Effect of the 2010 task force criteria on reclassification of cardiovascular magnetic resonance criteria for arrhythmogenic right ventricular cardiomyopathy

Abstract

BackgroundWe sought to evaluate the effect of application of the revised 2010 Task Force Criteria (TFC) on the prevalence of major and minor Cardiovascular Magnetic Resonance (CMR) criteria for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) versus application of the original 1994 TFC. We also assessed the utility of MRI to identify alternative diagnoses for patients referred for ARVC evaluation.Methods968 consecutive patients referred to our institution for CMR with clinical suspicion of ARVC from 1995 to 2010, were evaluated for the presence of major and minor CMR criteria per the 1994 and 2010 ARVC TFC. CMR criteria included right ventricle (RV) dilatation, reduced RV ejection fraction, RV aneurysm, or regional RV wall motion abnormalities. When quantitative measures of RV size and function were not available, and in whom abnormal size or function was reported, a repeat quantitative analysis by 2 qualified CMR physicians in consensus.ResultsOf 968 patients, 220 (22.7%) fulfilled either a major or a minor 1994 TFC, and 25 (2.6%) fulfilled any of the 2010 TFC criterion. Among patients meeting any 1994 criteria, only 25 (11.4%) met at least one 2010 criterion. All patients who fulfilled a 2010 criteria also satisfied at least one 1994 criterion. Per the 2010 TFC, 21 (2.2%) patients met major criteria and 4 (0.4%) patients fulfilled at least one minor criterion. Eight patients meeting 1994 minor criteria were reclassified as satisfying 2010 major criteria, while 4 patients fulfilling 1994 major criteria were reclassified to only minor or no criteria under the 2010 TFC.Eighty-nine (9.2%) patients had alternative cardiac diagnoses, including 43 (4.4%) with clinically significant potential ARVC mimics. These included cardiac sarcoidosis, RV volume overload conditions, and other cardiomyopathies.ConclusionsApplication of the 2010 TFC resulted in reduction of total patients meeting any diagnostic CMR criteria for ARVC from 22.7% to 2.6% versus the 1994 TFC. CMR identified alternative cardiac diagnoses in 9.2% of patients, and 4.4% of the diagnoses were potential mimics of ARVC.

Analysis of Statistical Biases in Studies Used to Formulate Guidelines

“…Patients in earlier studies of technology often display severe phenotype of disease and increasing SB. The addition of major criteria points for subjects with ARVC in first-degree family members and for identification of a known ARVC-causing mutation has increased the sensitivity for the diagnosis of subjects with familial disease (25,26).…”

Outcome of Apparently Unexplained Cardiac Arrest

“…3 Emerging evidence supports the use of provocative drug and exercise challenge, as well as advanced cardiac imaging to detect these latent causes for cardiac arrest. [4][5][6][7] A clear diagnosis allows for improved patient care through medication and lifestyle recommendations, and family screening to identify individuals who may be at risk of sudden death. We evaluated the diagnostic outcome of the first 200 patients with an unexplained cardiac arrest in the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER).…”

Review of Journal of Cardiovascular Magnetic Resonance 2015

“…Recent attention has been focussed on T1 imaging for the assessment of diffuse fibrosis in cardiomyopathy, [61, 62] with T1 standardisation, [63] technical developments, and the assessment of diffuse fibrosis in cardiomyopathy. Progress has been prominent in a wide range of unusual conditions including myocarditis, [64, 65] muscular dystrophy, [66, 67] myotonic dystrophy, [68] ARVC, [69, 70] systemic sclerosis, [71] non-compaction, [72, 73] radiation, [74] cocaine toxicity, [75] and iron loading. [76–78] This section describes further advances in this field.…”