A 4-month-old girl with Down syndrome showed unexpected deterioration of pulmonary hypertension. Despite aggressive pulmonary vasodilation therapy, the patient died at 5 months of age. Lung autopsy showed that the pulmonary veins were obliterated by intimal fibrous thickening, and the media of the veins was arterialised with an increase in elastic fibres. Pulmonary veno-occlusive disease should be considered in the management of individuals with Down syndrome.

Supraventricular tachycardia is the most common significant arrhythmia in children. If prolonged, it may cause heart failure and progress to cardiogenic shock warranting prompt treatment. The recommended interventions following vagal manoeuvres are intravenous adenosine and in the unstable patient electrical cardioversion. We present an infant with an unstable supraventricular tachycardia that was resistant to electrical cardioversion and recommended doses of adenosine. He reverted to sinus rhythm with a higher dose of adenosine, suggesting that such doses may be required in refractory supraventricular tachycardia.

The Melody® transcatheter pulmonary valve system was developed for placement within right ventricle-to-pulmonary artery conduits in patients with CHD for treatment of stenosis or regurgitation, providing an alternative to open-heart surgery. Abnormal systemic venous connections altering the catheter course to the right ventricle-to-pulmonary artery conduit may present a challenge to Melody® valve implantation. We present two such cases, in which the Melody® valve was successfully implanted in teenage patients with congenitally corrected transposition of the great arteries after Senning atrial switch operation. Despite the abnormal catheter course, the right ventricle-to-pulmonary artery was approachable via the right femoral vein allowing for deployment of the Melody® valve in the appropriate position. This suggests that systemic vein-to-left atrium baffles are not prohibitive of Melody® valve implantation. This is an important implication considering the substantial population of ageing patients with CHD who have undergone atrial switch. Melody® valve implantation can be considered as a viable option for treatment of these patients if they develop right ventricle-to-pulmonary artery conduit failure.

We present a case of anomalous direct connection of the right superior caval vein to the left atrium. The diagnosis was made in a 2-year-old child with unexplained cyanosis and apparently normal heart. Corrective surgery was performed, and the child recovered completely.

Patients with patent ductus arteriosus and significant left-to-right shunt develop Eisenmenger syndrome at an early age and are not operable after development of irreversible pulmonary artery hypertension. Patients with mitral stenosis, however, are treatable even with suprasystemic pulmonary artery pressures. A combination of these two lesions is rare. We document a patient with differential cyanosis who improved after corrective surgery of both the lesions. The importance of post-capillary pulmonary artery hypertension in shunt lesions needs to be better appreciated.

Infective endocarditis is a serious complication occurring after percutaneous pulmonary valve replacement with a Melody valve; it is of uncertain incidence, and presents a challenging diagnosis. Vegetations may be difficult to visualise with transthoracic and transoesophageal echocardiography. We report the role of intracardiac echocardiography in this condition by confirming the prosthesis involvement.

Treatment of Scimitar syndrome is usually surgical; however, if there is “dual drainage” – that is, one to the inferior caval vein and the other to the left atrium – it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation.

Accelerated junctional rhythm has been reported in children in the setting of acute rheumatic fever; however, we describe a hitherto unreported case of isolated junctional tachycardia in a child with streptococcal pharyngitis, not meeting revised Jones criteria for rheumatic fever. A previously healthy, 9-year-old girl presented to the emergency department with complaints of sore throat, low-grade fever, and intermittent chest pain. She was found to have a positive rapid streptococcal antigen test. The initial electrocardiogram showed junctional tachycardia with atrioventricular dissociation in addition to prolonged and aberrant atrioventricular conduction. An echocardiogram revealed normal cardiac anatomy with normal biventricular function. The patient responded to treatment with amoxicillin for streptococcal pharyngitis. The junctional tachycardia and other electrocardiogram abnormalities resolved during follow-up.

In this study, we describe an infant case of pulmonary atresia with intact ventricular septum associated with ventriculo-coronary arterial communication for which a modified Blalock–Taussig shunt operation was performed. He experienced repeated myocardial ischaemic attacks. Further examination revealed pulmonary sequestration in the right lower lobe. He therefore underwent a bidirectional Glenn operation and coil occlusion of the feeding arteries. His myocardial ischaemic attacks subsequently improved.

We report a case of Uhl’s anomaly imaged at 19 weeks of gestation by fetal echocardiography with pathological confirmation by anatomical gross heart specimen and tissue histology. Uhl’s anomaly of the right ventricle is a rare cardiac disorder with isolated right ventricular enlargement with almost complete absence of the right ventricular myocardium.

Acromelic dysplasias are a group of skeletal dysplasias characterised by short-limbed short stature with other distinctive phenotypic features including small hands and feet and stiff joints. Geleophysic dysplasia is an acromelic dysplasia that is associated with characteristic facial features, progressive cardiac valvular thickening, and tracheal stenosis. Owing to overlapping clinical features with other types of short-limbed skeletal dysplasias, it is important to make a precise diagnosis as they have different cardiac morbidity and mortality. We present the cases of three patients with geleophysic dysplasia and progressive mitral valve disease to emphasise the natural history of this disorder and provide guidance regarding cardiac health supervision in these individuals.

A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierre’s syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes–Stovin syndrome was diagnosed. Hughes–Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case. The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.

Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.

We describe the case of a 17-year-old male soccer player with T-wave inversion in precordial leads in resting electrocardiography, which also disclosed sinus bradycardia, early repolarization, and increased QRS voltage. These findings strongly suggested cardiomyopathy. The patient’s T-wave inversion disappeared during only 2 weeks of detraining, and it re-appeared 2 weeks after resumption of intensive training. This sudden change in electrocardiographic parameters over a short period helped in identifying the adolescent as having athlete’s heart.

A 10-month-old girl with type I Loeys–Dietz syndrome developed a conspicuous aortic root aneurysm that was well demonstrated on chest X-ray/CT reconstruction. She underwent successful valve-spare repair of the ascending aorta.

Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock–Taussig shunt at 11 days of age.

We describe the implantation of an absorbable, custom-made stent of polydioxanone to treat tracheomalacia in a 5-month-old patient with extrinsic compression by a double aortic arch. The use of an absorbable, oversized stent treated the tracheal collapse caused by vascular compression, avoided removal procedures, and allowed the infant’s growth. The use of an oversized stent prevented stent migration and gave minimal problems of granulation.

Laboratory investigations have shown the role of inflammation in the pathogenesis of pulmonary hypertension and improvement after anti-inflammatory drugs. Despite these observations, reports on the use of steroids to treat pulmonary hypertension in humans are absent from the literature. In this article, we report the use of glucocorticoids in the treatment of two children with pulmonary hypertension, demonstrating its potential utility.