals; Heart -- Physiology -- Periodicals; Electrophysiology -- Periodicals; 617.4120645
• DOI: 10.1093/europace/euz352
• Languages: English
• ISSNs: 1099-5129

Abstract

Abstract: Aims: To provide long-term outcome data on arrhythmogenic cardiomyopathy (ACM) patients with non-classical forms [left dominant ACM (LD-ACM) and biventricular ACM (Bi-ACM)] and an external validation of a recently proposed algorithm for ventricular arrhythmia (VA) prediction in ACM patients. Methods and results: Demographic, clinical, and outcome data were retrieved from all ACM patients encountered at our institution. Patients were classified according to disease phenotype (R-ACM; Bi-ACM; LD-ACM). Overall and by phenotype long-term survival were calculated; the novel Cadrin-Tourigny et al. algorithm was used to calculate the a priori predicted VA risk, and it was compared with the observed outcome to test its reliability. One hundred and one patients were enrolled; three subgroups were defined (R-ACM, n = 68; Bi-ACM, n = 14; LD-ACM, n = 19). Over a median of 5.41 (2.59–8.37) years, the non-classical form cohort experienced higher rates of VAs than the classical form [5-year freedom from VAs: 0.58 (0.43–0.78) vs. 0.76 (0.66–0.89), P = 0.04]. The Cadrin-Tourigny et al. predictive model adequately described the overall cohort risk [mean observed-predicted risk difference (O-PRD): +6.7 (−4.3, +17.7) %, P = 0.19]; strafing by subgroup, excellent goodness-of-fit was demonstrated for the R-ACM subgroup (mean O-PRD, P = 0.99), while in the Bi-ACM and LD-ACM ones the real observed risk appeared to be underestimated [mean O-PRD: −20.0 (−1.1, −38.9) %, P < 0.0001; −22.6 (−7.8, −37.5) %, P < 0.0001, respectively]. Conclusion: Non-classical ACM forms appear more prone to VAs than classical forms. The novel prediction model effectively predicted arrhythmic risk in the classical R-ACM cohort, but seemed to underestimate it in non-classical forms.