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Partial anomalous pulmonary venous connection


Partial anomalous pulmonary venous connection is a group of anomalies in which the venous drainage from part or all of one lung is connected to the right atrium or to one of the major systemic veins leading to the right atrium.

Morphology

The three most common forms of partial anomalous pulmonary venous connection are discussed in this chapter, including a description of the morphology of each. Computed tomography (CT) scans and operative photographs are used to demonstrate the morphologic appearance.

Figure 7-1, A Contrast-enhanced CT scan of partial anomalous pulmonary venous connection—right superior pulmonary vein to superior vena cava. The right upper lobe branch of the right superior pulmonary vein is connected to the superior vena cava. B Contrast-enhanced CT scan of partial anomalous pulmonary venous connection—right superior pulmonary vein to superior vena cava. The right middle lobe branch of the superior pulmonary vein is hypoplastic and connects normally to the left atrium. Hypoplasia of the right middle lobe vein is appreciated when compared with the normal-sized, unobstructed left superior pulmonary vein. This rare variant of partial anomalous pulmonary venous connection results in pulmonary venous obstruction from the right middle lobe. Collateral venous drainage is by way of the mediastinum, causing esophageal and tracheal varices associated with bleeding. C Operative photograph of partial anomalous pulmonary venous connection—right superior pulmonary vein to superior vena cava. The right superior pulmonary vein is connected to the superior vena cava above the cavoatrial junction. The superior vena cava is enlarged due to the extra volume of blood flow entering the cava from the right superior pulmonary vein. D Partial anomalous pulmonary venous connection—left pulmonary vein to innominate vein—viewed through a left thoracotomy. Branches of the left pulmonary vein are connected to a vein coursing vertically outside the pericardial sac. This vertical vein is the remnant of the left superior vena cava. It drains to the innominate (left brachiocephalic) vein. Pulmonary vein branches are controlled with vessel loops. E Operative repair of partial anomalous pulmonary venous connection—left pulmonary vein to innominate vein. The vertical vein (left pulmonary vein) is rotated inferiorly and medially for anastomosis to the left atrial appendage, using a flap of the appendage to create a large anastomosis.

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