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Clinical impact of cardiovascular magnetic resonance in evaluation for possible arrhythmogenic right ventricular dysplasia/cardiomyopathy

Background

This study examined the impact of CMR on clinical management in patients with undergoing evaluation for arrhythmogenic right ventricular dysplasia/cardiomyopathy(ARVD/C).

Methods

Patients referred for assessment of ARVD/C were evaluated. Using 2010 ARVD/C Task Force criteria, clinical history, family history, ECG and other test results were evaluated with and without CMR findings to determine definite, borderline or possible ARVD/C. CMR included assessment of right ventricular(RV) size, function, and regional wall motion(RWM). For alternative diagnoses, tissue characterization and late gadolinium enhancement were routinely performed. Qp:Qs was performed when intracardiac shunt was suspected by the supervising physician.

Results

311 consecutive patients (mean age 45±14 years, 53% male) were included. Prior to CMR, patients were classified as definite (n=1), borderline (n=1) or possible ARVD (n=18, Table). After CMR, 6(2%) were diagnosed with definite ARVD/C and underwent defibrillator implantation, 5(2%) were classified as borderline ARVD/C, and 9 (3%) remained possible ARVD/C. 51(16%) had alternative diagnoses (Figure/Table), resulting in a management change: 6(1.9%) patients had intracardiac shunt, 36(11.5%) had another cardiomyopathy or RV overload state, and 9(2.8%) had other diagnoses. 76(24%) had RV enlargement alone with normal RV function and absent RWM by CMR while 164(53%) without other major criteria had normal RV function, size, and RWM.

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Figure 1

Table 1 Clinical Impact of CMR on Diagnosis of ARVD/C vs. Alternative Diagnoses

Conclusions

CMR impacted clinical management by contributing to the diagnosis of definite or borderline ARVD/C in 4% of patients and by excluding the presence of significant RV dysfunction, enlargement, and RWM in over half of patients. CMR identified important alternative diagnoses in 16%.

Funding

No conflicts of interest to disclose.

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Correspondence to Andrew D Choi.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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Choi, A.D., Shanbhag, S.M., Kellman, P. et al. Clinical impact of cardiovascular magnetic resonance in evaluation for possible arrhythmogenic right ventricular dysplasia/cardiomyopathy. J Cardiovasc Magn Reson 17, Q53 (2015). https://doi.org/10.1186/1532-429X-17-S1-Q53

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Keywords

  • Cardiomyopathy
  • Right Ventricular
  • Cardiovascular Magnetic Resonance
  • Clinical Management
  • Late Gadolinium Enhancement