We present two procedures of transcatheter closure: that of an aorto-pulmonary window in a 12-month-old infant with a body weight of 7 kilograms, and that of ruptured sinus of Valsalva aneurysm into the right atrium in an adult patient. In the first case, we applied the muscular ventricular septal defect Cardio-O-Fix, while in the other we applied the patent ductus arteriosus Cardio-O-Fix occluder. The procedures were successful in both patients, and we achieved complete closure of the unwanted shunts.

Kawasaki disease is an acute vasculitis of childhood. Its clinical presentation is well known, and coronary artery aneurysms are classical complications. Shock and pleural or pericardiac effusion are rare presentations of the disease. In intensive care units, the disease may be mistaken for septic shock or toxic shock syndrome. Owing to the fact that immunoglobulin therapy improves the course of the disease, especially if given early, and thus the diagnosis should not be delayed.

Isolated persistent left superior caval vein is a rare anomaly of the systemic venous return. We describe an unsuccessful pacemaker implantation through the right subclavian vein, which later turned out to be due to a venous anomaly. This case and some other cases show that with the help of a multidetector computed tomography, an isolated persistent left superior caval vein is not a contraindication for subclavian approached pacemaker insertion.

We present the case studies of two adult patients with tetralogy of Fallot who were scheduled for surgery. After addressing the right ventricular outflow tract obstruction, the aorta was opened and the ventricular septal defect was approached in a straightforward manner as it was located just under the overriding aortic valve. The second patient presented with was a situs inversus, dextroapex Fallot. In this setting, the aortic approach simplified the repair expeditiously. After 2 years, both patients are in New York Heart Association class I, with no residual ventricular septal defect, no aortic regurgitation, and complete relief of right ventricular outflow tract obstruction.

Kawasaki disease constitutes an acute febrile vasculitis of unknown aetiology. It is considered the most common cause of acquired cardiac failure in children. Although standard treatment comprises intravenous immunoglobulin and aspirin, some children exhibit refractory disease, necessitating the use of alternative therapies such as corticosteroids and anti-tumour necrosis factor-alpha. For these cases, few controlled data are available. This report focuses on an extremely refractory classical Kawasaki disease with coronary artery aneurysms and ongoing inflammation. We discuss the therapeutic approaches and the potential pitfalls undertaken, which led to an unfavourable clinical outcome.

A 34-week premature neonate presented with drug-refractory permanent junctional incessant tachycardia and haemodynamic compromise. The patient underwent successful radiofrequency catheter ablation using a single-catheter approach. The child remains in sinus rhythm, without pharmacological treatment, 2 years after the procedure.

We present a patient with DiGeorge syndrome and an isolated right subclavian artery arising from the right pulmonary artery via a right-sided ductus arteriosus. The patient showed a subclavian and pulmonary steal with perfusion of the right arm and right lung via retrograde circulation in the right vertebral artery. The patient underwent successful surgical repair.

We report the case of a 1-month-old boy with an unusual association of supraventricular tachycardia and anomalous origin of the left coronary artery from the pulmonary artery. Although signs of infarction were visible on the first electrocardiogram, the presence of an arrhythmia did not immediately suggest a coronary anomaly. Echocardiography allowed the diagnosis, thus leading to appropriate care.

The arterial switch operation has become a safe operation in many centres. The complexity of the procedure has evolved over the last two decades. Several anatomical features can hardly be considered complex today, namely, normal coronary anatomy, circumflex coming off the right coronary artery, eccentric ostium, and early take-off of an infundibular artery. In addition, as peri-operative mortality becomes very low, the outcomes need to be evaluated on the peri-operative morbidity, late reoperations, and late deaths. The arterial switch operation remains complex in around 20% of the cases, where one or several complexity factors are associated. The complexity of the coronary arteries is a major factor. According to a classification essentially based on the course of the coronary arteries, complex coronaries include: double-looping coronaries, anterior-looping coronaries, intramural coronaries, and single coronary ostium. The most challenging coronary pattern remains the association of a single ostium with intramural course. Other features are equally complex: severe malalignment of the commissures, aortic arch obstruction, multiple ventricular septal defect, Taussig–Bing with subaortic obstruction, double-outlet right ventricle non-committed ventricular septal defect, transposition of the great arteries-intact ventricular septum >3 weeks, transposition of the great arteries-ventricular septal defect with high lung resistances and weight <2.5 kg. Owing to the fact that the risks of arterial switch operation vary according to the experience of the centres, we defined the arterial switch operation complexity based on a subjective approach as proposed by the Aristotle comprehensive score. The recent introduction of a morbidity score will allow to stratify more accurately the outcomes when the peri-operative mortality is very low or nil. The complexity of the coronary patterns tends to be well controlled today. It remains that rare coronary failures and aortic root dilation will occur in the long term, requiring a close follow-up of the most complex patients. Successfully achieving complex arterial switch operation implies a second learning curve.

Tall stature is a common feature of both Marfan syndrome and XYY syndrome. Differential diagnosis between these entities has important prognostic implications. We report the case of a 21-year-old young man with a previously known diagnosis of XYY syndrome, in whom the identification of a fibrilin-1 mutation was determinant to establish an appropriate diagnosis, medical follow-up, and genetic counselling.

Persistent fifth – double-lumen – aortic arch is a rarely seen entity, which is usually associated with other cardiac anomalies; it has been previously reported only in children. We report a new case of double-lumen aortic arch with a systemic-to-systemic connection diagnosed incidentally during cardiac multi-detector computer tomography evaluation of coronary artery bypass grafts in an adult.

Complex muscular ventricular septal defect poses difficult surgical management and is associated with high morbidity and mortality despite advancements in surgical therapy. Device closure of muscular ventricular septal defect has been encouraging and has been used in hybrid approach at a few centres. However, device closure has some limitations in patients with complex muscular ventricular septal defect. We report a case of perventricular device closure of a complex muscular ventricular septal defect in a beating heart with entrapped right ventricular disc and its surgical management.

Anomalies of the innominate vein are uncommon in congenital cardiac disease. We report a case of duplicate innominate veins forming a vascular ring encircling the ascending aorta. We postulate that this vascular ring represents the failure of both a dorsal and ventral precardinal anastomosis to regress.

Percutaneous upsizing of surgically placed Blalock–Taussig shunts is an uncommon practice. We report the case of an 8-month-old infant with single-ventricle physiology, who – due to comorbidities – was deemed unsuitable to proceed with Glenn operation. The 3.5-millimetre Blalock–Taussig shunt was stented successfully with a 5-millimetre pre-mounted stent, resulting in an increase in shunt diameter and oxygen saturation by nearly 30% and 10%, respectively.

A 7-year-old child was noted to have dextrojuxtaposition of the left atrial appendage at the time of surgical atrial septal defect repair. Given the favourable anatomic location and size of the atrial appendage, it was inverted and used to close the atrial defect. This is the first report of atrial septal defect repair using a juxtaposed atrial appendage. The cardiac anatomy and theoretical benefits of this repair are discussed.