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Different prognostic value of functional right ventricular parameters in arrhythmogenic right ventricular cardiomyopathy/dysplasia


Saguner, Ardan M; Vecchiati, Alessandra; Baldinger, Samuel H; Rüeger, Sina; Medeiros-Domingo, Argelia; Mueller-Burri, Andreas S; Haegeli, Laurent M; Biaggi, Patric; Manka, Robert; Lüscher, Thomas F; Fontaine, Guy; Delacrétaz, Etienne; Jenni, Rolf; Held, Leonhard; Brunckhorst, Corinna; Duru, Firat; Tanner, Felix C (2014). Different prognostic value of functional right ventricular parameters in arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation. Cardiovascular Imaging, 7(2):230-239.

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. METHODS AND 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.

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. METHODS AND 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.

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Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > Epidemiology, Biostatistics and Prevention Institute (EBPI)
04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
04 Faculty of Medicine > Institute of Biomedical Engineering
Dewey Decimal Classification:170 Ethics
610 Medicine & health
Language:English
Date:March 2014
Deposited On:27 Aug 2014 15:49
Last Modified:05 Apr 2016 18:21
Publisher:Lippincott, Williams & Wilkins
ISSN:1941-9651
Free access at:Publisher DOI. An embargo period may apply.
Publisher DOI:https://doi.org/10.1161/CIRCIMAGING.113.000210
PubMed ID:24515411

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