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

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Journal of Cardiovascular Magnetic Resonance201517 (Suppl 1) :Q53

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


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


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.


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.

Figure 1

Table 1

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


2010 Guidelines without CMR Findings, n (%)

2010 Guidelines with CMR Findings, n (%)

Definite Criteria for ARVD/C

1 (0.3)

6 (2)

Borderline Criteria for ARVD/C

1 (0.3)

5 (2)

Possible Criteria for ARVD/C

18 (5.8)

9 (3)

Patients with 1 or no Minor Criteria, not meeting 2010 Guidelines Definition of \"Definite\", \"Borderline\" or \"Possible\"

291 (93.6)

51 (16)

Alternate Diagnosis*

76 (24)

RV Enlargement Alone**

164 (53)

Normal RV***

* Alternative Diagnosis Resulting in Change in Management included 6 (1.9%) Intracardiac shunts, other Cardiomyopathy or RV Overload State 36 (11.5%), or Other Diagnosis 9 (2.8%)

**RV Enlargement Alone with normal RV Function and Regional Wall Motion

*** Normal RV Function, Size and Regional Wall Motion by CMR


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%.


No conflicts of interest to disclose.

Authors’ Affiliations

Advanced Cardiovascular Imaging Laboratory, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA
Division of Cardiology, Medstar Washington Hospital Center, Washington, DC, USA


© Choi et al; licensee BioMed Central Ltd. 2015

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 (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.