We report a case of a newborn infant with coarctation of the aorta and hypoplastic transverse aortic arch who was found to have a circumaortic double left innominate vein on echocardiography. This exceedingly rare finding was important for surgical planning and was confirmed during congenital heart surgery.

We present an infant with incidentally found retro-aortic left brachiocephalic vein draining directly into azygous vein. This rare vascular variant is asymptomatic but does have clinical implications with surgical and interventional procedures.

Body mass index, race/ethnicity, and payer status are associated with operative mortality in congenital heart disease (CHD). Interactions between these predictors and impacts on longer term outcomes are less well understood. We studied the effect of body mass index, race/ethnicity, and payer on 1-year outcomes following elective CHD surgery and tested the degree to which race/ethnicity and payer explained the effects of body mass index. Patients aged 2–25 years who underwent elective CHD surgery at our centre from 2010 to 2017 were included. We assessed 1-year unplanned cardiac re-admissions, re-interventions, and mortality. Step-wise, multivariable logistic regression was performed.

Of the 929 patients, 10.4% were underweight, 14.9% overweight, and 8.5% obese. Non-white race/ethnicity comprised 40.4% and public insurance 29.8%. Only 0.5% died prior to hospital discharge with one additional death in the first post-operative year. Amongst patients with continuous follow-up, unplanned re-admission and re-intervention rates were 14.7% and 12.3%, respectively. In multivariable analyses adjusting for surgical complexity and surgeon, obese, overweight, and underweight patients had higher odds of re-admission than normal-weight patients (OR 1.40, p = 0.026; OR 1.77, p < 0.001; OR 1.44, p = 0.008). Underweight patients had more than twice the odds of re-intervention compared with normal weight (OR 2.12, p < 0.001). These associations persisted after adjusting for race/ethnicity, payer, and surgeon.

Pre-operative obese, overweight, and underweight body mass index were associated with unplanned re-admission and/or re-intervention 1-year following elective CHD surgery, even after accounting for race/ethnicity and payer status. Body mass index may be an important modifiable risk factor prior to CHD surgery.

Pulmonary pressure is one of the most important parameters in the postoperative follow-up of patients who have undergone the Glenn procedure. Platelet activation markers, which are inexpensive and easily accessible blood count parameters, have been shown to be associated with the aetiology and pathogenesis of primary pulmonary artery hypertension. We examined the relationship between platelet activation markers and pulmonary pressures in the early postoperative period of patients who underwent the Glenn procedure.

Eighty-five patients who underwent the Glenn procedure in our clinic between January 2011 and March 2020 were included in the study retrospectively. Fifty-one patients were male and 34 were female, and age varied from 4 to 416 months, with a mean of 28.64 ± 51 months.

Patients with increased pulmonary blood flow on palliation before Glenn surgery had higher mean platelet volume values. However, no correlation was found between pulmonary pressures and platelet activation markers in the early postoperative period.

There was not similar study evaluating platelet activation markers in the paediatric age group before and after postoperative Glenn surgery in the literature. Therefore, even if platelet activation markers provide information about the pulmonary bed, they may be misleading due to other reasons that trigger bleeding and inflammatory processes in the early postoperative period.

Intracoronary wave intensity analysis in hypertrophic cardiomyopathy has shown a large backward compression wave due to compressive deformation of the intramyocardial coronary arteries in systole. The authors describe the angiographic evidence of this backward compression wave, which has not been described in this physiological context and can be a marker of poor prognosis.

A young adult with late diagnosis of scimitar syndrome underwent infradiaphragmatic baffling of the scimitar vein to left atrium through an intra-atrial tunnel using PhotoFix® bovine pericardium with recurrent extensive fibrovascular granulation of the patch causing pulmonary and systemic venous obstruction leading to eventual explantation of the bovine pericardium.

We present the case of a 13-year-old male with a complex congenital cardiac history who was supported with extracorporeal membrane oxygenation for 394 days while awaiting cardiac transplantation. The patient underwent successful cardiac transplantation after 394 days of support with veno-arterial extracorporeal membrane oxygenation and is currently alive 2 years after cardiac transplantation. We believe that this case represents the longest period of time that a patient has been supported with extracorporeal membrane oxygenation as a bridge to cardiac transplantation.

We also review the literature associated with prolonged support with extracorporeal membrane oxygenation. This case report documents many of the challenges associated with prolonged support with extracorporeal membrane oxygenation, including polymicrobial bacterial and fungal infections, as well as renal dysfunction. It is possible to successfully bridge a patient to cardiac transplantation with prolonged support with extracorporeal membrane oxygenation of over 1 year; however, multidisciplinary collaboration is critical.

A 17-year-old boy with a history of dyspnea attacks and chest pain was referred to our paediatric cardiology department. Electrocardiogram at presentation showed T-wave inversion in the inferior leads. Cardiovascular magnetic resonance imaging revealed the rare diagnosis of apical hypertrophic cardiomyopathy with subendocardial late gadolinium enhancement, missed by echocardiography.

We describe the healing process following transcatheter implantation of the Nit-Occlud ASD-R occluder (PFM medical, Cologne, Germany) for atrial septal defect closure in a sheep model with histological confirmation of neotissue formation covering the device.

This is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. She required extracorporeal cardiopulmonary support because of ventricular dysfunction and mitral regurgitation. Although she was temporarily weaned off the support after mitral valvuloplasty, she died from multiple organ failure. To the best of our knowledge, bilateral coronary ostial atresia associated with pulmonary atresia with ventricular septal defect has not been reported previously.

Gastrointestinal bleeding complicated with protein-losing enteropathy after the Fontan procedure has been often reported in recent years, but there is no established therapy for it.

We report the case of an 18-year-old boy who suffered from abdominal pain, melena, and anaemia due to intractable haemorrhagic protein-losing enteropathy after the Fontan procedure. He was successfully treated with octreotide therapy.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu’s arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu’s arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu’s arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu’s arteritis within the paediatric community.

Two cases of paediatric patients with gastric pacemakers causing distinct electrocardiographic artefact. Recognition of extracardiac artefact is essential for proper ECG interpretation in patients.

Anthracycline-related cardiomyopathy is of concern in children treated for acute myeloid leukemia (AML). Risk is dose-dependent, increasing with higher doses. We aim to highlight the risk of early-onset cardiotoxicity with low-cumulative anthracycline dose in a young Omani boy with AML. We conclude in the presence of other known risk factors for cardiac dysfunction, there is probably no risk-free anthracycline dose.

Chest radiography compares left ventricular decompression in the same patient supported with extracorporeal membrane oxygenation with atrial septal fenestration and subsequently supported with left ventricular assist device with apical cannulation.