Univentricular heart is a rare congenital heart malformation. Nowadays prognosis is considered to be strictly linked to surgical intervention, and survival into late adulthood is unusual.

In some patients native haemodynamic circulation balances pulmonary and systemic blood flow, allowing long-term survival without the need for surgery.

We report the case of a 74-year-old man with a univentricular heart in natural history, and we discuss the factors that might contribute to his extraordinary long-term survival.

Most commonly, hearts have either a single arterial trunk or two arterial trunks; however, rare reports exist of a “tritruncal” heart, a heart with three outflow tracts. Here, we present one of the first reports of successful surgical palliation of a triple-outlet right ventricle.

Truncus arteriosus communis is a rare CHD, accounting for only 1% of all congenital cardiac abnormalities. It has been associated with other malformations of the heart, mainly truncal valve (bicuspid/quadricuspid) and aortic arch abnormalities such as right, interrupted, and hypoplastic aortic arch. Cor tratriatrum sinistrum is another rare CHD, and it has been associated with other cardiac defects such as anomalous pulmonary venous drainage, ventricular septal defect, coarctation of the aorta, and tetralogy of Fallot. The combination of truncus arteriosus communis and cor tratriatrum sinistrum has not been reported so far. This case study describes the diagnosis of a unique case, including these two very rare cardiac defects and the successful surgical treatment thereafter.

Fatal arrhythmias in asymptomatic Kawasaki disease patients with normal left ventricular function have rarely been reported. In this study, we report the cases of two adult patients with largely unpredictable sudden cardiac arrest, despite almost-normal left ventricular function even after the diagnosis of presumed Kawasaki disease, as well as consider the mechanisms involved with reference to the literature.

Cardiac rhythm monitoring has been facilitated by the use of implantable loop recorders. New models of these devices are 87% smaller than before allowing for easier implantation and use in the paediatric population. Recommendations are for closure with adhesive. We report a device extrusion in a 6-year-old patient. Based on this, our practice has changed to include subcutaneous sutures this complication.

We present two cases of an unusual coronary artery anomaly, namely, a single coronary artery with retro-aortic course of the left circumflex artery and pre-pulmonic course of the left anterior descending artery, and one of which showed a dual left anterior descending artery as well – a combination that has not yet been reported in the literature.

Management of newborns with cyanotic CHD and bilateral adrenal haemorrhages has not previously been described in the literature. These abnormalities present unique challenges due to the potential for haemodynamic instability, need for open heart surgery and associated systemic anticoagulation in the newborn period, and the risk of catastrophic bleeding.

Despite several reports describing echocardiography for the guidance of ductal closure, two-dimensional angiography remains the mainstay imaging tool; three-dimensional rotational angiography has the potential to overcome some of the drawbacks of standard angiography, and reconstructed image overlay provides reliable guidance for device placement. We describe arterial duct closure solely from venous approach guided by live three-dimensional image overlay.

Percutaneous closure of muscular ventricular septal defects has been well described and has not attracted the same controversy or scrutiny as perimembranous defect closure. Therefore, the development of specifically designed devices has been limited. We report the first use of the Occlutech® muscular ventricular septal defect device. Does its design add any significant benefit?

Takotsubo cardiomyopathy, “broken heart syndrome”, is a well-known diagnosis in adults; however, this entity remains rare and is not well represented in the paediatric population. This report illustrates a case of takotsubo cardiomyopathy in a premature neonate with a brief discussion of the condition.

A 19-year-old woman with atrial septal defect treated percutaneously with an Amplatzer Septal Occluder 24 months earlier, who presented with a history of bacterial meningitis, was admitted with a diagnosis of endocarditis. After 6 weeks of treatment with antibiotics, the incompletely endothelialised occluder was surgically removed. The present report illustrates the need for long-term follow-up of patients who have received nitinol wire mesh occluders.

In this observational case report, we share our experience of achieving >40% LDL cholesterol reduction in four Chinese homozygous familial hypercholesterolaemia children below 8 years of age with a triple combination of atorvastatin, probucol, and ezetimibe for >6 years. Within a follow-up duration of 6–13 years, this triple therapy achieved significant reduction of LDL cholesterol as well as an impressive regression of xanthomas in all paediatric cases. All the children remained free from treatment-related adverse responses and cardiovascular events throughout follow-up.

Neonatal congestive heart failure in cerebral arteriovenous malformations carries a high risk of mortality. Severe heart failure leading to end-organ dysfunction may be a contraindication to early intervention. Although echocardiographic markers of haemodynamic instability exist, an objective echocardiographic predictor of severe heart failure is not available. We present a ratio of antegrade to retrograde flow in the aortic arch that objectively identifies those at the highest risk of mortality. This measurement can be easily obtained and has prognostic value at presentation, before clinical deterioration. Outcomes remain poor even when early intervention is offered, which may reflect irreversible neurological abnormalities.

We report two cases with levoatriocardinal vein and partial anomalous pulmonary venous drainage in left-sided obstructive lesions. This association may be difficult to recognise by echocardiography. Cardiac CT and MRI were crucial to define the diagnosis and to tailor the best therapeutic option.

The beneficial effect of exercise restriction in preventing sudden cardiac death in children with aortic stenosis remains unclear. We report the case of a 15-year-old boy with congenital aortic stenosis who was resuscitated after sudden cardiac arrest during exercise. The case led to the new concept that exercise restriction may prevent not only unpredictable ventricular ischaemic events and associated arrhythmias but also progressive ventricular hypertrophy.

We present the case of a 15-year-old boy who underwent arterial switch operation due to transposition of the great arteries with severe scoliosis, obstruction of the right coronary ostium, and severe stenosis of the pulmonary trunk. Balloon angioplasty caused a large aortopulmonary shunt provoking myocardial ischaemia and pulmonary hypertension. The traumatic “aortopulmonary window” was percutaneously occluded using an Amplatzer Septal Occluder device as a bridge to surgical repair.

Fibromuscular dysplasia is a common vascular disease, which mainly affects the renal arteries. In this study, we report the case of a 1-day-old infant who presented with severe left ventricular dysfunction immediately after birth, and subsequently died 3 days postnatally. At autopsy, an extensive myocardial infarction of the left ventricle and the septum was found, caused by an isolated fibromuscular dysplasia of left coronary artery.

Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.