An asymptomatic 5-year-old male was diagnosed with severe coarctation of the aorta despite normal peak flow velocity with pathology identified on the basis of Doppler flow profile.

Transcatheter closure of secundum-type atrial septal defects has become the treatment of choice in the majority of cases. Femoral venous access is the standard rout for device implantation. Anatomic abnormalities of venous system including interrupted inferior caval vein with azygous continuation can make the percutaneous procedure more complicated. In such instances, alternative methods of transjugular or transhepatic approach or surgical repair should be considered. We present the case of a 50-year-old male with secundum-type atrial septal defect and a rare form of segmental interruption of inferior caval vein and describe successful atrial septal defect closure through transfemoral approach.

Coronary artery disease and myocardial infarction are known complications of long-standing diabetes mellitus in adults, but coronary artery spasm is far more rare and has not been reported in children. We present a 15-year-old male in diabetic ketoacidosis who developed diffuse ST segment elevations and elevated troponin with normal coronary arteries on coronary angiography and no signs of pericarditis that was due to coronary artery spasm.

Although rare, children with active coronavirus disease 2019 are at risk of developing malignant arrhythmia. Herein, we present the first paediatric case of refractory ventricular tachycardia from acute fulminant myocarditis secondary to acute COVID-19 infection. This 5-year-old boy required venoarterial extracorporeal membrane oxygenation support, but made a complete recovery without significant morbidity.

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.

Pregnancy in a woman with double outlet right ventricle, ventricular septal defect, and pulmonary stenosis is presented. Her previous two pregnancies ended in fetal losses. In the current pregnancy, she had thrombocytopenia, placenta previa, and severe fetal growth restriction. She underwent an emergency caesarean section followed by post-partum haemorrhage and COVID-19 infection. Literature review has been performed.

Stenosis of the Inferior Caval Vein is rarely encountered in the paediatric setting. A 5-year-old male sustained severe injuries secondary to a fall from a three story balcony and was subsequently found to have severe stenosis of the inferior caval vein resulting in extensive lymphatic drainage with chylothorax, chyloperitoneum, and severe abdominal ascites. This was successfully treated with transcatheter stent placement resulting in complete resolution of the stenosis and significant clinical improvement allowing for transfer to a rehabilitation centre and eventual discharge home.

Aneurysm and pseudoaneurysm development is a known, albeit uncommon, complication after right ventricular outflow tract surgical reconstruction. Large right ventricular outflow tract aneurysms and pseudoaneurysms have not been extensively described in recent literature and we report our experience with this unusual complication in five patients at our institution over the last 8 years. Although uncommon, this complication has potentially important clinical implications. Thus, clinicians should be aware of its potential, particularly in certain anatomic conditions.

Transcatheter closure of ventricular septal defects is considered first-line therapy when anatomically appropriate but is often challenged by proximity to the conduction system in perimembranous defects, or irregular defect shape, especially residual defects that may remain post-operatively. Advancements in device design, however, have allowed for significant improvements in deployment techniques and overall safety. Here we describe the first use of the Lifetech Konar-multifunction™ occluder device in North America, and our specific use of this device to close complex post-operative muscular and a perimembranous-ventricular septal defects in the same patient.

We present a rare and challenging case of left ventricular aneurysm in an African child with no history of previous infection or trauma, admitted for surgical treatment, who presented non reversible cardiorespiratory arrest with cardiorespiratory resuscitation before surgery.

Dilated cardiomyopathy is characterised by dilatation and impaired contraction of the left ventricle or both ventricles, which is the most common childhood cardiomyopathy. In recent years, it has been recognised that many sorts of genetic mutations may contribute to dilated cardiomyopathy. We now report a rare association of dilated cardiomyopathy with site mutation of BMPR2 gene. We did not find such an association reported in the medical literature.

We report a case of a pseudoaneurysm in the sinus of Valsalva, secondary to infective endocarditis in a child with trisomy 21. The patient had a history of subaortic stenosis, bicuspid aortic valve, and ventricular septal defect. Patch closure of the ostium of the pseudoaneurysm and aortic valve replacement was performed. The patient was discharged without severe complications.

Malonyl-CoA, a product of acetyl-CoA carboxylase is a metabolic intermediate in lipogenic tissues that include liver and adipose tissue, where it is involved in the de novo fatty acid synthesis and elongation. Malonyl-CoA decarboxylase (MLYCD, E.C.4.1.1.9), a 55-kDa enzyme catalyses the conversion of malonyl-CoA to acetyl-CoA and carbon dioxide, thus providing a route for disposal of malonyl-CoA from mitochondria and peroxisomes, whereas in the cytosol, the malonyl-CoA pool is regulated by the balance of MLYCD and acetyl-CoA carboxylase activities. So far, 34 cases with different MLYCD gene defects comprising point mutations, stop codons, and frameshift mutations have been reported in the literature. Here, we describe the follow-up of a patient affected by malonic aciduria upon neonatal onset. Molecular analysis showed novel homozygous mutations in the MLYCD gene. Our findings expand the number of reported cases and add a novel variant to the repertoire of MLYCD mutations.

There are three cusps in a normally developed aortic valve. Abnormal excavation or fusion, during the embryological development of the aortic valve, results in a varying number of cusps. Bicuspid aortic valve is the most common, but more rarely, unicuspid and quadricuspid aortic valves can be seen.

Here, a case of a 16-year-old male with a unicommissural unicuspid aortic valve and a case of a 13-year-old female with a quadricuspid aortic valve were reported.

Kawasaki disease is usually self-limited, but it can lead to aneurysm, stenosis, thrombosis, and myocardial infarction in the coronary arteries. The most important complication of Kawasaki disease is coronary artery aneurysm. Coronary artery aneurysm or ectasia may be seen in 15–25% of patients who do not receive treatment. It develops in 5% of children who receive intravenous immunoglobulin at the appropriate time. Acute myocardial infarction is the most important cause of morbidity and mortality in Kawasaki patients with giant aneurysms. We present a 10-year-old girl who had a history of giant aneurysm in the coronary arteries and underwent percutaneous coronary intervention due to anterior myocardial infarction.

Acquired and congenital left ventricular to right atrial communication is rare, but nowadays, the frequency of the iatrogenic subgroup is increasing. Successful transcatheter closure of these defects with different devices has been reported. Herein, we presented successful closure of left ventricular to right atrial communication with Amplatzer Duct Occluder 2 after attempting to close with a failed Amplatzer Vascular Plug II device in a 7-year-old girl. This report supports that transcatheter closure of iatrogenic Gerbode defect with Amplatzer Duct Occluder 2 device is safe and effective.