We report the case of a 13-day-old infant with enteroviral myocarditis surviving an out-of-hospital cardiac arrest. She underwent orthotopic cardiac transplantation three months later. A year after the transplantation, she is alive and well. Enteroviral infection is common in neonates with high mortality in cases of enteroviral myocarditis. Cardiac transplantation is a treatment option for infants who fail to recover and remain dependent on inotropic support. This is the first report of an infant with out-of-hospital cardiac arrest secondary to enteroviral myocarditis surviving up to cardiac transplantation.

Prolongation of the QT interval and development of torsades de pointes are known in patients with complete heart block and profound bradycardia. We report the case of a patient with complete heart block and torsades, with long QT seen during a period of junctional tachycardia at a rate faster than the minimum pacemaker rate.

We report the case of a patient with tetralogy of Fallot with absent pulmonary valve and familial Alagille syndrome who successfully underwent cardiac repair. The patient’s sister had liver and congenital heart disease. The father had undergone liver transplantation but showed no significant cardiac abnormalities. A yet-unknown mutation of the JAG1 gene was discovered in this family with variable expression of Alagille syndrome.

We present the case of a 4-year-old girl with idiopathic hypereosinophilia syndrome, endomyocardial fibrosis, and mural thrombus. This condition is rarely seen in children outside the tropics. Myocardial biopsy is historically the standard for diagnosis. Reports in adult literature, however, have shown the utility of cardiac MRI as a non-invasive tool for diagnosis, prognosis, and monitoring. To our knowledge, this is the first reported case with serial cardiac MRI in a child.

Although the spectrum of physiology seen in infants and children with double-outlet right ventricle is broad, this anatomy in combination with an aortopulmonary window is extremely rare. We present an interesting case of an infant prenatally diagnosed with this rare CHD. To our knowledge, this is the first report of complete repair in the neonatal period for such a combination of defects.

Hypertrophic cardiomyopathy associated with Friedreich’s ataxia is progressive, and there are few, if any, effective treatments available at present. This case report describes a Friedreich’s ataxia patient who had a septal myectomy for the management of hypertrophic cardiomyopathy with improved symptoms over a 7-year period. This suggests that septal myectomy may be a viable option to relieve symptoms and interrupt progression of heart disease in appropriately selected Friedreich’s ataxia patients.

We have re-investigated an unusual cardiac specimen with juxtaposition of the atrial appendages. The original description dates to 1962, when the autopsy was performed at the Children’s Memorial Hospital in Chicago, now Ann & Robert H. Lurie Children’s Hospital of Chicago. The heart was subsequently stored in the Farouk S. Idriss Cardiac Registry at the same institution. The specimen shows usual atrial arrangement, but with the morphologically left appendage juxtaposed in a rightward manner, passing behind the heart rather than through the transverse sinus so as to reach its location inferior to the morphologically right appendage. The heart also demonstrated an inter-atrial communication between the cavities of the juxtaposed left appendage and the morphologically right atrium. We provide a detailed description of the morphology, and provide images of this lesion, which to the best of our knowledge has not previously been described.

Junctional ectopic tachycardia, after surgery for CHD, is a serious arrhythmia that can cause increased morbidity and mortality. We report a case of junctional ectopic tachycardia, preceded by sepsis, in a 4-year-old girl, 31 months after open-heart surgery. She was successfully treated using low-dose landiolol hydrochloride.

Berry syndrome is a rare CHD. Approximately 29 cases have been described in the literature. Surgical correction has been successfully performed as well. We report the case of a newborn diagnosed with Berry syndrome who was subsequently diagnosed with trisomy 13. Cytogenetic analysis should be performed before surgical repair for optimal management.

Cantrell syndrome includes the defects of the heart, pericardium, diaphragm, abdominal wall, and sternum. The operative mortality is usually high. We report here a one-stage surgical correction in a case of Cantrell syndrome with left ventricular diverticulum.

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.

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.

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.

Ventricular tachycardia is uncommon in children without CHD. We present the case of a 15-year-old boy who presented with severe diabetic ketoacidosis and ventricular tachycardia and was not responsive to traditional anti-arrhythmic therapy. This case highlights the importance of identification of the underlying metabolic derangement causing the arrhythmia to provide appropriate management.

D is an 8-year-old boy brought to his paediatrician for evaluation. His mother is concerned as his teacher has been frequently complaining that he is very restless and often disturbs the rest of the class by getting up on some pretext or the other. He is unable to concentrate on his work and gets distracted very easily. He makes many careless mistakes and can hardly finish his tasks on time. He is frequently reprimanded for talking during class. He often answers out of turn or before the question has been completed; however, so far, he has been managing to get passing grades. At home, he is constantly on the go while he is awake. If he is forced to sit, like at mealtimes, he fidgets a lot. He also needs to be constantly nagged to do everything, even his daily activities such as brushing his teeth, or he forgets to do them or leaves them incomplete. He takes ages to finish his food. It is a major job to get him to do his homework. His mother says that at home he has been like that since the last 2 to 3 years, but now she is concerned because of the difficulties he is experiencing at school as well. After obtaining his medical history, examination, and getting response from parents and teachers – using Vanderbuilt Assessment Scales – the paediatrician diagnoses him to have attention deficit hyperactivity disorder. Besides behavioural interventions, he considers medications for his management. The paediatrician is debating the merits of performing electrocardiogram and/or referring the boy to a cardiologist before starting stimulant medications. If you were caring for this patient, how would you proceed?