Author + information
- Received April 10, 2004
- Revision received September 17, 2004
- Accepted September 21, 2004
- Published online January 4, 2005.
- Harikrishna Tandri, MD*,
- Manoj Saranathan, PhD†,
- 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*,† ()
- ↵*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
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.
The Johns Hopkins ARVD program is funded by a private grant from the Bogle Foundation and the National Institutes of Health Research Grant 1 UO1 HL65594-01A1.
- Received April 10, 2004.
- Revision received September 17, 2004.
- Accepted September 21, 2004.
- American College of Cardiology Foundation