We describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.

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.

Cystic echinococcosis caused by infection with the larvae form of Echinococcus granulosus remains highly endemic and constitutes a public health concern in some regions of the world. In this case report, we present a rare children case of interventricular hydatid cyst with a size of approximately 5 cm and its successful treatment.

We report on successful endovascular retrieval of an accidentally flared Melody valve in an adult patient with an indication for percutaneous pulmonary valve implantation. The Melody valve was removed through a 24 F sheath, introduced via the right jugular vein, and the urgent open-heart surgery was avoided.

There is minimal data regarding antegrade-only accessory pathways in young patients. Given evolving recommendations and treatments, retrospective analysis of the clinical and electrophysiologic properties of antegrade-only pathways in patients <21 years old was performed, with subsequent comparison of electrophysiology properties to age-matched controls with bidirectional pathways. Of 522 consecutive young patients with ventricular pre-excitation referred for electrophysiology study, 33 (6.3%) had antegrade-only accessory pathways. Indications included palpitations (47%), chest pain (25%), and syncope (22%). The shortest value for either the accessory pathway effective refractory period or the pre-excited R-R interval was taken for each patient, with the median of the antegrade-only group significantly greater than shortest values for the bidirectional group (310 [280–360] ms versus 270 [240–302] ms, p < 0.001). However, the prevalence of pathways with high-risk properties (effective refractory period or shortest pre-excited R-R interval <250 ms) was similar in both study patients and controls (13% versus 21%) (p = 0.55). Sixteen patients had a single antegrade-only accessory pathway and no inducible arrhythmia. Six patients had Mahaim fibres, all right anterolateral with inducible antidromic reciprocating tachycardia. However, 11 patients with antegrade-only accessory pathways and 3 with Mahaim fibres had inducible tachycardia due to a second substrate recognised at electrophysiology study. These included concealed accessory pathways (7), bidirectional accessory pathways (5), and atrioventricular node re-entry (2). Antegrade-only accessory pathways require comprehensive electrophysiology evaluation as confounding factors such as high-risk conduction properties or inducible Supraventricular Tachycardia (SVT) due to a second substrate of tachycardia are often present.

Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

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.

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.

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

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.

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.

“All the vital mechanisms, however varied they may be, have only one object, that of preserving constant the conditions of life in the internal environment.”1

An essential function of the cardiopulmonary system is to generate sufficient flow of oxygenated blood around the circulation in order to maintain normal cellular metabolism. The systemic delivery of oxygen is a function of the cardiac output and the content of oxygen in the systemic arterial blood, while the extent to which metabolising tissues require this oxygen for the maintenance of their integrity and function defines the systemic consumption of oxygen. As metabolising tissues have no mechanism for storing oxygen, they depend on its continuous supply, which must at least match their changing demands. As a result, it is a fundamental requirement of survival that the systemic consumption of oxygen, at all times, is matched by appropriate levels of its delivery.

We describe the echocardiographic findings of a common arterial trunk with intact ventricular septum, mitral and left ventricular hypoplasia, atretic left ventricular outlet and bilateral, and unbalanced pulmonary artery stenoses.

We describe a rare case of acute pulmonary artery thromboembolism in a 17-year-old male patient who presented to our emergency department following a syncopal episode. A chest radiograph showed a convex pulmonic cone and an increased cardiothoracic index, and two-dimensional echocardiogram suggested near-occlusion of both pulmonary arterial branches. Multi-slice pulmonary angio-tomography revealed massive thrombosis of the pulmonary artery. He was treated with systemic anticoagulation and subsequently required surgical thrombectomy, with favourable early outcome. Although the cause of the thromboembolism remains unproven, we discuss possible etiologies.