Korean Circ J.  2010 Aug;40(8):357-367. 10.4070/kcj.2010.40.8.357.

Magnetic Resonance Imaging Assessment of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia in Children

• Yoo SJ
• Grosse-Wortmann L
• Hamilton R

• KMID: 2145526
• DOI: http://doi.org/10.4070/kcj.2010.40.8.357

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetically determined disease that progresses continuously from conception and throughout life. ARVC/D manifests predominantly in young adulthood. Early identification of the concealed cases in childhood is of utmost importance for the prevention of sudden cardiac death later in life. Magnetic resonance imaging (MRI) is routinely requested in patients with a confirmed or suspected diagnosis of ARVC/D and in family members of the patients with ARVC/D. Although the utility of MRI in the assessment of ARVC/D is well recognized in adults, MRI is a low-yield test in children as the anatomical, histological, and functional changes are frequently subtle or not present in the early phase of the disease. MRI findings of ARVC/D include morphologic changes such as right ventricular dilatation, wall thinning, and aneurismal outpouchings, as well as abnormal tissue characteristics such as myocardial fibrosis and fatty infiltration, and functional abnormalities such as global ventricular dysfunction and regional wall motion abnormalities. Among these findings, regional wall motion abnormalities are the most reliable MRI findings both in children and adults, while myocardial fibrosis and fat infiltration are rarely seen in children. Therefore, an MRI protocol should be tailored according to the patient's age and compliance, as well as the presence of other findings, instead of using the protocol that is used for adults. We propose that MRI in children with ARVC/D should focus on the detection of regional wall motion abnormalities and global ventricular function by using a cine imaging sequence and that the sequences for myocardial fat and late gadolinium enhancement of the myocardium are reserved for those who show abnormal findings at cine imaging. Importantly, MRI should be performed and interpreted by experienced examiners to reduce the number of false positive and false negative readings.

Keyword

Arrhythmogenic right ventricular cardiomyopathy/dysplasia; Children; Magnetic resonance imaging

MeSH Terms

Adult
Child
Compliance
Death, Sudden, Cardiac
Dilatation
Fertilization
Fibrosis
Gadolinium
Humans
Magnetic Resonance Imaging
Magnetic Resonance Spectroscopy
Magnetics
Magnets
Myocardium
Reading
Ventricular Dysfunction
Ventricular Function
Gadolinium

Figure

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  Fig. 1 Proposed pathogenesis and natural history of arrhythmogenic right ventricular cardiomyopathy/dysplasia.

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  Fig. 2 Short axis cine MRI images show right ventricular dilatation and randomly oriented prominent trabeculae in the apical part of the right ventricle. The 3-dimensional volume model on the right shows significant discrepancy in right (RV) and left (LV) ventricular volumes.

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  Fig. 3 An axial cine image shows thinning and crinkling of the inferior part of the free wall of the RV. Small outpouchings (arrows) give rise to an appearance of an accordion. RV: right ventricle, LV: left ventricle.

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  Fig. 4 A T1-weighted spin-echo image in axial plane shows a high signal intensity area in the subepicardial region of the posterior wall (arrow) of the left ventricle (LV). High signal intensity (arrow) is also seen in the moderator band of the right ventricle (RV). Both are considered fatty infiltration in the myocardium and the myocardial biopsy demonstrated fatty replacement of the myocardium.

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  Fig. 5 Idiopathic fatty myocardium. Black blood images obtained with the double-inversion recovery technique (upper panels) show high-signal intensity fat (arrow) in the lower part of the interventricular septum. Images obtained with the triple-inversion recovery technique (lower panels) show suppression of the high intensity signals of the subcutaneous and epicardial fat as well as the myocardial fat. The patient had pulmonary atresia with intact ventricular septum and underwent surgical pulmonary valvotomy. The ventricular function was normal.

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  Fig. 6 A late gadolinium enhancement image shows a thinned right ventricular (RV) wall. The apical part of the thinned free wall of RV shows enhancement (arrows). A small area of enhancement (arrow) is also seen in the posterior wall of the left ventricle (LV).

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  Fig. 7 Late gadolinium enhancement images obtained in diastole (left panel) and in systole (right panel) in a normal individual. Note that the myocardium of the free wall of the RV is better defined in systole than in diastole. RV: right ventricle, LV: left ventricle.

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  Fig. 8 Cine images in 2-chamber view of the right ventricle show right ventricular dilatation, poor systolic function, and dyskinesia of the anterior wall of the right ventricular outflow tract (arrow). The right ventricular contour was drawn on the diastolic image as shown in the left panel. The contour was copied and overlain on the systolic image as shown in the right panel. Note the minimal downward excursion of the base of the right ventricle in systole. The anterior wall of the right ventricular outflow tract shows protrusion in systole beyond the limit of right ventricular contour drawn on the diastolic image.

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