Usefulness of Combined Functional Assessment by Cardiac Magnetic Resonance and Tissue Characterization Versus Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy

doi:10.1016/j.amjcard.2016.08.056

The American Journal of Cardiology

Volume 118, Issue 11, 1 December 2016, Pages 1730-1736

https://doi.org/10.1016/j.amjcard.2016.08.056 Get rights and content

Current task force criteria (TFC) of cardiac magnetic resonance (CMR) for the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC/D) were generated by comparing probands (mean age of 44 years) to healthy participants of the multi-ethnic study of atherosclerosis (mean age of 60 years). These age differences may be a selection bias because right ventricular end-diastolic volume index decreases 4.6% per decade. Moreover, fat infiltration and late gadolinium enhancement were not included. We evaluated the diagnostic accuracy of TFC using the same methodology used by the task force but comparing probands and age- and gender-matched healthy controls and considering also other morphofunctional and tissue abnormalities detected by CMR. Forty-seven probands with previous diagnosis of ARVC/D (excluding probands if CMR was used for diagnosis) were compared with 216 age- and gender-matched healthy controls. TFC had optimal specificity (100%) but poor sensitivity (20% for major and 13% for minor criteria). The presence of any pre- and post-contrast signal abnormalities had 100% specificity and 81% sensitivity. The best diagnostic accuracy (98%) was achieved by the combined evaluation of any right ventricular wall motion abnormality (excluding hypokinesia) with any signal abnormality (including left ventricular fat infiltration and late gadolinium enhancement) yielding a 100% specificity and 96% sensitivity. Left ventricular was involved in 45% of the probands. Current TFC for CMR presented optimal specificity but poor sensitivity to identify patient with ARVC/D. Signal and wall motion parameters of CMR should be considered together to achieve the best diagnostic accuracy for the diagnosis of ARVC/D.

Section snippets

Methods

Forty-seven probands with previous diagnosis of ARVC/D were enrolled. To eliminate selection bias, we excluded probands if CMR was used for the diagnosis of the individual patient. A control group of 216 healthy subjects was included in the study with the following inclusion criteria: (1) absence of contraindications to CMR; (2) no personal history of cardiac and noncardiac disease; (3) no family history of genetic disease; (4) normal physical examination; (5) normal electrocardiogram; (6)

Results

The ARVC/D probands included 47 patients (29 men, mean ages 46 ± 17 years). The diagnosis of ARVC/D in probands is presented in Table 1. The population of healthy controls included 216 subjects (132 men, mean age 44 ± 20 years). No significant differences of age (p 0.54) and gender (p = 0.9) were found between the 2 groups.

CMR results are reported in Tables 2 and 3. Among the men, the RV EDVi ≥110 ml/m² was found in 3 probands (10%) and 10 controls (7.5%) (p = 0.65); an RV EDVì ≥100 and <110 in

Discussion

The main results of this study include: (1) TFC of CMR showed an optimal specificity (100%) but a poor sensitivity to identify probands; (2) RV dilation had low sensitivity and suboptimal specificity because it was found only in a minority of probands and in some healthy controls who practiced noncompetitive sports; (3) the highest accuracy (98%) was found when combining criteria including RV WM abnormalities and or any signal abnormalities (including LV fat infiltration and LGE).

The low

Disclosures

The authors have no conflicts of interest to disclose.

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CardiomyopathyUsefulness of Combined Functional Assessment by Cardiac Magnetic Resonance and Tissue Characterization Versus Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy

Section snippets

Methods

Results

Discussion

Disclosures

J Am Coll Cardiol

J Cardiovasc Magn Reson

Am J Cardiol

JACC Cardiovasc Imaging

J Am Coll Cardiol

J Am Coll Cardiol

Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia proposed modification of the task force criteria

Circulation

Reference right ventricular systolic and diastolic function normalized to age, gender and body surface area from steady-state free precession cardiovascular magnetic resonance

Eur Heart J

Cardiac cine MRI: quantification of the relationship between fast gradient echo and steady-state free precession for determination of myocardial mass and volumes

J Magn Reson Imaging

Cardiomyopathy
Usefulness of Combined Functional Assessment by Cardiac Magnetic Resonance and Tissue Characterization Versus Task Force Criteria for Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy