Despite its low prevalence, the potential diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT) should be at the forefront of a paediatric cardiologists mind in children with syncope during exercise or emotions. Over the years, the number of children with a genetic diagnosis of CPVT due to a (likely) pathogenic RYR2 variant early in life and prior to the onset of symptoms has increased due to cascade screening programmes. Limited guidance for this group of patients is currently available. Therefore, we aimed to summarise currently available literature for asymptomatic patients with a (likely) pathogenic RYR2 variant, particularly the history of CPVT and its genetic architecture, the currently available diagnostic tests and their limitations, and the development of a CPVT phenotype – both electrocardiographically and symptomatic – of affected family members. Their risk of arrhythmic events is presumably low and a phenotype seems to develop in the first two decades of life. Future research should focus on this group in particular, to better understand the development of a phenotype over time, and therefore, to be able to better guide clinical management – including the frequency of diagnostic tests, the timing of the initiation of drug therapy, and lifestyle recommendations.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery. Elimination of the arrhythmogenic substrate was confirmed and the device was removed successfully.

Intracardiac teratomas are rare primary tumours. We report the case of an infant prenatally diagnosed with an isolated multi-cystic mass developed in the right ventricle causing neonatal refractory ventricular arrhythmia. Despite rescue extracorporeal support and partial surgical resection, he died as almost all the previous reported perinatal intracardiac teratomas whatever the prenatal tolerance and the size of the tumour. The common poor outcome of fetal intracardiac teratomas should be known when counselling parents during pregnancy.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery. Elimination of the arrhythmogenic substrate was confirmed and the device was removed successfully.

A 16-year-old patient underwent successful ablation of ventricular arrhythmia originating from the aortic sinus of Valsalva following surgical unroofing of an anomalous right coronary artery. This case illustrates the complexity of decision making in the management of patients with anomalous coronary arteries and the importance of keeping an open mind when determining ventricular arrhythmia aetiology and origin.