Hypogenetic Lung Syndrome Scimitar Syndrome

In scimitar syndrome one lung is hypoplastic and drained by an anomalous pulmonary vein. The feeding pulmonary artery might be hypoplastic or atretic with systemic collaterals. Abnormal bronchial branching or bronchial hypoplasia in coexistance with abnormal pulmonary segmentation might occur. It is located predominantly on the right and is associated with congenital heart disease in approximately 25% of the patients. Most commonly the atrial septum is involved [22].

If the scimitar vessel drains into the junction of the inferior vena cava with the right atrium a left-to-right shunt is the result [23].

When symptomatic, scimitar syndrome usually occurs in the setting of congestive heart failure. However, the anomaly is often an asymptomatic finding on chest radiographs obtained for other reasons. The typical radiographic sign is an anomalous pulmonary venous trunk coursing vertically along the right atrium toward the right cardio-phrenic angle, imitating the appearance of a scimitar or a sword (Fig. 8). Multiplanar MR imaging can visualize the anatomic structures, a hypoplas-tic lung parenchyma and the abnormal course of the draining vein. For better detection of vascular structures and determination of the venous insertion site CE-MRA is superior to cross sectional imaging.

Scimitar Syndrome Images

Fig. 9. An 8-year-old girl with partial anomalous pulmonary venous return of both upper pulmonary veins. On the MIP reconstruction of the pulmonary CE MRA study (Gd-BOPTA, 0.1 mmol/kg) the upper left pulmonary vein shows a connection to the anonymous vein (arrow) with the connection defined as a so-called vertical vein and which is a remnant of the left superior caval vein. The corresponding right upper pulmonary vein also shows an anomalous connection to the superior caval vein (arrowhead)

Fig. 9. An 8-year-old girl with partial anomalous pulmonary venous return of both upper pulmonary veins. On the MIP reconstruction of the pulmonary CE MRA study (Gd-BOPTA, 0.1 mmol/kg) the upper left pulmonary vein shows a connection to the anonymous vein (arrow) with the connection defined as a so-called vertical vein and which is a remnant of the left superior caval vein. The corresponding right upper pulmonary vein also shows an anomalous connection to the superior caval vein (arrowhead)

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