We present a 5-year-old child with pulmonary atresia palliated at infancy with midline shunt now for complete repair with aneurysmally dilated aorta. Patient underwent a combined Rastelli procedure with supra-coronary ascending aorta replacement with hemi-arch repair. Patient was discharged on post-operative day 10 and was asymptomatic on follow-up.

Valved bovine jugular vein conduit is considered a suitable choice for paediatric population with congenital heart defect requiring right ventricle to main pulmonary artery connection. However, complications related to the use of this device have been reported, with conduit failure occurring mainly as a consequence of stenosis, conduit thrombosis, and valve regurgitation. We present a case of aneurysmal conduit failure of a valved bovine jugular vein conduit used to reconstruct the right ventricular outflow tract.

The Rastelli operation has been the most common procedure for the repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. A relatively recent approach is the Nikaidoh procedure. Despite the fact that it seems promising, the operation lacks long-term follow-up data. It has been postulated that patients with anomalous coronary arteries are high-risk candidates for the Nikaidoh procedure and its modifications. In this report, we present the case of a patient with transposition of the great arteries with remote restrictive ventricular septal defect and left ventricular outflow tract obstruction with coronary anomaly – with the right coronary artery originating from the left anterior descending coronary artery and crossing the right ventricular outflow tract – who underwent successful modified Nikaidoh operation.

When there is obstruction of the left ventricular outflow tract, there are several surgical approaches to the repair of discordant ventriculo-arterial connections in the setting of concordant atrioventricular connections. Choosing the optimal technique demands not only knowledge of the different surgical procedures, but also the understanding of the particular anatomic features present in a specific patient. These requisites are then essential to plan the operation, to foresee some difficult situations, and to avoid post-operative complications. In this review, we assess all these surgical and anatomic aspects, focussing on their relative importance in clinical assessment.