title: Fibrofatty Changes: Incidence at Cardiac MR Imaging in Patients with Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy.
abstract: Purpose To determine the incidence of ventricular fatty replacement and late gadolinium enhancement (LGE) at cardiac magnetic resonance (MR) imaging in patients with arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) and the relationship of these findings to disease severity. Materials and Methods This was a retrospective institutional review board-approved HIPAA-compliant study. All subjects provided written informed consent. Seventy-six patients with ARVD/C were enrolled from 2002 to 2012. Quantitative and qualitative cardiac MR imaging analyses of the RV and the left ventricle (LV) were performed to determine cardiac MR imaging-specific Task Force Criteria (TFC) and non-TFC features (ARVD/C-type pattern of fatty infiltration and/or nonischemic pattern LGE). Patients were separated into four groups on the basis of cardiac MR imaging TFC: (a) patients with major cardiac MR imaging criteria, (b) patients with minor criteria, (c) patients with partial criteria, and (d) patients with no criterion. Continuous variables were compared by using the independent Student t test and analysis of variance. Categoric variables were compared by using the Fisher exact test. Results Of 76 patients (mean age, 34.2 years ± 14 [standard deviation]; 51.3% men), 42 met major cardiac MR imaging criteria, seven met minor criteria, seven met partial criteria, and 20 met no criterion. Most probands (36 [80.0%] of 45) met major or minor cardiac MR imaging criteria. Only 13 (41.9%) of 31 family members met any cardiac MR imaging criterion. The most common non-TFC MR imaging features were RV fatty infiltration (28.9%) and LV LGE (35.5%). Non-TFC cardiac MR imaging features were seen in 88.1% of subjects with major criteria, in 28.6% of those with minor criteria, in 71.4% of those with partial criteria, and in 10.0% of those with no criteria. Conclusion In this large cohort of patients with ARVD/C, non-TFC findings of ventricular fatty infiltration and LGE were frequent and were most often found in those who met major cardiac MR imaging criteria and in probands. (©) RSNA, 2016 Online supplemental material is available for this article.
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Neda
authors.initials: N
authors.lastname: Rastegar
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Anneline S J M
authors.initials: AS
authors.lastname: Te Riele
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Cynthia A
authors.initials: CA
authors.lastname: James
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Aditya
authors.initials: A
authors.lastname: Bhonsale
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Brittney
authors.initials: B
authors.lastname: Murray
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Crystal
authors.initials: C
authors.lastname: Tichnell
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: David A
authors.initials: DA
authors.lastname: Bluemke
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Ihab R
authors.initials: IR
authors.lastname: Kamel
authors.affiliation: From The Russell H. Morgan Department of Radiology and Radiological Sciences (N.R., I.R.K., S.L.Z.) and Division of Cardiology (A.S.J.M.T.R., C.A.J., A.B., B.M., C.T., H.C., H.T.), Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD 21205; and Department of Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Md (D.A.B.).
authors.forename: Stefan L
authors.initials: SL
authors.lastname: Zimmerman
mesh_terms: D000328:Adult; D000369:Aged, 80 and over; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000000981:diagnostic imaging* / Q000473:pathology*; D003287:Contrast Media / Q000493:pharmacokinetics; D005260:Female; D005682:Gadolinium / Q000493:pharmacokinetics; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index
chemical_list: D003287:Contrast Media; D005682:Gadolinium