The surgical treatment of transposition of the great arteries, ventricular septal defect, and significant left ventricular outflow tract obstruction continues to evolve. The survival of an unrepaired transposition of the great arteries into late adulthood is a rarity. Even when large intracardiac shunts are present, it remains a lethal cyanotic CHD if it is not surgically corrected soon after birth. We present our experience of two cases, both of whom underwent a single-stage arterial switch operation and an aortic valve replacement for this defect.

Pulmonary hypertension with transposition of the great arteries is associated with significant morbidity and mortality. At the worst end of the spectrum are patients who undergo extracorporeal support perioperatively. We describe our experience with three patients who received preoperative extracorporeal support and separated from cardiopulmonary bypass successfully on conventional postoperative care, with no significant deficits on follow-up.

A neonate with transposition of the great arteries and intact ventricular septum presented without pulmonary over-circulation, and subsequently developed pulmonary haemorrhage after corrective surgery. Postoperative CT revealed an aortopulmonary collateral artery arising from the descending aorta, and we performed successful embolisation on postoperative day 9. Aggressive imaging modalities such as angiography and/or CT imaging with contrast can detect unexpected extra-pulmonary blood supply and guide further management.

Arterial switch surgery for d-transposition of great arteries is usually performed in the first 2 weeks of life before the left ventricle regresses. The arterial switch surgery that helps achieve anatomic and physiologic correction of this condition has better long-term outcomes than other surgical approaches. The procedure may still be attempted at an older age where the left ventricle has not regressed. We report a rare case of a 12-year-old child with d-transposition of great arteries, a remote muscular ventricular septal defect and isolated valvar pulmonic stenosis, which was corrected by an arterial switch surgery.

A ventricular septal defect in transposition of the great arteries is frequently closely related to the cardiac valves. The valvar function after arterial switch operation of patients with transposition of the great arteries and ventricular septal defect or intact ventricular septum was compared. We analysed the function of all cardiac valves in patients who underwent the arterial switch operations pre- and post-operatively, 1 year after the procedure and on follow-up. The study included 92 patients – 64 with transposition of the great arteries/intact ventricular septum and 28 with transposition of the great arteries/ventricular septal defect. The median age at surgery was 5.5 days in transposition of the great arteries/intact ventricular septum (0–73 days) and 7.0 days in transposition of the great arteries/ventricular septal defect (4–41 days). Follow-up was 51.7 months in transposition of the great arteries/intact ventricular septum (3.3–177.3 months) and 55 months in transposition of the great arteries/ventricular septal defect (14.6–164.7 months). Neo-aortic, neo-pulmonary, and mitral valvar function did not differ. Tricuspid regurgitation was more frequent 1 year post-operatively in transposition of the great arteries/ventricular septal defect (n = 4) than in transposition of the great arteries/intact ventricular septum. The prevalence of neo-aortic regurgitation and pulmonary stenosis increased over time, especially in patients with transposition of the great arteries/intact ventricular septum. The presence of a ventricular septal defect in patients undergoing arterial switch operation for transposition of the great arteries only has a minor bearing for the development of valvar dysfunction on the longer follow-up.

Despite early arterial switch repair of complete transposition of the great vessels, significant physiologic aberrations may still occur. Two cases of vascular disease in the left pulmonary arterial system are described and potential mechanisms are discussed. We conclude a small number of children with simple transpostion may develop asymmetric patterns of pulmonary blood flow without evidence of anatomic narrowing of either arterial vessel.

The arterial switch operation has become the standard of surgical repair for total correction of complete transposition (concordant atrioventricular and discordant ventriculoarterial connections). A standard nomenclature for classification of the numerous anatomic relationships the coronary arteries may assume, however, has yet to be universally adopted. Inconsistent use of varied nomenclatures remain an obstacle to effective communication between physicians and surgeons. In this review we offer a unified nomenclature incorporating five important relationships: the configuration of the sinuses; their location; the configuration of the arterial orifices; the pattern of branching of the major coronary arteries; and the course of each artery.

Surgical banding of the pulmonary artery in patients with transposition of the great arteries leads to a rapid increase of the left ventricular mass. The purpose of this animal experiment was to induce ventricular systolic hypertension in the “subpulmonary ventriclerdquo; by intravascular obstruction to pulmonary flow. The first experiment was performed in an anesthetized animal with an open thorax. A balloon-tipped catheter was introduced into the femoral vein and advanced to the pulmonary artery. The balloon was positioned in the pulmonary trunk and was progressively inflated until a systemic pressure was achieved in the “pulmonary ventricle”. This was accomplished within 40 minutes without a significant reduction in cardiac output, which was continuously monitored by an electromagnetic flowprobe. In the second experiment an analogous procedure was performed in a closed chest, awake, spontaneously breathing animal. The catheter was maintained in proper place for 24 hours. Subsequent macroscopic examination of the heart demonstrated a remarkable increase in the ventricular mass. The results of this preliminary experiment demonstrate the possibility for creating “intraluminal pulmonary banding”. The potential advantages of this technique are the nonsurgical approach and the ability to adjust the grade of obstruction to pulmonary flow.

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.