Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry

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. A cohort of 96...

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Published inEuropean heart journal Vol. 32; no. 9; pp. 1105 - 1113
Main Authors Pinamonti, Bruno, Dragos, Andreea Mihaela, Pyxaras, Stylianos A, Merlo, Marco, Pivetta, Alberto, Barbati, Giulia, Di Lenarda, Andrea, Morgera, Tullio, Mestroni, Luisa, Sinagra, Gianfranco
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Published England 01.05.2011
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Abstract 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. 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). 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.
AbstractList AIMSWe 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.METHODS AND RESULTSA 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).CONCLUSIONIn 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.
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. 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). 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.
Author Di Lenarda, Andrea
Sinagra, Gianfranco
Pivetta, Alberto
Dragos, Andreea Mihaela
Morgera, Tullio
Pinamonti, Bruno
Pyxaras, Stylianos A
Mestroni, Luisa
Barbati, Giulia
Merlo, Marco
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  surname: Pinamonti
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  organization: Cardiovascular Department, Azienda Ospedaliera Ospedali Riuniti and University of Trieste, Trieste, Italy. bpinamonti@hotmail.com
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  givenname: Andreea Mihaela
  surname: Dragos
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  givenname: Stylianos A
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  surname: Merlo
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/21362707$$D View this record in MEDLINE/PubMed
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Snippet We sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right...
AIMSWe sought to examine the clinical presentation and natural history and to identify long-term prognostic predictors in patients with arrhythmogenic right...
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SubjectTerms Adult
Amiodarone - therapeutic use
Anti-Arrhythmia Agents - therapeutic use
Arrhythmogenic Right Ventricular Dysplasia - drug therapy
Arrhythmogenic Right Ventricular Dysplasia - mortality
Arrhythmogenic Right Ventricular Dysplasia - surgery
Death, Sudden, Cardiac - epidemiology
Female
Heart Failure - mortality
Heart Transplantation - mortality
Humans
Kaplan-Meier Estimate
Male
Middle Aged
Observer Variation
Prognosis
Registries
Ventricular Dysfunction, Left - drug therapy
Ventricular Dysfunction, Left - mortality
Ventricular Dysfunction, Left - surgery
Ventricular Dysfunction, Right - drug therapy
Ventricular Dysfunction, Right - etiology
Ventricular Dysfunction, Right - mortality
Young Adult
Title Prognostic predictors in arrhythmogenic right ventricular cardiomyopathy: results from a 10-year registry
URI https://www.ncbi.nlm.nih.gov/pubmed/21362707
https://search.proquest.com/docview/864959285
Volume 32
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