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

Division of Cardiology, The Johns Hopkins University, Baltimore, MD 21287, USA.
Journal of the American College of Cardiology (Impact Factor: 16.5). 02/2005; 45(1):98-103. DOI: 10.1016/j.jacc.2004.09.053
Source: PubMed


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).
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.
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.
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).
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.

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    • "CMR with LGE can detect intramyocardial fibrosis and this finding can precede functional abnormalities, potentially allowing for better detection of disease at an early stage than with TFC.9,15 However the finding of LGE is well described in a wide variety of pathologies, including dilated cardiomyopathy, in acute and chronic myocarditis, in pulmonary hypertension and, of course, in ischaemic heart disease.16–20 This is reflected in our study by 3 patients who had LGE on CMR and had diagnoses other than ARVC. "
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    ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare but important cause of sudden cardiac death. We investigated the role of cardiac magnetic resonance imaging (CMR) in the evaluation of patients with suspected ARVC referred by a general cardiology service. Ninety-two patients (mean age 48 ± 15, 49% female), referred for CMR assessment of possible ARVC, were reviewed. CMR included both functional and tissue characteristic imaging. No patients had ARVC based on the 1994 Task Force Criteria (TFC) prior to CMR, but 4 met proposed Modified TFC; 15% met one major (±1 minor) TFC, 71% 1 or 2 minor TFC, and 14% no TFC. Reasons for CMR referral included symptomatic arrhythmia of likely RV origin (28%), Electrocardiogram/Holter abnormalities (28%), echocardiographic features suspicious of ARVC (19%), and family history of ARVC (8%). CMR findings strongly suggestive of ARVC were found in nine patients (10%), although only three were considered typical. Of these patients two met 1 major TFC and seven met 1 or 2 minor TFC. CMR findings included RV thinning, aneurysm, and diastolic out-pouching, but only 1 patient had definite fatty infiltration of the RV. Incidentally, CMR detected important, previously undiagnosed pathology, including anomalous pulmonary venous drainage (2 patients) and non-ischaemic cardiomyopathy (6%). CMR was normal in 63%, with minor abnormalities in 29%. CMR may play an important diagnostic role in the evaluation of possible ARVC. Patients who do not meet TFC for diagnosis may have CMR features typical of ARVC. Additionally CMR may detect other hitherto undiagnosed structural or functional abnormalities that alter patient management. However the majority of patients referred have a low pretest probability of ARVC, and the rate of normal CMR scans is high.
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    • "Although the prognostic value of CMR is still under investigation, LGE appears to predict inducibility of sustained ventricular tachycardia and occurrence of RV dysfunction [100]. However, lack of specificity of LGE pattern combined with revision of taskforce imaging criteria (requiring the combination of wall motion abnormality with global RV dilatation or dysfunction) may not have improved the sensitivity of detection of ARVC despite keeping high specificity [101]. "
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    • "Over the past decade late gadolinium-enhanced MRI has become established as one of the most useful techniques for non-invasive measurement of myocardial viability [43], [44], [45], [46], [47], [48]. In recent years late gadolinium-enhanced MRI has been able to detect fibrosis in patients with hypertrophic [49] and dilated [50] cardiomyopathy, systemic vasculitus [43], arrhythmogenic right ventricular disease [45] and DMD [25], [26], [27] and Becker muscular dystrophy [22], [27]. "
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