Transposition of the great arteries combined with totally anomalous pulmonary venous connection is extremely rare outside of heterotaxy syndrome. Most reported cases have been treated by a modified atrial switch operation. We report the successful treatment of a neonate with this rare association, repaired by arterial switch operation and connection of the pulmonary venous return to the left atrium.

We report three cases of pulmonary venous stenosis developing after repair of totally anomalous pulmonary venous connection. In each case, we used steroids as a therapeutic measure following re-operation. In two cases, we were successful in preventing significant re-stenosis. The third patient rapidly developed stenosis (mean pulmonary wedge pressure approximately 20 mmHg at cardiac catheterisation) after stopping steroids. This patient subsequently died after a third operation. The remaining patients continue to do well.

Totally anomalous pulmonary venous connection to the azygos vein is a rare congenital heart malformation in which all the pulmonary venous blood returns anomalously to the azygos vein. Among 111 consecutive patients with totally anomalous pulmonary venous connection undergoing surgical correction at our institution between June 1982 and September 1997, this malformation was present in seven cases. By echocardiography, using a subxyphoid short-axis view at the atrial level and a modified suprasternal sagittal view, the malformation was diagnosed when the pulmonary venous confluence was traced posteriorly and superiorly relative to the right pulmonary artery and right bronchus, finally reaching reach the superior caval vein. Totally anomalous pulmonary venous connection to the azygos vein was misdiagnosed in the first two patients, both by echocardiography and angiocardiography. In the subsequent five patients, a precise diagnosis was obtained by echocardiography. Echocardiography, therefore, can be considered an accurate diagnostic tool permitting recognition of totally anomalous pulmonary venous connection to the azygos vein, and permitting corrective surgery without recourse to catheterization and angiography.

We describe post azygos obstruction of the superior caval vein following repair of a mixed variety of totally anomalous pulmonary venous connection. Balloon dilation was immediately successful, and has provided persistent relief at a follow-up of 20 months.

Scimitar syndrome is a rare congenital anomaly that affects classically the right lung and the heart. We present a rare variant that involves both lungs in association with totally anomalous pulmonary venous connection, horseshoe lung, and right pulmonary cyst.