Right ventricular failure after placement of left ventricular assist device in paediatric heart failure is associated with increased mortality. We report successful use of intravenous prostacyclin for right ventricular support and pulmonary hypertension after initiation of left ventricular assist device support. This suggests that intravenous prostacyclins may be an important therapy in right ventricular failure following ventricular assist device implantation.

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.

Anomalous circumflex coronary artery origin from pulmonary artery is a very rare congenital heart disease. Misdiagnosis of this condition may lead to inadequate myocardial protection during cardiopulmonary bypass surgery.

Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom.

We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians.

Our tool enables automatic creation of graphical summaries, displayed as a Microsoft PowerPoint presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window.

Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.

With the rapid expansion of the COVID-19 pandemic, the disease burden and its consequences on the paediatric population has been progressively recognised. Although COVID-19 infection in children presents as asymptomatic to mild illness, instances of hyperinflammation and multi-organ involvement following the viral infection have been described. This condition, known as the multisystem inflammatory syndrome in children (MIS-C), has gained a wide global attention. Despite the global efforts to uncover the disease characteristics and management, a clear pathogenesis and a unified treatment regimen have not been reached yet. This paper tackles the epidemiology of the MIS-C, discusses its suggested pathogenesis, drives through its varying clinical presentations, and evaluates the different treatment regimens employed in managing MIS-C.

Ductus arteriosus is a physiological structure if not closed after birth, may lead to many complications. Today, trans-catheter closure of patent ductus arteriosus with Occluder devices is the preferred method. Surgical ligation is used only in certain cases such as large symptomatic patent ductus arteriosus in very small infants and premature babies; unfavourable structure of the duct or economic considerations. In this article, we described haemodynamic and morphological characteristics of five patients with large patent ductus arteriosus which were occluded with Amplatzer device.

From 23 January, 2010 to 31 July, 2018, five patients referred to our clinic with large patent ductus arteriosus and pulmonary arterial hypertension for further evaluation. After assessing them with various diagnostic methods, we decided to close defect with ventricular septal defect Occluder device. Patients aged 21–44 years and one of them was male. Ductus closure was successfully done with ventricular septal defect Occluder device. Closure was successful for all of them but in one case, whose device was embolized to pulmonary artery after 24 hr and he underwent surgery.

Trans-catheter closure of large patent ductus arteriosus in adult patients with pulmonary hypertension is feasible. Despite the fact that complications may occur even with the most experienced hands, the ‘double disk’ Amplatzer ventricular septal defect muscular Occluder could be advantageous in this setting.

Currently, there is an increasing prevalence of liver nodules in patients following the Fontan operation. The appropriate non-invasive modalities have been applied to assess a diagnosis of Fontan-associated liver disease. The aims of this study were to determine the prevalence and associated factors for the presence of liver nodules using CT scan. A cross-sectional study of 34 patients older than 15 years of age was recruited. Ultrasound upper abdomen, ultrasound liver elastography, and CT scan of the upper abdomen were performed after the Fontan operation. The median age of patients was 20 years (range 14–36 years). The median age at the Fontan operation was 7 years (range 5–17 years) and the duration after the Fontan operation was 12 years (range 4–22 years). The prevalence of liver nodules was 62% as detected by CT scans. Hepatic vein pressure in patients with liver nodules was significantly higher than in those without liver nodules. Hepatic vein pressure above 13 mmHg was a factor associated with liver nodules. There was little agreement between the ultrasound of the upper abdomen and CT scan of the upper abdomen in the evaluation of liver nodules. Hepatic pressure was the only associated factor for the occurrence of liver nodules in patients following the Fontan operation. The prevalence of liver nodules was very high after the Fontan operation. The upper abdomen CT scan should be performed for the surveillance of liver nodules in every Fontan patient over the age of 15 years.

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.

The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

Acquired diaphragmatic hernia is a rare complication of pediatric intervention or surgery. In this study, we report an infant with iatrogenic diaphragmatic hernia following neonatal complex congenital cardiac surgery, and then we review the associated literature.

A 47-year-old with repaired ventricular septal defect and pulmonary valve stenosis as a child presents with chronic intermittent chest pain. CT evaluation for transcatheter pulmonary valve replacement revealed right coronary artery compression between a sternal wire and dilated right ventricle. Removal of the sternal wire resulted in improved symptoms.

We present a clinical case of a teenager in whom a Brugada syndrome electrical pattern was found which was finally diagnosed as a Brugada phenocopy due to a wrong setup of the high-pass filter on the electrocardiogram

We report a case of congenital giant left ventricular aneurysm with severely depressed systolic cardiac function who underwent early surgical resection with subsequent recovery of left ventricular systolic function.

HAPPY NEW YEAR! IT BECOMES INCREASINGLY EVI-dent that it is a truism that years go past quicker as we become older. Ironically, as time goes by faster, we must devote more of our attention to the future. For readers of Cardiology in the Young, as we enter our fifth year of publication, the future is indeed bright. As of this issue you join readers on six continents and scores of countries. As editors, we are most grateful for your continued support. You will notice changes that we are incorporating into this volume, beginning with a complete redesign of our cover and minor changes in the size of the journal. Further substantive editorial changes will be incorporated throughout the year, and we will keep you fully informed as we go. Exciting changes will occur as we augment participation from colleagues in Japan, China and throughout Asia. Please share with us your ideas, as we are amenable to any change or addition that will further improve the quality of Cardiology in the Young.

We describe three cases of newborns with complex CHD characterised by communication challenges. These communication challenges were categorised as patient, family, or system-related red flags. Strategies for addressing these red flags were proposed, for the goal of optimising care and improving quality of life in this vulnerable population.