We present the case, with tomographic three-dimensional reconstructions, of an adult patient affected by congenital absence of one pulmonary valve cusp with completely normal morphology of the other two cusps.

Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned. The boy subsequently underwent a successful surgical closure thereafter.

Congenital heart block is a rare and lethal condition in paediatric population associated with maternal connective tissue disorders and rarely with structural cardiac disease like atrioventricular canal defects with or without left isomerism and congenitally corrected transposition of great arteries. Pacing in neonate if indicated is generally accomplished by epicardial pacing systems. However, in cases of significant bradycardia and haemodynamic instability, temporary pacemaker implantation via transvenous approach remains as a suitable option. Despite the advances in percutaneous catheter interventions, use of transvenous pacing in newborn is extremely challenging due to inadvertent risk of vessel injury, thrombus formation and mortality, and most of the time technical inability to place the lead within the right ventricular cavity. We report a case of congenital complete atrioventricular block in a premature male with birth weight of 1.51 kg who was managed with temporary pacemaker implantation through umbilical vein.

Fontan fenestration allows right-to-left shunting increasing cardiac output and oxygen delivery. Increased shunting occurs as cardiac function and ventricular end-diastolic pressures improve, potentially decreasing oxygen saturation. Complete closure may result in impaired Fontan haemodynamics and low cardiac output; however, there are no dedicated devices to reduce fenestration size. We describe Fontan fenestration size reduction using the Atrial Flow Regulator.

Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

In cases where femoral access is untenable for secundum atrial septal defect closure, the internal jugular vein can be used instead. We report a case of atrial septal defect closure in a patient with significant vascular thrombosis, requiring the use of the left internal jugular vein for access. To the best of our knowledge, this is the first report that documents the use of the left internal jugular vein for atrial septal defect closure in a patient with situs solitus anatomy.

We present two cases of patients with iatrogenic femoral arteriovenous fistula who underwent successful embolisation using three-dimensional shape detachable coils. A 49-year-old male with Tetralogy of Fallot had arteriovenous fistula which developed from the common femoral artery to the femoral vein with an aneurysm and a 17-year-old female with single ventricle after total-cavo-pulmonary-connection had two arteriovenous fistulas which developed from the internal iliac artery to the femoral vein. A total of six and seven pieces of detachable coils were necessary for complete occlusion, respectively. No complications were recorded. The advantage of the detachable coil is a wide variation and repositioning until the coil achieves good stabilisation and an ideal configuration.

Bridging bronchi are the rarest of the major airway anomalies reported in the literature. In this brief report, we present a case of a symptomatic adult male patient presenting with a type 2 bridging bronchus associated with left pulmonary artery sling.

We describe the case of a 2-month-old born with hypoplastic left heart syndrome who presented with fever and vomiting and was found to be infected with the novel corona virus (COVID-19). He underwent treatment with supplemental oxygen, heparin, and dexamethasone. After a 6-day hospitalisation, he recovered remarkably well without major adverse effects.

Platypnea–orthodeoxia syndrome is a rare clinical entity characterised by positional dyspnoea and arterial desaturation while in the upright position, the symptoms generally occurring in adults. We describe a 12-year-old girl diagnosed with platypnea–orthodeoxia syndrome associated with patent foramen ovale. The symptoms resolved following percutaneous patent foramen ovale closure.

Coronary abnormalities are frequent in pulmonary atresia and intact ventricular septum, mainly in patients with a very diminutive right ventricle. They severely impact on early and late prognosis. We describe an 8-year-old girl who presented with myocardial ischaemia, late after uneventful Fontan completion. The importance of precise delineation of the coronary anatomy upon initial assessment and during follow-up is emphasised.

The essence of so-called heterotaxy is the potential disharmony between the arrangement of the bronchuses, abdominal organs, and the atrial appendages. Accurate description of the heart, however, can only be provided by specific description of these features, all of which are readily discernible in the clinical setting. We argue that, when accurate description of the atrial and visceral arrangement is provided, along with appropriate description of the intracardiac findings, no further accuracy is gained by suggesting that an individual heart is “heterotaxic”.