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J Am Coll Cardiol, 2005; 45:98-103, doi:10.1016/j.jacc.2004.09.053
© 2005 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: CARDIAC MAGNETIC RESONANCE

Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging

Harikrishna Tandri, MD*, Manoj Saranathan, PhD{dagger}, E. Rene Rodriguez, MD*, Claudia Martinez, MD*, Chandra Bomma, MD*, Khurram Nasir, MBBS*, Boas Rosen, MD*, João A.C. Lima, MD*, Hugh Calkins, MD* and David A. Bluemke, MD, PhD*,{dagger}

* Division of Cardiology
{dagger} Department of Radiology, The Johns Hopkins University, Baltimore, Maryland

Manuscript received April 10, 2004; revised manuscript received September 17, 2004, accepted September 21, 2004.

* Reprint requests and correspondence: Dr. David A. Bluemke, MRI Building, Room 143, Department of Radiology, The Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, Maryland 21287 (Email: dbluemke{at}jhmi.edu).

OBJECTIVES: We evaluated the role of myocardial delayed-enhancement (MDE) magnetic resonance imaging (MRI) for noninvasive detection of fibrosis in Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).

BACKGROUND: Arrhythmogenic right ventricular dysplasia/cardiomyopathy is characterized by fibro-fatty replacement of the right ventricle (RV) leading to arrhythmias and RV failure. Endomyocardial biopsy can demonstrate fibro-fatty replacement of the RV myocardium; however, the test is invasive and carries a risk of perforation.

METHODS: Thirty consecutive patients were prospectively evaluated for ARVD/C. Magnetic resonance imaging was performed on a 1.5-T scanner. Ten minutes after intravenous administration of 0.2 mmol/kg of gadodiamide, MDE-MRI was obtained. Diagnosis of ARVD/C was based upon the Task Force criteria and did not include MRI findings.

RESULTS: Twelve (40%) of 30 patients met the Task Force criteria for ARVD/C. Eight (67%) of the 12 ARVD/C patients demonstrated increased signal on MDE-MRI in the RV compared with none (0%) of the 18 patients without ARVD/C (p < 0.001). Endomyocardial biopsy was performed in 9 of the 12 ARVD/C patients. Of the nine patients, four had fibro-fatty changes consistent with the diagnosis of ARVD/C. Each of these patients had increased RV signal on MDE-MRI. None of the patients without ARVD/C had any abnormalities either on histopathology or on MDE-MRI. Electrophysiologic testing revealed inducible sustained ventricular tachycardia (VT) in six of the eight ARVD/C patients with delayed enhancement, compared with none of the ARVD/C patients without delayed enhancement (p = 0.01).

CONCLUSIONS: Noninvasive detection of RV myocardial fibro-fatty changes in ARVD/C is possible by MDE-MRI. Magnetic resonance imaging findings had an excellent correlation with histopathology and predicted inducible VT on programmed electrical stimulation, suggesting a possible role in evaluation and diagnosis of patients with suspected ARVD/C.

Abbreviations and Acronyms
  ARVD/C = arrhythmogenic right ventricular dysplasia/cardiomyopathy
  CNR = contrast-to-noise ratio
  ECG = electrocardiogram
  EP = electrophysiologic
  FOV = field of view
  LBBB = left bundle branch block
  MDE = myocardial delayed enhancement
  MRI = magnetic resonance imaging
  ROI = region of interest
  RV = right ventricle
  RVEDV = right ventricular end diastolic volume
  RVEF = right ventricular ejection fraction
  RVOT = right ventricular outflow tract
  VT = ventricular tachycardia




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