TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE INTO THE AZYGOS VEIN

¹ From the Departments of Radiology, Pediatrics, and Surgery, Hospital for Sick Children, and University of Toronto, Toronto, Ontario, Canada

The clinical, roentgenologic, angiocardiographic and operative or postmortem features in 4 cases of total anomalous pulmonary venous drainage into the azygos vein are presented. The findings were affected by the resistance to blood flow in the pulmonary circulation and the size of the interatrial communication. In the presence of a nonobstructed venous return, there was cardiomegaly, increased pulmonary artery vascularity, mild cyanosis, moderate dyspnea, cardiac failure and hepatomegaly. When venous return was obstructed, the heart was of normal or near-normal size, pulmonary venous congestion and edema were prominent without pulmonary plethora, cyanosis and dyspnea were more pronounced and there was cardiac failure and hepatomegaly. Death in the latter group tended to occur early.

Roentgenologically, the presence of a mass in the right tracheobronchial angle was noted in 2 cases in which venous obstruction was absent or mild. This was shown to represent a dilated azygos vein arch alone or in combination with the terminal portion of a dilated anomalous pulmonary channel draining the lungs. When the anomalous pathway was long or very narrow with resultant moderate to severe obstruction, no mass was visible on the plain roentgenogram, as the azygos vein was not distended. Selective angiography showed the anatomy of the anomalous venous return in 3 cases in which this procedure was performed and the results were correlated with the surgical or postmortem findings.

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