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.

We present the case of a 3-month-old boy with pulmonary arterial hypertension after corrective repair of total anomalous pulmonary venous connection. The patient developed severe pulmonary arterial hypertension with a high mean pulmonary arterial pressure of 45 mmHg. We performed continuous monitoring of pulmonary arterial pressure using a tip deflecting microcatheter in the intensive care unit. We successfully managed this patient based on real-time pulmonary arterial pressure measurements. Continuous real-time monitoring of pulmonary arterial pressure using this microcatheter enables individualized targeted therapy for infants with pulmonary arterial hypertension.

Percutaneous treatment of aortic coarctation is based on angioplasty and/or stenting of the isthmus. We report a case of a 28-year-old girl suffering from aortic coarctation syndrome (coarctation + ventricular septal defect + bicuspid aorta). She underwent coarctectomy with subclavian flap and pulmonary bandage followed by ventricular septal defect closure and bandage removal in her first year of life. When she was 27 years old, a follow-up echocardiography detected an isthmic pressure gradient and a demodulated Doppler in abdominal aorta. A cardiac catheterisation confirmed the diagnosis of aortic re-coarctation. An AndraStent® XL 48 mm was implanted with a resolution of the isthmic gradient. One year later, because of the reappearance of demodulated Doppler in abdominal aorta, a chest X-ray was performed, which showed a stent third-grade fracture. The fracture was corrected by positioning a covered stent cheatham platinum 45 mm through the fragments. The rarest complication after stenting procedures is the fracture of the device with an incidence between 0.01% and 0.08%. Pressure overload beyond the elastic threshold of the material and the pulsatile tension exerted by the blood flow on the walls of the stent are the main mechanisms at the base of the fracture, together with the compliance of the tissue. A vessel that underwent multiple surgical rearrangements could interfere with and complicate the physiopathology at the basis of the fracture. In conclusion, stenting is a safe technique to treat aortic coarctation; stent fracture is a rare event, and different anatomical and haemodynamic factors are related to this complication.

Catecholaminergic polymorphic ventricular tachycardia is a rare (prevalence: 1/10,000) channelopathy characterised by exercise-induced or emotion-triggered ventricular arrhythmias. There is an overall paucity of genotype-phenotype correlation studies in patients with catecholaminergic polymorphic ventricular tachycardia, and in vitro and in vivo effects of individual mutations have not been well characterised. We report an 8-year-old child who carried a mutation in the coding exon 8 of RYR2 (p.R169L) and presented with emotion-triggered sudden cardiac death. He was also found to have left ventricular hypertrophy, a combination which has not been reported before. We discuss the association between genetic variation in RYR2, particularly mutations causing replacement of arginine at position 169 of RYR2 and structural cardiac abnormalities.

We report an unusual case of a 12-month-old boy diagnosed with anomalous origin of a single coronary artery from the pulmonary artery associated with patent ductus arteriosus. The patient survival was attributed to left-to-right shunt (patent ductus arteriosus) allowing for appropriate myocardial perfusion. Successful surgical correction involved patent ductus arteriosus closure, mitral annuloplasty and reimplantation of the coronary artery into the aortic root.

Isolated aortic regurgitation and myocardial infarction are a rare congenital defect among neonatal patients. We present a case of a neonate with an unusual aortic valve morphology causing both regurgitation and obstruction of the left coronary artery ostium. Despite both non-invasive and invasive imaging modalities, accurate diagnosis of the valve morphology was only determined by direct visualisation at the time of surgical repair. To the knowledge of authors, this particular aortic valve morphology in neonatal population has not been previously reported in the literature.

A 16-year-old Caucasian male was diagnosed with recurrent infective endocarditis associated with his second Melody valve placed inside a previously treated infected Melody valve. The replacement of Melody valve after the first infective endocarditis episode might increase the risk of the second episode of infective endocarditis.

We report the case of a 59-year-old patient with a complex atrial septal defect in whom a 40-mm Amplatzer™ septal occluder was surgically extracted 50 days following implantation. Deployment manoeuvres were challenging leading to an immediate pericardial effusion that was closely monitored and uneventfully drained after 11 days. A dry pericardium was documented until 4 weeks of outpatient routine follow-up. However, the device was surgically explanted 2 weeks later, when an urgent chest computed tomography performed for worrisome symptoms showed pericardial effusion recurrence with peripheral contrast enhancement. Surprisingly, the surgical view showed a well-positioned device and an intact pericardium. We discuss the atypical sequence of clinical findings misleading our clinical judgement and precipitating surgery.

A hyperinflammatory response to COVID-19 is being described in children. While this presents, and responds to management, similar to that of Kawasaki Disease it is being coined a new entity. But is it really? We explore how this phenomenon may be Kawasaki Disease with a new trigger.