Coronary sinus defect is a rare type of atrial septal defect. We report two patients who had a coronary sinus atrial septal defect without persistent left superior caval vein, where the orifice of the coronary sinus was closed using the Amplatzer Septal Occluder. The procedure was successful, without any complications including conduction disturbance.

Congenital aortocaval fistula is a very rare anomaly. Clinically, it resembles conditions that cause left-to-right shunt of blood. We report a case of such anomaly in combination with a secundum atrial septal defect in a 13-month-old girl who presented with failure to thrive and exertional respiratory symptoms. The aortocaval fistula was occluded percutaneously using an Amplatzer® Duct Occluder.

Left ventricular pseudoaneurysm is especially rare in childhood, and its main treatment option should be surgery. We describe the case of a 9.5-year-old boy who first underwent mitral vegetation excision and then an unsuccessful pseudoaneurysm operation. Owing to pseudoaneurysmal sac dimensions, inferior caval vein syndrome developed. We delivered the Amplatzer Vascular Plug 4 into the pseudoaneurysm and treated the inferior caval vein syndrome with a bare Cheatham–Platinum stent. The patient was asymptomatic at the last follow-up.

Arrhythmias in patients with congenital heart disease present a challenge to the care of these patients and can result in significant morbidity and mortality. Transposition of the great arteries and common arterial trunk are no exceptions. It is important to identify risk factors for arrhythmia development in the peri-operative period. The peri-operative arrhythmia burden may relate to the underlying congenital heart disease, haemodynamic perturbations, operative events, and potential residual lesions. In addition, these patients are at risk for developing arrhythmias later in life, and non-invasive and potentially invasive arrhythmia surveillance should be a routine part of the care of these patients. This article highlights important strategies to manage arrhythmia development and prevention in this patient population.

A 12-year-old girl presented with a first prolonged syncope. She was successfully resuscitated by external defibrillation after recording torsade de pointes tachycardia. Repeated electrocardiograms and a 12-channel Holter monitoring showed an intermittent prolongation of the QT interval. Genetic analysis identified a heterozygous point mutation in the KCNH2 gene, which is thought to be associated with a rather mild clinical phenotype of the long QT syndrome.

A 12-year-old girl with hypoplastic right heart, double inlet left ventricle, and transposition of the great arteries had a lateral tunnel Fontan operation 8 years previously. She developed symptoms related to a combination of stenosis in the inferior caval venous pathway, a fenestration, and leak in the lateral tunnel. These were successfully treated with a custom-made stent graft.

Although mortality rates for patients with d-transposition of the great arteries remain quite low, these patients have a unique circulation that requires careful management in the peri-operative period. Despite the improved mortality for patients with common arterial trunk, the course in the intensive care unit is remarkable for significant morbidity and utilisation of significant resources. Pre-operative patient management focuses on balancing competing circulations, pulmonary and systemic, which exist in parallel rather than in series, as in the normal circulation. Post-operative patient management in both lesions focuses on optimising systemic output, respiratory status, and mitigating the effects of cardiopulmonary bypass. In this article, we review pre- and post-operative intensive care management in neonates with d-transposition of the great arteries and common arterial trunk.

Myocardial ischaemia and infarction in pulmonary atresia and intact ventricular septum with right ventricular-dependent coronary circulation is a well-established complication. We report an interesting case of an acquired aneurysm in the ventricular septum in a patient who underwent staged palliation.

The authors describe successful balloon angioplasty of aortic coarctation in a preterm neonate weighing 670 grams. The intervention was performed in an open incubator to ensure stable temperature comfort and to minimise the risk of hypothermia during the procedure of obtaining surgical vessel access, performing balloon angioplasty, and closure of the wound.

Primary care cardiology, also known as ambulatory or outpatient cardiology, for the longitudinal management of patients with common arterial trunk or with transposition of the great arteries is both poorly described and has limited evidence to justify its basis. This article discusses some of the various complications that these patients can develop, reviews the medical literature, and describes a framework for care of these complex patients from infancy to transition to adult congenital cardiac specialist care.

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.

Acquired complete atrioventricular block that is caused by infectious myocarditis is usually transient and has a favourable outcome. We report the case of a 15-year-old girl who had complete infra-Hisian atrioventricular block due to adeno viral myocarditis and received a permanent pacemaker at the age of 10 months. The pacemaker lost its function at the age of 7 years. However, she experienced a late recurrence of complete atrioventricular block 10 years later. Complete atrioventricular block is rarely recovered if it persisted for 2 weeks. Even in the patients with late recovery, long-term follow-up and pacemaker therapy are still needed.

We report an infantile case of Loeys–Dietz syndrome prenatally diagnosed with congenital complex heart disease – double outlet right ventricle and interruption of the aortic arch. The patient also showed prominent dilatation of the main pulmonary artery. Emergency bilateral pulmonary artery banding was performed on the 9th day. However, on the 21st day, the patient died of massive bleeding due to rupture of the right pulmonary artery. Subsequently, a mutation of the TGFBR1 gene was detected. As cardiovascular lesions of Loeys–Dietz syndrome appear early and progress rapidly, the prognosis is generally poor. Patients require periodic examination and early intervention with medical therapy such as Losartan administration and surgical therapy. Early genetic screening is thought to be useful for the prediction of complications as well as vascular disease.

Despite various medications for Kawasaki disease, a small number of children have been undergoing interventions for severe coronary artery complications. Transcatheter intervention is a feasible alternative to coronary artery bypass grafting in a patient with chronic totally occluded lesion after Kawasaki disease, even by stent fracture.