We have previously shown that children with a bioelectrical impedance spectroscopy phase angle at 50° (PA 50°) of <2.7 on postoperative day 2 had a four-fold increase in the risk of prolonged paediatric intensive care length of stay. In this study, we demonstrate a relationship between a baseline measure of phase angle 200/5° and postoperative length of stay.

A 4.5-year-old child with severe idiopathic pulmonary arterial hypertension underwent right-heart catheterisation, showing suprasystemic pulmonary pressure and a tiny patent ductus arteriosus with trivial right-to-left shunting. The ductus arteriosus was successfully stented with a bare metal stent. At 1-year follow-up, a remarkable improvement was observed in clinical status, functional capacity, and serology. Echocardiography confirmed a favourable cardiac remodelling.

Background: Childhood obesity has increased in the last half of the century. The aim of this study was to evaluate the frequency of obesity in the children with congenital or acquired heart disease. Methods: A total of 1410 children were assessed in this study. The study population was composed of 518 children (289 boys, 229 girls) as control group and 892 children (477 boys, 415 girls) as heart disease group. Patients were grouped into four categories: (I) “Clinic control subjects”; (II) “mild heart disease” that has not been treated with either surgical or catheter intervention; (III) congenital heart disease treated with surgical and/or catheter intervention; and (IV) “arrhythmias”. A body mass index ⩾85th percentile was defined as overweight, ⩾95th percentile as obese, and <5th percentile was defined as underweight. Results: We did not detect any association between heart disease and obesity. There was no difference in the rates of overweight, obesity, and underweight between the healthy control subjects and patients with heart disease (8.1%, 13.3%, and 5.0%; 9.0%, 10.7%, and 4.7%, respectively, p=0.145). All subgroups had a similar prevalence of underweight, overweight, and obesity as the healthy control population. Within the heart disease population, the overall prevalence rates for overweight, obesity, and underweight were similar between the boys and girls. Conclusion: Obesity is a common problem in children with heart disease, at least in general population. It is an important additional risk factor for long-term cardiovascular morbidity and mortality in children with heart disease. Precautions to prevent obesity should be a part of paediatric cardiologist’s examination.

A quiet and bloodless field providing optimal surgical conditions has been a crucial prerequisite for the performance of complex cardiac repairs in early life. The use of deep hypothermic circulatory arrest has fulfilled this role, and has been a catalyst for the development of neonatal and infant cardiac surgery. The recently increased awareness of possibly increased incidence of adverse neurological events and developmental outcome associated with this technique,1–5 however, has led to a general trend away from its use. In its place, techniques have been developed to provide cerebral perfusion during reconstruction of the aortic arch and the Norwood operation. Some have described the techniques as regional low-flow perfusion. In our opinion, they are described more accurately as antegrade regional cerebral perfusion. In this review, we discuss the recently described techniques for such antegrade regional cerebral perfusion during surgery on the aortic arch, with emphasis both on the Norwood operation and the observed physiological changes in the cerebral and systemic circulations. The neurologic and developmental outcomes following the use of the technique are still unknown.

Kounis Syndrome is characterised by the concurrence of acute coronary syndrome with mast cell activation induced by inflammatory mediators released during an allergic reaction. Although several factors and diseases were reported to be associated with Kounis Syndrome, branched-chain amino acid supplements have not been previously reported as a cause of Kounis Syndrome. We present a 17-year-old boy admitted to our hospital with thoracic pain after the ingestion of a branched-chain amino acid supplement.

Severe pulmonary hypertension is a contraindication for liver transplantation owing to high mortality. However, decision-making regarding the treatment approach for patients with bilateral peripheral pulmonary artery stenosis, typically complicated by elevated main pulmonary artery and right ventricle pressures, can be challenging. Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.

Cardiac fibromas are rare benign tumours of connective tissue that occur most frequently in children within the left ventricle. Spontaneous regression has not been observed, and surgical intervention is usually required. We have successfully treated a 1-year old girl with a giant fibroma of the right ventricle using the principles of the Batista procedure. The diagnosis was primarily made using transthoracic echocardiography.

Increasing numbers of children who have undergone intracardiac repair of tetralogy of Fallot have survived to adulthood, but often have residual haemodynamic and/or electrical abnormalities. We report the unusual observation of 4 instances of cerebrovascular accidents in young adults subsequent to surgical repair of tetralogy of Fallot.

Aim: This study aimed to compare the effects of dexmedetomidine–propofol and ketamine–propofol sedation on haemodynamic stability, immobility, and recovery time in children who underwent transcatheter closure of atrial septal defects. Methods: In all, 46 children scheduled for transcatheter closure of atrial septal defects (n = 46) were included. The dexmedetomidine–propofol group (n = 23) received dexmedetomidine (1 μg/kg) and propofol (1 mg/kg) for induction, followed by dexmedetomidine (0.5 μg/kg/hour) and propofol (100 μg/kg/minute) for maintenance. The ketamine–propofol group (n = 23) received ketamine (1 mg/kg) and propofol (1 mg/kg) for induction, followed by ketamine (1 mg/kg) and propofol (100 μg/kg/minute) for maintenance. Results: In all, 11 patients in the dexmedetomidine group (47.8%) and one patient (4.3%) in the ketamine group demonstrated a decrease ≥20% from the baseline in mean arterial pressure (p = 0.01). Heart rates decreased ≥20% from the baseline value in 10 patients (43.4%) in the dexmedetomidine group and three patients (13%) in the ketamine group (p = 0.047). Heart rate values were observed to be lower in the dexmedetomidine group throughout the procedure after the first 10 minutes. The number of patients requiring additional propofol was higher in the dexmedetomidine group (p = 0.01). The recovery times were similar in the two groups – 15.86 ± 6.50 minutes in the dexmedetomidine group and 19.65 ± 8.19 minutes in the ketamine group; p = 0.09. Conclusion: The ketamine–propofol combination was less likely to induce haemodynamic instability, with no significant change in recovery times, compared with the dexmedetomidine–propofol combination. The ketamine–propofol combination provided good conditions for the intervention.

Background: Children with myocarditis have multiple risk factors for thrombotic events, yet the role of antithrombotic therapy is unclear in this population. We hypothesised that thrombotic events in critically ill children with myocarditis are common and that children with myocarditis are at higher risk for thrombotic events than children with non-inflammatory dilated cardiomyopathy. Methods: This is a retrospective chart review of all children presenting to a single centre cardiac intensive care unit with myocarditis from 1995 to 2008. A comparison group of children with dilated cardiomyopathy was also examined. Antithrombotic regimens were recorded. The primary outcome of thrombotic events included intracardiac clots and any thromboembolic events. Results: Out of 45 cases with myocarditis, 40% were biopsy-proven, 24% viral polymerase chain reaction-supported, and 36% diagnosed based on high clinical suspicion. There were two (4.4%) thrombotic events in the myocarditis group and three (6.7%) in the dilated cardiomyopathy group (p = 1.0). Neither the use of any antiplatelet or anticoagulation therapy, use of intravenous immune globulin, presence of any arrhythmia, nor need for mechanical circulatory support were predictive of thrombotic events in the myocarditis, dilated cardiomyopathy, or combined groups. Conclusions: Thrombotic events in critically ill children with myocarditis and dilated cardiomyopathy occurred in 6% of the combined cohort. There was no difference in thrombotic events between inflammatory and non-inflammatory cardiomyopathy groups, suggesting that the decision to use antithrombotic prophylaxis should be based on factors other than the underlying aetiology of a child's acute decompensated heart failure.

Exercise capacity is a modifiable factor in patients with CHD that has been related to surgical outcomes in adults. We hypothesised that this was true for children undergoing surgical pulmonary valve replacement; therefore, the relationship of preoperative percent predicted peak oxygen consumption to surgical outcomes as measured by total hospital length of stay was explored.

Chest pain in young adults presents a unique diagnostic challenge, placing young patients at an increased risk to be misdiagnosed, as this patient population typically does not demonstrate the traditional risk factors associated with cardiovascular disease. This study details the case of a 16-year-old male who presented with new-onset chest pain and ST elevation on electrocardiogram. His history was unremarkable for known cardiac risk factors, but laboratory evaluation demonstrated markedly elevated troponins and electrocardiographic findings confirmed ST-segment elevation myocardial infarction. Coronary angiography demonstrated 100% occlusion of the left anterior descending artery, which was managed with percutaneous transluminal coronary angioplasty, thrombectomy, and bare-metal stenting. The patient had an uneventful recovery. This study examines the major causes of ST elevation myocardial infarction in young adults and reviews the major differences between younger and older myocardial infarction populations with emphasis on risk factor profile, pathophysiological mechanisms, clinical presentation, angiographic findings, and prognosis. This review highlights the need for consideration of a wide differential in younger subsets of the population presenting with chest pain and ST elevation. The implementation of current adult management protocols and guidelines for ST elevation myocardial infarction should not be overlooked due to age. Given the potential for premature death and long-term disability with resulting individual and societal consequences, it is crucial to understand the importance of correct diagnostic evaluation in this clinical scenario.

The Long List

The Long List is a comprehensive hierarchical system of coding and classification for the diagnosis and treatment of cardiac disease. The list was first published in 2000, as part of the standard coding system recommended by the Association for European Paediatric Cardiology for use across Europe. It embraces the entirety of the diagnosis and therapy of children with congenital and acquired cardiac disease. The Long List, as a whole, was adopted by the Association in 1998. Its development and expansion has been detailed previously. In essence, it grew from a list of 507 purely diagnostic terms published in 1985, through to a 1,777 item, hierarchical 12 tree structure in the Netherlands by 1988, and then to a single hierarchical structure of over 4,300 items in London by 1994. Although originally aimed at the clinician or surgeon treating cardiac disease first appearing in infancy or childhood, during this time it was enlarged to encompass abnormalities first diagnosed in fetal life, as well as cardiac disease first acquired during adult life. The list published in 2000 was composed of 4,777 items. These were made up of 3,906 individual terms, most of whom were mutually exclusive and unambivalent, given the constraints of clinical ambiguities and differing cultures of practice.