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Uhl’s anomaly of the right ventricle is an unusual cardiac disorder with almost complete absence of right ventricular myocardium, normal tricuspid valve, and preserved septal and left ventricular myocardium. A 12 year old boy was admitted with the complaint of fatigue with effort. A holosystolic murmur was heard on left lower sternal border. Electrocardiography showed right atrial dilation and low voltage in all leads. Cardiomegaly was apparent on chest x ray. Echocardiography revealed a greatly enlarged right atrium and ventricle. The right ventricular free wall seemed very thin.
Electrocardiographic gated multislice computed tomography (CT) showed an extremely thin walled right ventricle with almost complete absence of right ventricular free wall myocardium (panels A and B below). The interventricular septum and left ventricular myocardium were normal. Although magnetic resonance imaging can also show the absence of right ventricular myocardium, three dimensional reconstructed multislice CT imaging (right panel) makes it easier to see the difference between the normal left ventricular myocardium and the thin walled right ventricle.
This is the first case of Uhl’s anomaly that has been demonstrated by multislice CT, and the images suggest that multislice CT can be used for definition of many cardiac pathologies.