We report the case of a patient with Donohue syndrome who died of heart failure due to obstructive hypertrophic cardiomyopathy. A literature survey revealed that hypertrophic cardiomyopathy was present in 30% of these patients and was often fatal. Therefore, every patient with Donohue syndrome should be screened for hypertrophic cardiomyopathy.

We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

In this study, we present the case of an extremely low birth weight infant with severe coarctation of the aorta after ductus ligation. We treated the patient with balloon angioplasty using the descending aorta as a new access route. This method reduced many complications typically observed during the intervention for extremely low birth weight infants, which may expand the available treatment options for extremely low birth weight infants.

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.

This report describes the clinical course and multi-modality imaging findings in an anti-SSA-antibody-exposed fetus with suspected myocardial inflammation. Postnatal cardiac MRI – using fast acquisition, free-breathing with feed-and-swaddle technique – was used to evaluate for myocardial fibrosis/inflammation. This is the first published report, to our knowledge, of ductal constriction temporally associated with oral dexamethasone therapy in an anti-SSA-antibody-exposed fetus and of the use of this unique postnatal MRI protocol in this setting.

Although nesiritide has been used in adults with left heart failure, the experience in the paediatric population is limited. We reviewed and analysed our experience with continuous nesiritide infusion as adjunct therapy in children with biventricular dysfunction due to diverse aetiologies and suffering from oliguria despite intravenous diuretics and inotropic therapies for heart-failure management.

Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure.

We report a case of aortic pseudoaneurysm associated with a fractured bare Cheatham-Platinum stent following stenting for aortic coarctation. These complications were recognised 6 years after the implantation procedure and were successfully managed by percutaneous stent graft implantation. Staged approach for stent dilatation might prevent development of aortic pseudoaneurysms. In addition, careful follow-up is warranted after stenting for aortic coarctation, particularly in patients with recognised aortic wall injury.

Surgical vegetectomy may be indicated in patients with unresolving sepsis, heart failure, recurrent embolism, or the presence of large vegetations >10 mm in size. Percutaneous vegetectomy using a snare may be a reasonable option instead of open-heart surgery in selected patients. We describe the case of a patient with operated tetralogy of Fallot and infective endocarditis who underwent vegetectomy via a percutaneous approach.

We report a case of a newborn, affected by tuberous sclerosis complex, with a prenatally diagnosed giant cardiac rhabdomyoma associated with a large renal angiomyolipoma presenting as a duct-depending lesion not treatable by surgery. After receiving everolimus, a mammalian target of rapamycin inhibitor, we observed a rapid, significant, and durable reduction of both lesions without remarkable side effects.

Congenital complete atrioventricular block is a known lethal condition. Although antenatal diagnosis and the technical advances of pacemaker treatment have reduced its mortality, treatment of premature babies with significant myocardial damage remains a challenge. In this paper, we report the case of a premature low-birth-weight infant with congenital complete atrioventricular block and extremely low ventricular rate, fetal hydrops, and myocarditis who was successfully treated with staged permanent pacemaker implantation.

Unilateral pulmonary artery atresia is a rare congenital defect. The affected lung in unilateral pulmonary artery atresia usually receives its blood supply from many collateral vessels including the coronary artery. We report a case of an elderly woman with suspected anginal symptoms who had a coronary artery fistula functioning as a collateral vessel due to unilateral pulmonary artery atresia.

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?

An alternative approach for transcatheter closure of atrial septal defect is necessary in patients with absent inferior caval vein connection. In this report, we describe the successful transcatheter atrial septal defect closure via the transjugular approach using a steerable guide catheter.