When Fontan and Baudet devised what has become known as the Fontan operation, they understandably assumed that inflow and outflow valves should be included to achieve a pump-like action of the subpulmonary right atrial cavity. Over the following years, however, it became apparent that valves did not function satisfactorily in this situation. Worse, the implanted valves had a tendency to become obstructive, which often lead to critical elevation of the already raised systemic venous pressure. Surgeons gradually realised that the outcomes of surgery designed to create the Fontan circulation were likely to be better without inclusion of valves in the subpulmonary right atrium, and they stopped putting them in.

We describe an individualised approach in the rare situation of a tunnel between the left aortic sinus of Valsalva and the junction of the superior caval vein with the right atrium. It is necessary, in this rare anomaly, to identify the origin of the left coronary artery prior to intervention.

We describe an occurrence of long QT syndrome in association with persistent patency of the arterial duct in members of a family. Patency of the arterial duct was diagnosed in family members of 4 successive generations. The index case had long QT syndrome associated with such a patent duct. Another patient had long QT syndrome, but associated with a ventricular septal defect. We postulate that there may be a common genetic mechanism for long QT syndrome and persistent patency of the arterial duct.

The single beat method was used to evaluate right and left ventriculo-arterial coupling in an asymptomatic patient with congenitally corrected transposition. The ratio of ventricular end-systolic to arterial elastances was normal for the left ventricle coupled to the pulmonary circulation, and depressed for the right ventricle coupled to the systemic circulation. This result suggests that chronic uncoupling of the right ventricle to the systemic circulation might play a role in the pathophysiology produced by congenitally corrected transposition.

We describe an infant with duct-dependent cardiac disease diagnosed prenatally who was born prematurely, and at extremely low weight. Treatment by infusion of prostaglandin maintained ductal patency for 66 days, permitting weight to be gained whilst under the care of a regional unit for neonatal intensive care prior to transfer for palliative cardiac surgery.

We treated successfully using the Ross procedure a 14-year old with a congenitally stenotic bifoliate aortic valve associated with anomalous origin of the right coronary artery. The anomalous artery arose from the same aortic sinus that gave rise to the main stem of the left coronary artery, and reached the right atrioventricular groove by traversing the tissue plane between the aortic root and the subpulmonary infundibulum. Both coronary arteries were reimplanted using a single arterial button.

We report a fatal dissection of the descending aorta as a complication after a two-staged implantation of a stent to relieve aortic coarctation in a young female with Turner's syndrome. Implantation of the stent, with dilation up to 70 percent, and half a year later re-dilation to 100 percent, was without any complication. A week after the re-dilation, however, the patient suffered acute dissection of the descending aorta. We initially stabilized the situation, but some days later her condition deteriorated, and she died. We discuss the various options for treating coarctation and re-coarctation. As far as we know, this is the first description of implantation of a stent in the setting of coarctation in Turner's syndrome, albeit with an unfortunate conclusion.

The arterial switch operation has become the preferred surgical procedure for the management of simple and complex transposition, defined on the basis of concordant atrioventricular but discordant ventriculo-arterial connections, with excellent results over the short-term, and thus far over the long-term. When transposition is associated with a ventricular septal defect, and there is additional obstruction within the left ventricular outflow tract, the complexity of the surgical repair is increased, and the surgical options are not as attractive.

Over a period of 6 days, a three-week-old male developed a long-segment coarctation, with sub-total obstruction of the descending aorta, immediately distal to the left subclavian artery. On the 24th day of life, the stenotic region was repaired by placement of a pulmonary allograft patch measuring 3 centimetres in length. Severe diffuse vascular medial thickening was discovered at the operation. Subsequent fluorescence in-situ hybridization proved positive for Williams' syndrome. To our knowledge, this is the first report of rapidly progressive infantile arteriopathy in the setting of Williams' syndrome.

An 18-month-old boy, referred because of an infection of the airways, was found to have a right-sided heart, a hypoplastic right lung, absence of the right pulmonary artery, and persistence of the hepatic venous plexus. The benign association of this unusual variant of the scimitar syndrome and persistence of the hepatic venous plexus needs to be recognised in order to avoid extensive investigations and surgery involving the inferior caval vein.

As far as we are aware, right-sided bacterial endocarditis has not previously been described as presenting with systemic illness and testicular swelling. We report a teenager who presented with this unusual combination as a consequence of right-sided endocarditis. He presented with high fever, with chills and rigor, along with painful enlargement of the left testicle, a productive cough with progressive breathlessness, and joint pains. His blood culture was positive for Staphylococcus aureus, and a computerised tomographic scan of the chest revealed multiple pulmonary emboluses. Ultrasound of the testicles showed features of inflammation, and an echocardiogram revealed a vegetation on the tricuspid valve.

Numerous techniques have been described for translocation of the coronary arteries in the setting of the arterial switch procedure. In the case we describe, the close alignment to the aortic and the pulmonary wall of the main stem supplying the left anterior descending and circumflex arteries, and dual origin of this vessel and the right coronary artery from sinus #2, required an individual approach. After taking down the main stem feeding the left anterior descending and circumflex arteries in the usual manner, we opted for a connection maintaining the artery in its existing site, and punched a hole into the neo-aorta just in front of the coronary orifice. We then used a piece of glutaraldehyde-preserved pericardium as a hood, anastomosing the right coronary artery to the new aorta by means of the usual trap door technique.

We report the rare combination of a severely hypoplastic left ventricle and discordant ventriculoarterial connections, with associated subpulmonary obstruction of the left ventricular outflow tract. The long tortuous arterial duct originated at an acute angle from the aorta, suggesting that the subpulmonary obstruction developed early in fetal life. Residual flow via the hypoplastic left ventricle to the pulmonary circulation after insertion of an aortopulmonary shunt resulted in haemodynamic deterioration, which was instantly reversed by closing the pulmonary trunk.

After repair of an atrioventricular septal defect with common atrioventricular junction in a 2-month-old girl, rapid atrial tachycardia, in combination with junctional ectopic tachycardia, led to severe postoperative cardiovascular compromise. Intercurrent runs of ectopic atrial tachycardia made atrial pacing impossible, despite high doses of intravenous amiodarone. Following the addition of flecainide to the infusion, we were able to control the rhythm, and when combined with atrial pacing, this led to an immediate haemodynamic improvement. Treatment of refractory supraventricular tachycardias with amiodarone combined with flecainide can be very effective in the setting of postoperative cardiac intensive care.

The presence of an obstructed aortic arch in patients with transposition, or the Taussig-Bing variant of double outlet right ventricle, presents a formidable surgical challenge. Over the years, there have been several controversies with respect to primary versus staged repair, the best technique for reconstruction of the aortic arch, and whether to use circulatory arrest or antegrade regional cerebral perfusion. In this review, I will address all these issues and describe my favoured surgical approach at The Montreal Children's Hospital, namely the single-stage arterial switch operation with concomitant repair of the aortic arch with a patch fashioned from a pulmonary homograft, all conducted using antegrade regional cerebral perfusion.

The arterial switch operation is now recognized as the procedure of choice for treatment of infants and children born with the combination of concordant atrioventricular and discordant ventriculo-arterial connections, usually known simply as transposition. Although the Mustard and Senning atrial switch procedures achieved widespread acceptance and success, the accumulating observations at mid- to late follow-up of, first, the increasing frequency of arrhythmias and sudden death, second, late right ventricular dysfunction and severe tricuspid regurgitation and, third, high operative mortality in such patients with ventricular septal defects, have led to the worldwide use of the arterial switch operation for both simple and complex forms of transposition.1

Radiofrequency lesions can, theoretically, be the substrate for new persistent arrhythmias. As far as we know, this has never previously been encountered after transcatheter ablation of accessory pathways. A child with Wolff–Parkinson–White syndrome was referred for radiofrequency catheter ablation of a left-sided accessory pathway. After successful ablation of the accessory pathway using a retrograde transaortic approach, the child developed an incessant wide QRS complex tachycardia at slow rate that was resistant to pharmacologic interventions. The focus of the tachycardia was identical to the ventricular site of insertion of the eliminated accessory pathway.

Aorto-left ventricular tunnels are anomalous communications between the ascending aorta and the left ventricle. Prenatal diagnosis, followed by successful surgical management, is rare. We describe a fetus diagnosed with aorto-left ventricular tunnel at 35 weeks who achieved a favourable outcome after immediate postnatal surgical repair.

We describe a 6-year-old boy with a right-sided heart part of the Kartagener syndrome, complicated by presence of a perimembranous ventricular septal defect. The defect was closed interventionally using the Amplatzer™ asymmetrical occluder for ventricular septal defects. The procedure was event-free. Non-operative closure of a ventricular septal defect in this patient demonstrated no other difficulty than mirror-imaged thinking, thus giving evidence that such treatment also can be offered to patients with positional anomalies.

We performed an arterial switch operation in a patient with double outlet right ventricle with non-committed ventricular septal defect, and abnormal insertion of the tension apparatus of the tricuspid valve which produced moderate tricuspid regurgitation. This required extensive enlargement of the ventricular septal defect between the attachments of the cords of the tricuspid valve so as to create the interventricular rerouting that made possible the arterial switch operation. Postoperatively, we produced a straight, unobstructed, left ventricular outflow tract, improved the extent of tricuspid regurgitation, and achieved low right atrial pressures. Enlargement of the interventricular communication can set the scene for biventricular repair in this particular subset of patients with both arterial trunks arising from the morphologically right ventricle.