A 14-year-old boy with the asplenia-cardiovascular anomaly syndrome is reported. In addition to dextrocardia and multiple intracardiac defects, which are typical of this syndrome, he had total anomalous pulmonary venous connection to a right ascending vertical vein. Severe stenosis of the innominate vein caused diversion of pulmonary venous flow through the cerebral venous system before it reached the heart, and resulted in both pulmonary venous and cerebral venous hypertension. Symptomatic improvement occurred after operative removal of the innominate obstruction.
Reynolds JL, Chua FL, Hewitt RL. Intracerebral Pulmonary Venous Drainage. Am J Dis Child. 1973;125(6):871-875. doi:10.1001/archpedi.1973.04160060071017