We present a case of a 31-year-old male with a large atrial septal defect, who was found to have interrupted inferior caval vein with azygous continuation to the superior caval vein, which precluded transcutaneous closure by device. The defect was successfully closed with a 33 mm Occlutech Figula septal occluder using a sub-mammary small thoracotomy incision and per-atrial approach without using cardiopulmonary bypass. The patient was discharged home after 48 hours of procedure.

We utilised a cast preoperatively prepared on the basis of the patient’s computed tomography data to clarify unusual orientation of the great arteries (transposition of great arteries, coarctation of the aorta, and abnormally branching central pulmonary arteries) in a 2.2-kg neonate. The three-dimensional model was very useful for designing the surgical procedure – being easy to handle for real and precise simulation of the reparative manoeuvres.

We present a case of a 7-month-old boy with tetralogy of Fallot associated with unilateral pulmonary aplasia and herniation of the liver and small bowel loops in the right hemithorax.

Ventricular septal defect is the most common type of CHD, and transcatheter ventricular septal defect closure has been shown to be an alternative to surgical closure with acceptable mortality and morbidity as well as encouraging results. Short-term and mid-term follow-ups have indicated the safety and efficacy of transcatheter closure, but long-term follow-up results were rare. In this report, we first found that aortic regurgitation occurred in patients 9–12 years following transcatheter closure and regurgitation were gradually increased. The findings indicate that the long-term outcome of transcatheter closure of ventricular septal defect may not be as satisfied as expected.

In the accompanying article appearing in this issue of the Journal, Prabhu and his colleagues, from Bengalaru in India, describe their experience with patients having a right aortic arch. They discuss the fact that the anomalous arrangements they encountered can all be interpreted on the basis of the hypothetical double arch proposed by Edwards. They point to the fact that interpretation of the developmental changes underscoring the production of the double arch is currently confused by reference to the so-called Rathke diagram, in which six sets of arteries are shown extending through the mesenchyme of the pharyngeal arches. As the authors point out, Graham and his associates have now shown that the alleged fifth set of pharyngeal arches do not exist. Based on our own observations, we endorse this statement. It means that new explanations must now be provided for the lesions previously described on the basis of persistence of the alleged artery of the fifth pharyngeal arch. We have previously claimed to have observed such an artery in a human fetus. We now believe, on the basis of our latest findings, that our earlier observation is better explained on the basis of presence of a collateral channel. We suggest that the so-called “fifth arch arteries” are themselves then best explained either on the basis of existence of such collateral channels, or remodelling of the aortic sac, which is the manifold, during development, that gives rise to the pharyngeal arch arteries.

We introduce a new surgical technique where an extracardiac tunnel is created using pedicled autologous pericardium in an 8-month-old boy who was diagnosed with ventricular septal defect and anomalous connection of the right superior pulmonary vein to the superior vena cava.

Differential diagnosis of an intracardiac mass is difficult when detected only by echocardiography before a biopsy is completed. However, treatment cannot be postponed until the biopsy results are obtained. We report the case of a 12-year-old girl who presented with an intracardiac mass in the mitral valve mimicking infective endocarditis and severe mitral regurgitation. The mass was finally diagnosed as valvulitis associated with systemic juvenile idiopathic arthritis, which was complicated with macrophage activation syndrome. After careful exclusion of acute infectious disease, we started steroid pulse therapy and administered tocilizumab to treat the cytokine storm before performing the surgery. Finally, we performed mass excision and mitral valve replacement after immunosuppressant therapy.

Persistent left superior vena cava is a thoracic venous return anomaly. Tetralogy of Fallot is one of the most common congenital anomalies seen with persistent left superior vena cava. We are presenting a successful cardiac resynchronisation therapy device implantation in a patient with repaired tetralogy of Fallot and persistent left superior vena cava combination which has not been previously reported in the literature.

Known as a benign arrhythmia and normally requiring no specific treatment, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. Here, we present a child with left coronary cusp-originating accelerated idioventricular rhythm, degenerating into torsades de pointes and resulting in cardiac arrest, which was ablated with a cryocatheter. An 11-year-old boy, followed due to asymptomatic accelerated idioventricular rhythm before, was referred to our department because he had experienced an aborted cardiac arrest during sleep. He had been resuscitated for 5 minutes. Twenty-four-hour Holter-ECG revealed incessant accelerated idioventricular rhythm, consisting up to 90% of the whole record and two torsades de pointes attacks, triggered by accelerated idioventricular rhythm-induced “R on T” phenomenon, and resulting in syncope and cardiac arrest. Transthoracic echocardiography revealed no structural cardiac defect but mild left ventricular systolic dysfunction with an ejection fraction of 45% and shortening fraction 23%. An electrophysiologic study was conducted, and accelerated idioventricular rhythm focus was mapped to left aortic coronary cusp. A cryocatheter with an 8-mm tip was preferred for successful ablation of the accelerated idioventricular rhythm focus, due to close neighbourhood to coronary ostium. The patient was discharged in 3 days without any premature ventricular contractions or accelerated idioventricular rhythm and with normalised cardiac functions. After 9 months on follow-up, he was still asymptomatic, without any premature ventricular contractions or accelerated idioventricular rhythm and with normal cardiac functions. Although the clinical course of accelerated idioventricular rhythm is known as benign, accelerated idioventricular rhythm can rarely degenerate to a life-threatening arrhythmia. In such cases, electrophysiologic study and catheter ablation are a good option in such cases with accelerated idioventricular rhythm for an ultimate cure.

Percutaneous closure is the gold standard treatment for atrial septal defects, but the procedure can be complex in case of femoral thrombosis. Although unusual for congenital interventionists, transhepatic atrial septal defect closure is an attractive alternative to the internal jugular vein, especially when approaching the interatrial septum. Herein, we report the case of an adult patient with significant co-morbidities who had successful transhepatic atrial septal defect closure after a failed transjugular attempt. We describe the use of an absorbable haemostatic gelatin sponge to efficiently and safely achieve haemostasis after the use of a large vascular sheath with combined anticoagulation and antiplatelet therapy.

Coronary artery embolism is extremely rare in children with Fontan palliation. We report a 10-year-old boy with hypoplastic left heart syndrome post-Fontan procedure who presented with acute onset chest pain and ST-segment changes on electrocardiogram. Cardiac catheterisation revealed coronary artery emboli requiring stent placement. Our case highlights the importance of evaluating chest pain in a systematic manner in Fontan patients as acute coronary artery embolism can be a part of the spectrum of well-known thromboembolic complications.

Kawasaki disease is an acute febrile medium-vessel vasculitis affecting children. Though coronary artery aneurysm is a common complication, stenosis and occlusion are rare. Here, we report a case of Kawasaki disease with concomitant aneurysm and complete occlusion of different coronary arteries in the same patient.

Prenatal closure of foramen ovale without CHD is a rarely reported entity. Therefore, clinical and echocardiographic findings are poorly defined in these patients. We report a patient with prenatal closure of foramen ovale that presented with severe pulmonary hypertension of the newborn and left ventricular failure. Judicious management strategies were utilised to successfully treat both life-threatening conditions.

A 25-year-old patient with signs of cirrhosis on ultrasound and CT presented with portal vein thrombosis on routine follow-up examinations; retrograde hepatic wedge angiography demonstrated only the right-sided portal vein branch. Development of a portosystemic collateral vessel to the left-sided renal vein prevented signs of hypersplenism. This unique complication of portal vein thrombosis should be considered during long-term surveillance.

An extremely low birthweight infant (940 grams) with a rare variant of obstructed infracardiac total anomalous pulmonary venous return underwent stenting of the venous duct as bridge to later surgical intervention. While technically challenging, this procedure represents a bridge to surgery for infants who might otherwise not be surgical candidates.

We present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.

Ductal arterial spasm is a very potentially dangerous incidence during percutaneous device closure of patent ductus arteriosus (PDA), which, otherwise, is a very safe catheter intervention. It is essential to notice its occurrence before device sizing and deploying. Without awareness, it can mislead device selection and can result in serious complication. In this report, we shared our nightmare of ductal spasm during transcatheter closure of PDA in two children which had led to death in one patient.

The association of complete atrioventricular canal with transposition of the great arteries is rare, with a prevalence of less than 3–5%. We present an 18-month-old patient with a complete atrioventricular canal, side-by-side transposition of the great arteries, and anomalous coronary anatomy, managed initially with pulmonary banding and then by arterial switch with complete atrioventricular canal repair at early infancy.