Rationale and Objectives
To analyze the statistical biases in the studies used to derive cardiac magnetic resonance–based major and minor criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC).
Materials and Methods
ARVC is a rare disorder of the heart that can lead to sudden death in young adults. Cardiac magnetic resonance imaging (CMR) plays a role in the diagnosis by contributing to the criteria set by experts. The original criteria emphasized qualitative analysis of CMR. The criteria were modified in 2010 to provide quantitative cutoffs.
Results
We apply the Quality Assessment of Diagnostic Accuracy Studies (QUADAS-2) tool for systematic review of diagnostic accuracy to the studies cited in the guidelines written in 1994 and revised in 2010. We use the signaling questions in QUADAS-2 to identify different types of statistical bias.
Conclusions
The studies have understandable biases that affect the sensitivity and specificity of CMR in the diagnosis of ARVC, as well as the truth of the disease state. There is potential to overdiagnose ARVC particularly in low prevalence populations.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiomyopathy that primarily affects the right ventricle (RV) and is potentially fatal if untreated . The incidence is 1 in 5000 individuals . Affected individuals can present with ventricular arrhythmias and sudden cardiac death and typically present in the third decade of life. There is a genetic component of unclear magnitude .
A task force was established in 1994 , and another convened in 2009, , to develop criteria for the diagnosis of ARVC ( Table 1 ). Cardiac magnetic resonance imaging (CMR) contributes to the criteria for the diagnosis of ARVC.
Table 1
Summary of the Original (1994) and Modified (2010) Task Force Criteria for the Diagnosis of ARVC/D
Adapted from .
Global or Regional Dysfunction and Structural Alterations Original Task Force Criteria (1994) Modified Task Force Criteria (2010) Not divided according to modality:
By MRI:
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ARVC/D, arrhythmogenic right ventricular cardiomyopathy/dysplasia; BSA, body surface area; F, female; LV, right ventricle; M, male; MRI, magnetic resonance imaging; RVEDV, right ventricular end-diastolic volume; RVEF, right ventricular ejection fraction; Se, sensitivity; Sp, specificity.
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Method
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An Overview of Bias
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Analysis
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Table 2
The QUADAS-2 Tool was Used to Evaluate Studies Included in the Development of the Original and Revised Task Force Criteria for the Diagnosis of ARVC/D
ARVC/D, arrhythmogenic right ventricular cardiomyopathy/dysplasia; QUADAS-2, Quality Assessment of Diagnostic Accuracy Studies.
Each study is evaluated on four domains–patient selection, index test, reference standard, and flow and timing–for the risk of bias and applicability concerns. The format of the results reported here follows the downloadable template provided at http://www.quadas.org .
Table 3
Evaluation of Biases Introduced into the Studies Used to Develop the Original and Modified Task Force Criteria for ARVC/D
Study IB VB DGSB SB IGSB Original Task Force Criteria Ricci et al., 1992 X X Robertson et al., 1985 X X Daubert et al., 1988 X X Blomström et al., 1988 X X X Daliento et al., 1990 X X Revised Task Force Criteria Marcus et al., 2009 X X X X Sen-Chowdhry et al., 2006 X X Bluemke et al., 2003 X X Bomma et al., 2004 X X X X
ARVC/D, arrhythmogenic right ventricular cardiomyopathy/dysplasia; DGSB, double gold standard bias; IB, incorporation bias; IGSB, imperfect gold standard bias; SB, spectrum bias; VB, verification bias.
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Discussion
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