Most cases of severe or critical pulmonary stenosis are detected early and interventional management is routine within the first days of life. We present a case of a thirteen-year-old boy diagnosed with pulmonary stenosis and atrial septal defect with low ventricle ejection fraction. The patient underwent staged pulmonary balloon valvuloplasty and interventional atrial septal defect closure with good results.

Coronary artery compression by epicardial leads is a rare complication in children and can be difficult to identify with potentially lethal outcomes. Herein, we report the case of a previously asymptomatic paced-dependant 5-year-old girl who presented to our institution with resuscitated cardiac arrest. We describe the atypical sequence of clinical findings misleading initial diagnosis. Hardware failure and the commonly occurring lead fracture were incriminated in the mechanism of cardiac arrest, precipitating implantation of a new pacing system while concealing dynamic compression of the left anterior descending coronary artery.

Limited literatures report the management of congenital left atrial appendage aneurysm (LAAA) which is extremely rare. Chest X-ray firstly showed an enlarged left cardiac silhouette for a 3-year-old patient with pneumonia. Echocardiography and magnetic resonance imaging confirmed a large cyst attached to the left atrium. Aneurysmectomy was performed through lateral thoracotomy using step-by-step method and under the guidance of transoesophageal echocardiography. We aim to show the safety and efficacy of this approach applied to children associated with congenital LAAA.

Loeys–Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

We report a case of a 25-year-old man diagnosed with an unusual case of cor triatriatum sinister with a mobile left atrial thrombus. He was hospitalised with aggravating dyspnoea. Transthoracic echocardiography revealed a membrane-like structure traversing the left atrial and a small orifice of about 7.1 mm. The mean pressure gradient was 12.94 mmHg across the orifice of the membrane-like structure and there was a mobile mass in the post-erosuperior chamber. The anomaly was rectified by a surgical resection. Timely diagnosis and surgical repair may prevent stroke in patients with unusual cor triatriatum sinister.

Heuristics and cognitive biases constantly influence clinical decision-making and often facilitate judgements under uncertainty. They can frequently, however, lead to diagnostic errors and adverse outcomes, particularly when considering rare disease processes that have common, masquerading presentations. Herein, we present two such cases of newborn infants with hypertensive renal disorders that were initially thought to have cardiomyopathy.

Device occlusion of perimembranous ventricular septal defect is gaining popularity with the emergence of newer, softer occluders and improved technical know-how. We report a 26-year-old lady with a moderate size perimembranous ventricular septal defect who had a new onset of bundle branch block shortly after device closure. The patient subsequently developed a complete atrio-ventricular heart block.

A 11-year-old with history of mitral valve replacement presented with low-grade fever, breathlessness and multiple episodes of haemoptysis for 2 days. Detailed echocardiographic evaluation revealed possible prosthetic valve thrombosis, which was confirmed by fluoroscopy. She was thrombolysed with low dose infusion of tenecteplase. Post thrombolysis her symptoms improved, valve mobility was restored, and haemoptysis subsided. Left sided prosthetic valve thrombosis presenting predominantly with haemoptysis is very rare.

Pulmonary hypertension is a rare and complex disease with poor prognosis. Paediatric cases are infrequent and usually associated with congenital heart disease. Management is problematical due to the limited therapy available and poor evidence of efficacy. Recently a new medication, selexipag (UptraviR), a prostacyclin receptor agonist, has been approved for the treatment of pulmonary artery hypertension in adults. We report our experience using selexipag in four paediatric patients with pulmonary hypertension associated with congenital heart disease.

A four- and a half-month-old girl with severe dilated cardiomyopathy due to neonatal enterovirus myocarditis, treated with diuretics and milrinone for the past 4 months, was infected with SARS-CoV-2. The disease course was characterised by high fever and gastrointestinal symptoms. Cardiac function, as measured by echocardiography, remained stable. The treatment focused on maintaining a normal heart rate and a stable fluid balance. In children with severe underlying cardiac disease, even a mild SARS-CoV-2 infection can require close monitoring and compound treatment.

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.

The differential diagnosis of a left atrial mass on echocardiogram includes thrombus, vegetation, tumour, and other rare causes like septal aneurysm, pulmonary vein remnant, and septal haematoma. We present interesting echocardiographic images of a rare condition which can masquerade as a left atrial mass following cardiac surgery.

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.

Large coronary artery fistulas draining to the left heart structures causing heart failure are very rare. Interventional closure of such fistulas is limited to isolated reports, often in asymptomatic patients. Technical differences in these interventions include either deep arterial cannulation till their exit or transseptal arteriovenous circuit formation. Transcatheter closure of three large symptomatic fistulas in small children is reported.

A rare case of a patient with a large type IV aortico–left ventricular tunnel who underwent successful repair at 1 day after birth was described. To the best of our knowledge, no such cases that aortic opening was over 10 mm have been reported in the English literature. Our case demonstrated no leak or significant aortic regurgitation by a two-patch repair.

Arteriovenous malformations may present with significant haemodynamic compromise in the neonatal period, typically with high output cardiac failure that may be accompanied by hypoxia and right ventricular dysfunction. Targeted neonatal echocardiography performed by trained neonatologists provides an enhanced physiology-based approach that can guide treatment and minimise complications. We present a case of a large hepatic vascular malformation whose therapy was guided by targeted neonatal echocardiography to prevent paradoxical embolisation of procedural glue to the systemic circulation.

“Torsades de pointes”, a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting “torsades de pointes” after administration of fluconazole and rapidly improved upon cessation of the drug.

We report an innovative treatment strategy for fetal Ebstein’s anomaly with a circular shunt. We used transplacental non-steroidal anti-inflammatory drugs, at the 29th gestational week, to constrict the ductus arteriosus avoiding fetal demise. We addressed the critical neonate with an urgent Starnes procedure. Finally, instead of following the usual single-ventricle palliation pathway after the Starnes procedure, we achieved successful two-ventricle repair with the cone technique at 5 month old.

Isolated origin of the subclavian artery is a congenital anomaly of the aortic arch in which one subclavian artery is attached to the ipsilateral pulmonary artery through ligamentous arteriosus. An isolated right subclavian artery with the left-sided aortic arch is an extremely rare condition. We report on an asymptomatic 2-year-old-girl, who was referred because of an incidental cardiac a murmur. She was diagnosed by echocardiography to have an isolated right subclavian artery connected to the right pulmonary artery in a left aortic arch with a ventricular septal defect. MRI confirmed the findings.