title: Different prognostic value of functional right ventricular parameters in arrhythmogenic right ventricular cardiomyopathy/dysplasia.
abstract: BACKGROUND
  The value of standard 2-dimensional transthoracic echocardiographic parameters for risk stratification in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is controversial.
  
  
  RESULTS
  We investigated the impact of RV fractional area change (FAC) and tricuspid annulus plane systolic excursion (TAPSE) for the prediction of major adverse cardiovascular events (MACE) defined as the occurrence of cardiac death, heart transplantation, survived sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, or arrhythmogenic syncope. Among 70 patients who fulfilled the 2010 ARVC/D Revised Task Force Criteria and underwent baseline transthoracic echocardiography, 37 (53%) patients experienced MACE during a median follow-up period of 5.3 (interquartile range, 1.8-9.8) years. Average values for FAC, TAPSE, and TAPSE indexed to body surface area (BSA) decreased over time (P=0.03 for FAC, P=0.03 for TAPSE, and P=0.01 for TAPSE/BSA, each versus baseline). In contrast, median RV end-diastolic area increased (P=0.001 versus baseline). Based on the results of Kaplan-Meier estimates, the time between baseline transthoracic echocardiography and experiencing MACE was significantly shorter for patients with FAC <23% (P<0.001), TAPSE <17 mm (P=0.02), or right atrial short axis/BSA ≥25 mm/m(2) (P=0.04) at baseline. A reduced FAC constituted the strongest predictor of MACE (hazard ratio, 1.08 per 1% decrease; 95% confidence interval, 1.04-1.12; P<0.001) on bivariable analysis.
  
  
  CONCLUSIONS
  This long-term observational study indicates that TAPSE and dilation of right-sided cardiac chambers are associated with an increased risk for MACE in patients with ARVC/D with advanced disease and a high risk for adverse events. However, FAC is the strongest echocardiographic predictor of adverse outcome in these patients. Our data advocate a role for transthoracic echocardiography in risk stratification in patients with ARVC/D, although our results may not be generalizable to lower-risk ARVC/D cohorts.
authors: Department of Cardiology, University Heart Center Zurich, Zurich, Switzerland.
authors: Ardan M
authors: AM
authors: Saguner
authors: Alessandra
authors: A
authors: Vecchiati
authors: Samuel H
authors: SH
authors: Baldinger
authors: Sina
authors: S
authors: Rüeger
authors: Argelia
authors: A
authors: Medeiros-Domingo
authors: Andreas S
authors: AS
authors: Mueller-Burri
authors: Laurent M
authors: LM
authors: Haegeli
authors: Patric
authors: P
authors: Biaggi
authors: Robert
authors: R
authors: Manka
authors: Thomas F
authors: TF
authors: Lüscher
authors: Guy
authors: G
authors: Fontaine
authors: Etienne
authors: E
authors: Delacrétaz
authors: Rolf
authors: R
authors: Jenni
authors: Leonhard
authors: L
authors: Held
authors: Corinna
authors: C
authors: Brunckhorst
authors: Firat
authors: F
authors: Duru
authors: Felix C
authors: FC
authors: Tanner
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*; D016001:Confidence Intervals; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000209:etiology; D018450:Disease Progression; D018618:Echocardiography, Doppler, Color; D005260:Female; D005500:Follow-Up Studies; D006325:Heart Atria / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012372:ROC Curve; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D013557:Switzerland / Q000453:epidemiology; D013997:Time Factors; D014693:Ventricular Fibrillation / Q000453:epidemiology / Q000209:etiology; D016278:Ventricular Function, Right / Q000502:physiology*
keywords: arrhythmogenic right ventricular dysplasia; cardiomyopathies; echocardiography