title: Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry.
abstract: OBJECTIVE
  We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) as information concerning the natural history and risk stratification of ARVC is still incomplete.
  
  
  RESULTS
  A cohort of 96 ARVC patients (68% males, 35 ± 15 years) was enrolled and underwent structured diagnostic protocol and follow-up. Primary study endpoints were death and heart transplantation (HTx). Clinical and echo-Doppler data were assessed as prognostic indicators. Sixty-five per cent of patients had right ventricular (RV) systolic dysfunction (RV fractional area change < 33%) and 24% had left ventricular (LV) systolic dysfunction (LV ejection fraction <50%). During a mean follow-up of 128 ± 92 months, 20 patients (21%) experienced cardiac death or underwent HTx. At multivariate analysis (Model 1), RV dysfunction [hazard ratio (HR): 4.12; 95% confidence interval (CI): 1.01-18.0; P = 0.05], significant tricuspid regurgitation (HR: 7.6; 95% CI: 2.6-22.0; P < 0.001), and amiodarone treatment (HR: 3.4; 95% CI: 1.3-8.8; P = 0.01) resulted as predictors of death/HTx. When inserting in the model, the 'ordinal dysfunction' (Model 2), which considers the presence of both RV and LV dysfunctions, this variable emerged as an independent prognostic predictor (HR: 6.3; 95% CI: 2.17-17.45; P < 0.001). At the receiver operating characteristic analysis, Model 2 was significantly more accurate in predicting long-term outcome compared with Model 1 (area under the curve 0.84 vs. 0.78, respectively; P = 0.04).
  
  
  CONCLUSIONS
  In our tertiary referral centre ARVC population, the presence of LV dysfunction at diagnosis has an incremental power in predicting adverse outcome compared with RV dysfunction alone.
authors.affiliation: Cardiovascular Department, Azienda Ospedaliera Ospedali Riuniti and University of Trieste, Trieste, Italy. bpinamonti@hotmail.com
authors.forename: Bruno
authors.initials: B
authors.lastname: Pinamonti
authors.forename: Andreea Mihaela
authors.initials: AM
authors.lastname: Dragos
authors.forename: Stylianos A
authors.initials: SA
authors.lastname: Pyxaras
authors.forename: Marco
authors.initials: M
authors.lastname: Merlo
authors.forename: Alberto
authors.initials: A
authors.lastname: Pivetta
authors.forename: Giulia
authors.initials: G
authors.lastname: Barbati
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Lenarda
authors.forename: Tullio
authors.initials: T
authors.lastname: Morgera
authors.forename: Luisa
authors.initials: L
authors.lastname: Mestroni
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
mesh_terms: D000328:Adult; D000638:Amiodarone / Q000627:therapeutic use; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000188:drug therapy / Q000401:mortality* / Q000601:surgery; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D005260:Female; D006333:Heart Failure / Q000401:mortality; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015588:Observer Variation; D011379:Prognosis; D012042:Registries; D018487:Ventricular Dysfunction, Left / Q000188:drug therapy / Q000401:mortality / Q000601:surgery; D018497:Ventricular Dysfunction, Right / Q000188:drug therapy / Q000209:etiology / Q000401:mortality; D055815:Young Adult
chemical_list: D000889:Anti-Arrhythmia Agents; D000638:Amiodarone