We describe a previously asymptomatic 7-year-old girl with a sudden cardiac arrest during elective pacemaker revision. Later imaging identified epicardial pacemaker lead strangulation of the left anterior descending and left circumflex coronary arteries. Anaesthetic induction led to a reduction in myocardial perfusion, precipitating the arrest. Extreme care should be taken during anaesthesia if cardiac strangulation is suspected.

A 13-year-old girl with a single ventricle and bilateral systemic-to-pulmonary shunts developed hypoxia due to shunt stenosis, which was caused by a methicillin-sensitive Staphylococcus aureus abscess. Stent implantation associated with appropriate antibiotic administration was crucial to dilate and maintain shunt patency.

Noonan syndrome is the second most common genetic syndrome associated with congenital heart disease after Trisomy 21. The two most common cardiac lesions associated with Noonan syndrome are pulmonary stenosis and hypertrophic cardiomyopathy. Although the incidence of mitral valve disease in Noonan syndrome ranges between 2 and 6%, rapid progression of mitral valve dysplasia causing severe mitral valve regurgitation and left atrial dilatation is seldom seen. Most cases of mitral valve disease have been diagnosed either on routine echocardiographic surveillance or when presented with heart failure symptoms. We describe an 18-month-old boy with Noonan syndrome presenting in atrial flutter due to a massively enlarged left atrium caused by severe mitral valve regurgitation which developed and progressed in less than 17 months.

Hepatoblastoma is the most common primary liver tumour in children; however, there are no reported incidences in the single-ventricle congenital heart disease population. We present three cases in patients with single-ventricle heart disease with a mean age at diagnosis of 19.7 (±4) months. The diagnosis was made prior to Fontan completion in all three cases. These represent the only documented hepatoblastoma cases in single-ventricle patients.

Tricuspid valve atresia with severe pulmonary stenosis is one of the common cyanotic diseases in neonate. Child can succumb due to profound cyanosis and arterial hypoxaemia after closure of patent ductus arteriosus. Evolving procedure of right ventricular outflow tract stenting may be considered as a palliative procedure in such vulnerable group, destined for a later definitive management. The right ventricular outflow tract stenting is described essentially for tetralogy of Fallot physiology with a catheter course across tricuspid valve. We describe a case of successful right ventricular outflow tract stenting in a 5-day-old symptomatic neonate. We discuss the possible routes and the tips to facilitate right ventricular outflow tract stenting in such a case. This happens to be the first reported case description with successful stenting of neonate with tricuspid atresia with critical pulmonic stenosis.

LMNA mutations cause a variety of inherited diseases referred to as laminopathies which are associated with a wide spectrum of disease phenotypes, ranging from skeletal muscle disease, pre-mature ageing, metabolic disorders, and cardiac abnormalities. We present a case of a 14-year-old boy with dilated cardiomyopathy induced by the LMNA mutation (p. R429C) and described its electrocardiogram and imaging features.

Optimal pre-participative screening in young athletes is still controversial. We sought to evaluate the strategy of including point-of-care ultrasound to electrocardiogram. In total, 1188 young competitive athletes were screened in different sports institutions. This proved to be a useful strategy by improving diagnostic performance primarily with respect to detect structural abnormalities and also by minimising positive false cases of electrocardiogram alone.

Lymphatic vessel dysplasia is associated with Fontan-associated protein-losing enteropathy. Extra nodal non-Hodgkin lymphomas including mucosa-associated lymphoid tissue (MALT lymphoma) are associated with lymphatic vessel dysplasia. Here, we describe the case of a 7-year-old with Fontan-associated protein-losing enteropathy who developed MALT lymphoma with a clinical course indicative of interaction between these pathologies and improvement in protein-losing enteropathy after MALT lymphoma treatment. This case suggests a pathophysiologic overlap which has implications for the management of Fontan-associated protein-losing enteropathy.

Patients with a known genetic cause of aortic root dilation usually have a single underlying aetiology, either a single gene defect as in Marfan syndrome or chromosomal anomaly as in Turner syndrome. However, it is possible, although unlikely, for a patient to inherit multiple independent risk factors for aortic root dilation. We describe such a patient, who inherited Marfan syndrome and a very unusual form of mosaic Turner syndrome. Long-term follow-up of this patient may provide insight into the natural history of this unique genetic combination.