Arrhyyhmogenic right ventricular dysplasia; radiologic findings of the left ventricle: A case report and review of the literature

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is characterized by progressive fibro fatty replacement of the right ventricular myocardium which constitutes a substrate for electrical instability and a focus of ventricular arrhythmias. Most of the patients have some degree of left ventricular involvement which also affects the right ventricle by the same disease process. From thirty percent to 50% of patients with ARVD have a family history of the disease. The most common pattern of inheritance is autosomal dominant. Clinically, ARVD usually presents with ventricular arrhythmias, and there is approving evidence that this is the underlying disease in a substantial number of sudden deaths among young, especially healthy individuals. The diagnosis of ARVD relies on the presence of structural and functional abnormalities of the right ventricle, electrophysiological abnormalities, and family history. The imaging modalities are conventional angiography, echocardiography (ECO), radionuclide angiography, ultra fast computed tomography, and magnetic resonance imaging. (MRI). We are presenting a case of ARVD with ECO, computed tomography (CT), and MRI findings.

Keywords

Dysplasia - Arrhythmia - Arrhythmogenic right ventricular dysplasia - Magnetic resonance image - computed tomography - echocardiography

Publikationsverlauf

Artikel online veröffentlicht:
02. August 2021

• References

• 1 Kayser H, Wall E, Sivananthan M, Blooker T, Roos A.Diagnosis of arrhythmogenic right ventricular dysplasia.Radiographics 2002;22:639-650.
• 2 Linstr φm L, Wilkenshoff M, Larsson H, Wranne B.Echocardiographic assessment of arrhythmogenic right ventricular cardiomyopathy. Heart 2001;86:31 -38.
• 3 Corrado D, Basso C, Thiene G.Arrhythmogenic right ventricular cardiomyopath: diagnosis, prognosis, and treatment.Heart 2000;83:588-595.
• 4 Pasquale CG, Heddle WF.Left sided arrhythmogenic ventricular dysplasia in siblings. Heart 2001;86:128-130.
• 5 Kimura F, Sakai F, Sakomura Y, Fujimura M, Ueno E, Matsuda N, Kasanuki H, Mitsuhashi N. Helical CT features of arrhythmogenic right ventricular cardiomyopathy. Radiographics 2002;22:1111-1124.
• 6 Markus F, Towbin J, Zareba W, Moss A, Calkins H, Brown M, Gear K. Arrhythmogenic right ventricular dysplasia/cardiomyopathy.Circulation 2003;107:2975-2978.
• 7 Gilbert-Barness E. Review: Metabolic cardiomyopathy and conduction system defects in children. Annals of clinical & laboratory science 2004;34(1):15-34.
• 8 Fisher NG, Gilbert TJ. Arrhythmogenic right ventricular dysplasia. An illustrated review highlighting developments in the diagnosis and management of this potentially fatal condition. Postgrad med j 2000;76:395-398.
• 9 Basso C, Thiene G, Corrado D, Angelini A, Valente M. Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy, or myocarditis? Circulation 1996;64:983-991.
• 10 Blake LM, Schheinmann MM, Higgins CB. MR features of arrhythmogenic right ventricular dysplasia. AJR 1994;162:809-812.
• 11 White RD, Trohman RG, Flamm SD, et al. Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR imaging of myocardial abnormalities. Radiology 1998;207:743-751.
• 12 Pantanowitz L. Fat infiltration in the heart. Heart 2001;85:253.
• 13 Diamon Y, Watanabe S, Takeda S, Hijikata Y, Komuro I. Two-layered appearance of noncompaction of the ventricular myocardium on magnetic resonance imaging. Circulation j2002;66:619-621.
• 14 Tandri H, Saranathan M, Rodriguez R, Martinez C, Bomma C, Nasir K, Rosen B, Lima J, Calkins Hugh, Bluemke D. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement MRI. Journal of the American College of Cardiology 2005;45:98-103.