Noninvasive Multimodality Imaging in ARVD/C.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial cardiomyopathy resulting in progressive right ventricular (RV) dysfunction and malignant ventricular arrhythmias. Although ARVD/C is generally considered an inherited cardiomyopathy, the arrhythmogenic nature of the disease is striking. Affected individuals typically present in the second to fourth decade of life with arrhythmias originating from the right ventricle. Over the past decade, pathogenic ARVD/C-causing mutations have been identified in 5 genes encoding the cardiac desmosome. Disruption of the desmosomal connection system between cardiomyocytes may be represented structurally by ventricular enlargement, global or regional contraction abnormalities, RV aneurysms, or fibrofatty replacement. These abnormalities are typically observed in predilection areas, including the subtricuspid region, basal RV free wall, and left ventricular posterolateral wall. As such, structural and functional abnormalities on cardiac imaging constitute an important diagnostic criterion for the disease. This paper discusses the current status and role of echocardiography, cardiac magnetic resonance imaging, and computed tomography for suspected ARVD/C.
Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Department of Medicine, Division of Cardiology, University Medical Center Utrecht, Utrecht, the Netherlands.
Anneline S J M
ASJM
Te Riele
Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Harikrishna
H
Tandri
Department of Medicine, Division of Cardiology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
Danita M
DM
Sanborn
Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, Maryland; Radiology and Imaging Sciences, National Institutes of Health Clinical Center, Bethesda, Maryland. Electronic address: david.bluemke@nih.gov.
David A
DA
Bluemke
D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D004452:Echocardiography; D006801:Humans; D008279:Magnetic Resonance Imaging; D064847:Multimodal Imaging / Q000379:methods*; D009206:Myocardium / Q000473:pathology*; D011237:Predictive Value of Tests; D011379:Prognosis; D014057:Tomography, X-Ray Computed; D016278:Ventricular Function, Right
arrhythmogenic right ventricular dysplasia/cardiomyopathy; cardiac magnetic resonance; computed tomography; echocardiography; imaging