Long-termfollow-up analysis of a highly characterized arrhythmogenic cardiomyopathy cohort with classical and non-classical phenotypes-a real-world assessment of a novel predictionmodel: Does the subtype really matter

Michela Casella, Alessio Gasperetti, Fassini Gaetano, Mattia Busana, Elena Sommariva, Valentina Catto, Rita Sicuso, Stefania Rizzo, Edoardo Conte, Saima Mushtaq, Daniele Andreini, Luigi Di Biase, Corrado Carbucicchio, Andrea Natale, Cristina Basso, Claudio Tondo, Antonio Dello Russo

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)797-805
Number of pages9
JournalEuropace
Volume20
Issue number5
DOIs
StatePublished - Jan 1 2020

Keywords

  • Arrhythmogenic cardiomyopathy
  • Arrhythmogenic right ventricular dysplasia/cardiomyopathy
  • Implantable cardioverter-defibrillator
  • Left dominant
  • Sudden death risk
  • Ventricular arrhythmias

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Fingerprint Dive into the research topics of 'Long-termfollow-up analysis of a highly characterized arrhythmogenic cardiomyopathy cohort with classical and non-classical phenotypes-a real-world assessment of a novel predictionmodel: Does the subtype really matter'. Together they form a unique fingerprint.

Cite this