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Abstract

Cardiovascular imaging is a rapidly evolving field that requires familiarity with the appearances of pediatric and adult cardiovascular diseases on chest radiographs as well as images obtained with computed tomography, magnetic resonance imaging, and angiography. To accurately identify congenital abnormalities affecting the heart and vessels of the thorax, radiologists must recognize the imaging features and understand their pathophysiologic origin. The cardiovascular imaging signs of congenital anomalies that are most often seen in radiologic practice include the egg on a string (seen in transposition of the great arteries), snowman (total anomalous pulmonary venous return), scimitar (partial anomalous pulmonary venous return), gooseneck (endocardial cushion defect), figure of three and reverse figure of three (aortic coarctation), boot-shaped heart (tetralogy of Fallot), and box-shaped heart (Ebstein anomaly). (c) RSNA, 2007.

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Most cited references 16

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Venous anomalies of the thorax.

C. Anders Olson, H V Posniak, Brittany Pierce … (2004)

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Morphological aspects of Ebstein's anomaly in adults.

G Thiene, A Angelini, C Frescura … (2000)

The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.