BACKGROUND: Early recognition and accurate risk stratification are important in the management of arrhythmogenic right ventricular cardiomyopathy (ARVC). Identification of predictors of outcome by cardiovascular magnetic resonance (CMR) in patients undergoing evaluation for ARVC is limited. We investigated the predictive value of morphological abnormalities detected by CMR for major clinical events in patients with suspected ARVC. METHODS: We performed a longitudinal study on 369 consecutive patients with at least one criterion for ARVC. Abnormal CMR was defined by the presence of one of the following: increased right ventricular (RV) volumes, reduced RV ejection fraction, RV regional wall motion abnormalities, myocardial fatty infiltration, and myocardial fibrosis. The end-point was a composite of cardiac death, sustained ventricular tachycardia, ventricular fibrillation, and appropriate ICD discharge. RESULTS: Twenty patients met the composite end-point over a mean follow-up of 4.3±1.5 years. An abnormal CMR was an independent predictor of outcomes (p<0.001). The presence of multiple abnormalities heralded a particular high risk of events (HR 23.0, 95% CI 5.7-93.2, p<0.001 for 2 abnormalities; HR 35.8, 95% CI 9.7-132.6, p<0.001 for 3 or more abnormalities). The positive predictive value of an abnormal CMR study was 21.0% for an adverse event, whilst the negative predictive value of a normal CMR study was 98.8% over the follow-up period. CONCLUSIONS: CMR provides important prognostic information in patients under evaluation for ARVC. A normal study portends a good prognosis. Conversely, the presence of multiple abnormalities identifies a high risk group of patients who may benefit from ICD implantation.
BACKGROUND: Early recognition and accurate risk stratification are important in the management of arrhythmogenic right ventricular cardiomyopathy (ARVC). Identification of predictors of outcome by cardiovascular magnetic resonance (CMR) in patients undergoing evaluation for ARVC is limited. We investigated the predictive value of morphological abnormalities detected by CMR for major clinical events in patients with suspected ARVC. METHODS: We performed a longitudinal study on 369 consecutive patients with at least one criterion for ARVC. Abnormal CMR was defined by the presence of one of the following: increased right ventricular (RV) volumes, reduced RV ejection fraction, RV regional wall motion abnormalities, myocardial fatty infiltration, and myocardial fibrosis. The end-point was a composite of cardiac death, sustained ventricular tachycardia, ventricular fibrillation, and appropriate ICD discharge. RESULTS: Twenty patients met the composite end-point over a mean follow-up of 4.3±1.5 years. An abnormal CMR was an independent predictor of outcomes (p<0.001). The presence of multiple abnormalities heralded a particular high risk of events (HR 23.0, 95% CI 5.7-93.2, p<0.001 for 2 abnormalities; HR 35.8, 95% CI 9.7-132.6, p<0.001 for 3 or more abnormalities). The positive predictive value of an abnormal CMR study was 21.0% for an adverse event, whilst the negative predictive value of a normal CMR study was 98.8% over the follow-up period. CONCLUSIONS: CMR provides important prognostic information in patients under evaluation for ARVC. A normal study portends a good prognosis. Conversely, the presence of multiple abnormalities identifies a high risk group of patients who may benefit from ICD implantation.
Authors: Tuna Ustunkaya; Benoit Desjardins; Riley Wedan; C Anwar A Chahal; Stefan L Zimmerman; Nissi Saju; Sohail Zahid; Apurva Sharma; Yuchi Han; Natalia Trayanova; Francis E Marchlinski; Hugh Calkins; Harikrishna Tandri; Saman Nazarian Journal: JACC Clin Electrophysiol Date: 2019-08-28
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Authors: Jens Cosedis Nielsen; Yenn-Jiang Lin; Marcio Jansen de Oliveira Figueiredo; Alireza Sepehri Shamloo; Alberto Alfie; Serge Boveda; Nikolaos Dagres; Dario Di Toro; Lee L Eckhardt; Kenneth Ellenbogen; Carina Hardy; Takanori Ikeda; Aparna Jaswal; Elizabeth Kaufman; Andrew Krahn; Kengo Kusano; Valentina Kutyifa; Han S Lim; Gregory Y H Lip; Santiago Nava-Townsend; Hui-Nam Pak; Gerardo Rodríguez Diez; William Sauer; Anil Saxena; Jesper Hastrup Svendsen; Diego Vanegas; Marmar Vaseghi; Arthur Wilde; T Jared Bunch; Alfred E Buxton; Gonzalo Calvimontes; Tze-Fan Chao; Lars Eckardt; Heidi Estner; Anne M Gillis; Rodrigo Isa; Josef Kautzner; Philippe Maury; Joshua D Moss; Gi-Byung Nam; Brian Olshansky; Luis Fernando Pava Molano; Mauricio Pimentel; Mukund Prabhu; Wendy S Tzou; Philipp Sommer; Janice Swampillai; Alejandro Vidal; Thomas Deneke; Gerhard Hindricks; Christophe Leclercq Journal: Europace Date: 2020-08-01 Impact factor: 5.214