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J Am Coll Cardiol, 2006; 48:2132-2140, doi:10.1016/j.jacc.2006.07.045 (Published online 31 October 2006).
© 2006 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

Cardiovascular Magnetic Resonance in Arrhythmogenic Right Ventricular Cardiomyopathy Revisited

Comparison With Task Force Criteria and Genotype

Srijita Sen-Chowdhry, MA, MBBS, MRCP*,{dagger},*, Sanjay K. Prasad, MD, MRCP{dagger}, Petros Syrris, PhD*, Ricardo Wage, DCR(R){dagger}, Deirdre Ward, MBBS, MRCPI*, Robert Merrifield, PhD{ddagger}, Gillian C. Smith, MSc{dagger}, David N. Firmin, PhD{dagger}, Dudley J. Pennell, MD, FACC{dagger} and William J. McKenna, MD, DSc, FACC*

* Cardiology In The Young, The Heart Hospital, University College London, London, United Kingdom
{dagger} Cardiovascular Magnetic Resonance Unit, National Heart and Lung Institute, Imperial College, London, United Kingdom
{ddagger} Wolfson Foundation Medical Image Computing Laboratory, Imperial College, London, United Kingdom

Manuscript received February 27, 2006; revised manuscript received July 12, 2006, accepted July 23, 2006.

* Reprint requests and correspondence: Dr. Srijita Sen-Chowdhry or Professor William J. McKenna, Cardiology In The Young, The Heart Hospital, 16–18 Westmoreland Street, London W1G 8PH, United Kingdom. (Email: srijita{at}doctors.org.uk).

OBJECTIVES: We sought to assess the utility of cardiovascular magnetic resonance (CMR) in the evaluation of arrhythmogenic right ventricular cardiomyopathy (ARVC) in relation to diagnostic criteria and genotype.

BACKGROUND: Timely diagnosis of ARVC is difficult as clinical findings may be subtle and nonspecific in early disease. The role of CMR is controversial owing to the absence of a standardized protocol, insufficient experience with the modality, and inherent difficulties in imaging the right ventricle.

METHODS: Comprehensive CMR examination was performed in 232 patients undergoing evaluation for suspected ARVC. CMR outcomes were compared with: 1) prospective clinical diagnosis using Task Force guidelines, with and without the proposed modifications for familial ARVC; and 2) gene-carrier status in 35 individuals from genotyped families.

RESULTS: CMR studies were positive in all 64 patients who prospectively fulfilled Task Force criteria, resulting in 100% sensitivity. Specificity in relation to Task Force criteria was low (29%). Of the 119 apparent false positives detected by CMR, however, 63 fulfilled modified diagnostic criteria for familial ARVC and 7 were obligate gene carriers, suggesting that CMR frequently identifies individuals with early disease, in whom Task Force criteria are relatively insensitive. This was borne out by evaluation of genotyped individuals (26 gene-positive and 9 gene-negative), in whom CMR had a sensitivity of 96% and a specificity of 78%.

CONCLUSIONS: CMR is a valuable component of the diagnostic workup for ARVC when performed with a dedicated protocol by specialists with experience in analysis of volumes, right ventricular wall motion, and delayed-enhancement imaging.

Abbreviations and Acronyms
  ARVC = arrhythmogenic right ventricular cardiomyopathy
  CMR = cardiovascular magnetic resonance
  ECG = electrocardiogram
  LE = late enhancement
  LV = left ventricle/left ventricular
  ROC = receiver-operating characteristic
  RV = right ventricle/right ventricular
  RWMA = regional wall motion abnormalities
  SCD = sudden cardiac death
  TF = Task Force




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