Objective: To establish whether the use of near-infrared spectroscopy is potentially beneficial in high-risk cardiac infants in United Kingdom paediatric intensive care units. Design: A prospective observational pilot study. Setting: An intensive care unit in North West England. Patients: A total of 10 infants after congenital heart surgery, five with biventricular repairs and five with single-ventricle physiology undergoing palliation. Interventions: Cerebral and somatic near-infrared spectroscopy monitoring for 24 hours post-operatively in the intensive care unit. Measurement and main results: Overall, there was no strong correlation between cerebral near-infrared spectroscopy and mixed venous oxygen saturation (r=0.48). At individual time points, the correlation was only strong (r=0.74) 1 hour after admission. The correlation was stronger for the biventricular patients (r=0.68) than single-ventricle infants (r=0.31). A strong inverse correlation was demonstrated between cerebral near-infrared spectroscopy and serum lactate at 3 of the 5 post-operative time points (1, 4, and 12 hours: r=−0.76, −0.72, and −0.69). The correlation was stronger when the cerebral near-infrared spectroscopy was <60%. For cerebral near-infrared spectroscopy <60%, the inverse correlation with lactate was r=−0.82 compared with those cerebral near-infrared spectroscopy >60%, which was r=−0.50. No correlations could be demonstrated between (average) somatic near-infrared spectroscopy and serum lactate (r=−0.13, n=110) or mixed venous oxygen saturation and serum lactate. There was one infant who suffered a cardiopulmonary arrest, and the cerebral near-infrared spectroscopy showed a consistent 43 minute decline before the event. Conclusions: We found that cerebral near-infrared spectroscopy is potentially beneficial as a non-invasive, continuously displayed value and is feasible to use on cost-constrained (National Health Service) cardiac intensive care units in children following heart surgery.

Borderline left ventricle refers to a spectrum of left ventricular underdevelopment, typically associated with other cardiac anomalies. The left ventricle may be mildly hypoplastic, as is sometimes seen accompanying aortic coarctation, or it can be severely hypoplastic, as is seen in hypoplastic left heart syndrome. For patients with a borderline left ventricle that is at either extreme, the treatment decision is relatively straightforward. Those with the most severe form of left ventricle hypoplasia will require single ventricle palliation or cardiac transplantation, whereas those with the mildest form may not need any intervention. It is the management strategy of children that fall within the grey zone of the spectrum, which continues to be controversial and remains variable within and among different institutions. Cardiac diseases with associated left ventricle hypoplasia include critical aortic stenosis, mitral stenosis, coarctation of the aorta, arch hypoplasia, cor triatriatum, unbalanced common atrioventricular canal, Shone’s complex, total anomalous pulmonary venous return, and complex conotruncal abnormalities. In this review, we will discuss the assessment and management of infants with borderline left ventricle with critical aortic stenosis or arch obstruction and associated mitral anomalies.

Background: Most children in the Third World do not have access to treatment for heart diseases, as the priorities of health care are different from the developed countries. Materials and methods: Since 2009, teams supported by the Chain of Hope and Spanish medical volunteers have travelled twice a year to help develop paediatric cardiac services in the Cardiac Center in Ethiopia, undertaking four missions each year. As of December 2012, 296 procedures were performed on 287 patients. The procedures included 128 duct occlusions, 55 pulmonary valve dilations, 25 atrial septal defect closures, 14 mitral valve dilations, and others. The local staff were trained to perform a majority of these cases. Results: Procedural success was achieved in 264 (89.2%). There were three deaths, five device embolisations, and three complications in mitral valve dilation. During the visits, the local staff were trained including one cardiologist, six nurses, and two technicians. The local team performed percutaneous interventions on its own after a couple of years. The goal is also to enable the local team to perform interventions independently. Conclusion: Training of an interventional cardiology team in a sub-Saharan setting is challenging but achievable. It may be difficult for a single centre to commit to sending frequent missions to a developing country to make a meaningful contribution to the training of local teams. In our case, coordination between the teams from the two countries helped to achieve our goals.

Background: Detailed anatomic variants of the interatrial septum in patients with right-to-left shunt and contribution of specific anatomies to the risk of ischaemic recurrences has not yet been comprehensively classified. Objective: To report a classification of the anatomic variants of the interatrial septum as observed by intracardiac echocardiography and its correlation with clinical and functional characteristics. Methods: We retrospectively reviewed the medical and instrumental data of 520 consecutive patients (mean age 44±15. 5 years, 355 women) who had over a 10-year period undergone intracardiac echocardiography and right-to-left shunt catheter-based closure. The four main features used to analyse were: (a) diameter of the oval fossa, (b) presence and length of the channel, (c) presence and degree of atrial septal aneurysm, and (d) rim thickness. The presence of Eustachian valve was also tabulated. Results: The combinations of interatrial septum anatomical features were classified into six main anatomical subgroups. Recurrent embolism, multiple ischaemic foci on brain magnetic resonance imaging, high grade shunt, and permanent shunt before transcatheter closure procedure were associated with type 2, type 4, and type 6. Type 4 anatomical subtype (OR 4.1, 1.5–8 [95% CI], p<0.001) and type 2+presence of Eustachian valve (OR 4.3, 1.6–9 [95% CI], p<0.001) were the strongest predictors of recurrent ischaemic events before transcatheter closure. Conclusion: Our study showed that interatrial septum anatomy greatly differs among patients with right-to-left shunt, as well as the risk of ischaemic recurrences in different anatomies.

Acute heart failure is a common and serious complication of congenital and acquired heart disease, and it is associated with significant morbidity, mortality, and costs. When a patient is admitted to the hospital with acute heart failure, there are several important goals for the hospital admission, including maintaining adequate perfusion, establishing the underlying aetiology for the heart failure, patient and family education, and discharge from the hospital in a stable condition. The pathway to home discharge is variable and may include inotropic therapy, mechanical circulatory support, and/or heart transplantation. This review will cover the epidemiology, presentation, and management of acute heart failure in children.

Background: It is important to identify early predictors of functional limitations in children after congenital heart surgery to optimise their independence as they prepare for school. The purpose of this study is to determine potentially modifiable predictor variables of functional abilities in pre-school children who underwent complex cardiac surgery at 6 weeks of age or earlier. Methods: This prospective inception cohort study comprised a sample of 165 survivors (63% boys) who had complex cardiac surgery (75% biventricular repairs) at Stollery Children’s Hospital, Edmonton, Alberta. We excluded children with chromosomal abnormalities. When children were 4–5 years of age, the parents completed the Adaptive Behavioral Assessment System II. Regression analysis was used to assess the association between multiple risk factors and each of the four continuous composite scores. Results: The mean scores for the practical domain and general adaptive composite score of the Adaptive Behavioural Assessment System were lower than the conceptual and social domains, with 13.3% of the children having a delay in the practical domain. There was a significant association between the general adaptive (p=0.003; 0.012), conceptual (p=0.0004; 0.042), social (p=0.0007; 0.028), and the practical (p=0.046; 0.003) domain composite scores with the mother’s education and preoperative plasma lactate, respectively. Conclusion: Maternal education may be a marker for the social context of children, and warrants societal attention to improve functional outcomes. Preoperative lactate as a potentially modifiable variable may warrant increased attention to early diagnosis and aggressive resuscitation of young infants with congenital heart disease.

Introduction: Digoxin or propranolol are used as first-line enteral agents for treatment of infant supraventricular tachycardia. We used a large national database to determine whether enteral digoxin or propranolol was more effciacious as first-line infant supraventricular tachycardia therapy. Materials and Methods: The Pediatric Health Information System database was queried over 10 years for infants with supraventricular tachycardia initiated on enteral digoxin or propranolol monotherapy. Patients were excluded for Wolff–Parkinson–White, intravenous antiarrhythmics (other than adenosine), or death. Success was considered as discharge on the initiated monotherapy. Risk factors for successful monotherapy and risk factors for readmission for supraventricular tachycardia for patients discharged on monotherapy were determined. Results: A total of 374 patients (59.6% male) met the study criteria. Median length of stay was 7 days (interquartile range of 3–16 days). Patients had CHD (n=199, 53.2%) and underwent cardiac surgery (n=123, 32.9%), ICU admission (n=238, 63.6%), mechanical ventilation (n=146, 39.0%), and extracorporeal membrane oxygenation (n=3, 0.8%). Pharmacotherapy initiation was at median 37 days of life (interquartile range of 12–127 days) and 47.3% were initiated on digoxin. Success was similar between digoxin (73.1%) and propranolol (73.5%). Initial therapy with digoxin was not associated with success (odds ratio 1.01, 95% CI 0.64–1.61, p=0.93). Multivariable analysis demonstrated hospital length of stay (odds ratio 0.98, 95% CI 0.98–1.00) and involvement of a paediatric cardiologist (odds ratio 0.46, 95% CI 0.29–0.75) associated with monotherapy failure, and male gender (odds ratio 1.66, 95% CI 1.03–2.67) associated with monotherapy success. No variables were significant for readmission on multivariable analysis. Discussion: Digoxin or propranolol may be equally efficacious for inpatient treatment of infant supraventricular tachycardia.

This review offers a critical-care perspective on the pathophysiology, monitoring, and management of acute heart failure syndromes in children. An in-depth understanding of the cardiovascular physiological disturbances in this population of patients is essential to correctly interpret clinical signs, symptoms and monitoring data, and to implement appropriate therapies. In this regard, the myocardial force–velocity relationship, the Frank–Starling mechanism, and pressure–volume loops are discussed. A variety of monitoring modalities are used to provide insight into the haemodynamic state, clinical trajectory, and response to treatment. Critical-care treatment of acute heart failure is based on the fundamental principles of optimising the delivery of oxygen and minimising metabolic demands. The former may be achieved by optimising systemic arterial oxygen content and the variables that determine cardiac output: heart rate and rhythm, preload, afterload, and contractility. Metabolic demands may be decreased by a number of ways including positive pressure ventilation, temperature control, and sedation. Mechanical circulatory support should be considered for refractory cases. In the near future, monitoring modalities may be improved by the capture and analysis of complex clinical data such as pressure waveforms and heart rate variability. Using predictive modelling and streaming analytics, these data may then be used to develop automated, real-time clinical decision support tools. Given the barriers to conducting multi-centre trials in this population of patients, the thoughtful analysis of data from multi-centre clinical registries and administrative databases will also likely have an impact on clinical practice.

Objective: There are limited data on the outcomes of children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. The primary aim of this project is to identify the aetiology and outcomes of extracorporeal membrane oxygenation in children receiving delayed (≥7 days) extracorporeal membrane oxygenation after cardiac surgery. Patients and methods: We conducted a retrospective review of all children ≤18 years supported with delayed extracorporeal membrane oxygenation after cardiac surgery between the period January, 2001 and March, 2012 at the Arkansas Children’s Hospital, United States of America, and Royal Children’s Hospital, Australia. The data collected in our study included patient demographic information, diagnoses, extracorporeal membrane oxygenation indication, extracorporeal membrane oxygenation support details, medical and surgical history, laboratory, microbiological, and radiographic data, information on organ dysfunction, complications, and patient outcomes. The outcome variables evaluated in this report included: survival to hospital discharge and current survival with emphasis on neurological, renal, pulmonary, and other end-organ function. Results: During the study period, 423 patients undergoing cardiac surgery were supported with extracorporeal membrane oxygenation at two institutions, with a survival of 232 patients (55%). Of these, 371 patients received extracorporeal membrane oxygenation <7 days after cardiac surgery, with a survival of 205 (55%) patients, and 52 patients received extracorporeal membrane oxygenation ≥7 days after cardiac surgery, with a survival of 27 (52%) patients. The median duration of extracorporeal membrane oxygenation run for the study cohort was 5 days (interquartile range: 3, 10). In all, 14 patients (25%) received extracorporeal membrane oxygenation during active cardiopulmonary resuscitation with chest compressions. There were 24 patients (44%) who received dialysis while being on extracorporeal membrane oxygenation. There were eight patients (15%) who had positive blood cultures and four patients (7%) who had positive urine cultures while being on extracorporeal membrane oxygenation. There were nine patients (16%) who had bleeding complications associated with extracorporeal membrane oxygenation runs. There were 10 patients (18%) who had cerebrovascular thromboembolic events associated with extracorporeal membrane oxygenation runs. Of these, 19 patients are still alive with significant comorbidities. Conclusions: This study demonstrates that mortality outcomes are comparable among children receiving extracorporeal membrane oxygenation ≥7 days and <7 days after cardiac surgery. The proportion of patients receiving extracorporeal membrane oxygenation ≥7 days is small and the aetiology diverse.

The Fontan operation is the final step of palliation for patients with a functionally single ventricle. Since its introduction in the 1970s, the Fontan surgery has become part of a successful surgical strategy that has improved single ventricle mortality. In recent years, we have become more aware of the limitations and long-term consequences of the Fontan physiology. Pulmonary vascular resistance plays an important role in total cavopulmonary circulation, and has been identified as a potential therapeutic target to mitigate Fontan sequelae. In this review, we will discuss the results of different pulmonary vasodilator trials and the use of pulmonary vasodilators as a treatment strategy for Fontan patients.

Background: Lesions of adjacent structures have been reported after closure of large atrial septal defects with the Amplatzer septal occluder. In children, growth of the heart should modify the initial relationship between the device and surrounding structures. Aim: To compare the relationship between large Amplatzer septal occluder and adjacent cardiac structures at short-, mid-, and long-term follow-up in at-risk paediatric population using echocardiography. Methods: A total of 25 children (4.6±2.9 years old, 18 girls) with the largest atrial septal defect devices implanted between 1997 and 2002 were enrolled prospectively for complete echocardiogram 17.8±10.5 months (mid-term follow-up) and 8.8±0.9 years (long-term follow-up) after the procedure. Results were compared with the echocardiogram carried out 2.1±3.4 days after the procedure (short-term follow-up). Results: The minimal distance between the left disk and the mitral valve increased: 1.4±2.0 mm at short-term and 5.1±2.3 mm at long-term follow-up (p<0.05), leading to less contact between the disk and the anterior leaflet and less mitral regurgitation (10 at short-term, 4 at long-term follow-up, p<0.05). The number of devices straddling the aorta decreased from 17 to 12 at long-term follow-up (p<0.05). There was protrusion of disk in the venous structure in seven patients on the first echocardiogram, which disappeared at long-term follow-up. Conclusion: Although frequently in close contact with the aortic root, mitral valve, or venous returns, large devices tend to centre and move away from the surrounding structures, with decreased risk for long-term distortion.

Aim: We aimed to investigate the role of adiponectin in acute rheumatic fever by evaluating correlations with cytokines and acute-phase reactants. Methods: Patients were divided into three groups by clinical findings. Group 1 included 8 patients with only chorea, Group 2 included 13 patients with arthritis and carditis, and Group 3 included 12 patients with only carditis. A total of 54 age- and gender-matched children were enrolled in the control group. Blood samples were drawn for analysing acute-phase reactants, adiponectin, tumour necrosis factor-α, interleukin-6, and interleukin-8 levels at baseline on Days 2, 5, 10, and 15, and at 8 weeks. Results: There was no statistically significant difference between baseline age, gender, body mass index, serum triglyceride, total cholesterol, and low-density lipoprotein levels of the study and control groups (p>0.05). No correlation was found between baseline plasma adiponectin levels, age, body mass index, follicle-stimulating hormone, luteinising hormone, oestradiol, total testosterone, and blood lipid levels of the study and control groups (p>0.05). We found that adiponectin and interleukin-6 levels increased, tumour necrosis factor-α levels decreased, and interleukin-8 levels remained unchanged in acute rheumatic fever, which is an inflammatory disease. Moreover, adiponectin level was higher and tumour necrosis factor-α level was lower in the improvement period in comparison with the acute period, particularly in the carditis group. Conclusion: It was considered that, increasing throughout the treatment period, adiponectin may have anti-inflammatory effects in acute rheumatic fever. In addition, adiponectin levels are associated with a decline in inflammatory mediators in rheumatic fever.

Objective: We sought to determine the prevalence of attention-deficit/hyperactivity disorder in a population of children who underwent neonatal heart surgery involving repair of the aortic arch for Norwood Stage I, interrupted aortic arch, and combined repair of aortic coarctation with ventricular septal defect. Methods: Children between the ages of 5 and 16 were surveyed using the ADHD-IV and the Child Heath Questionnaire-50. Classification as attention-deficit/hyperactivity disorder was defined for this study as either a parent-reported diagnosis of attention-deficit/hyperactivity disorder or ADHD-IV inattention score of ⩾93 percentile. Results: Of the 134 surveys, 57 (43%) were returned completed. A total of 25 (44%) children either had a diagnosis of attention-deficit/hyperactivity disorder and/or ADHD-IV inattention score ⩾93 percentile. Eleven of the 13 (85%) children with interrupted aortic arch, 3 of the 7 (42.9%) children with combined coarctation/ventricular septal defect repair, and 9 of the 33 (27.3%) children with hypoplastic left-heart syndrome were classified as having attention-deficit/hyperactivity disorder. Only 7 of the 25 (28%) children received medical treatment for this condition. Quality of life indicators in the Child Heath Questionnaire-50 Questionnaire were highly correlated with the ADHD-IV scores. Conclusion: The risks for the development of attention-deficit/hyperactivity disorder are multifactorial but are significantly increased in this post-surgical population. This study revealed a low treatment rate for attention-deficit/hyperactivity disorder, and a significant impact on the quality of life in these children.

Heart failure in children can result from a wide range of aetiologies and can manifest in systolic and/or diastolic dysfunction. Echocardiography is the primary test for the diagnosis and follow-up of children with heart failure. In this article, we critically review standard echocardiographic measurements that have been shown to have prognostic importance in children with various types of heart failure. Each of the common forms of cardiomyopathy that is encountered in childhood – dilated, hypertrophic, restrictive, left ventricular non-compaction, and arrhythmogenic right ventricular cardiomyopathy – is discussed separately. Special attention is paid to the failing right ventricle, both in the systemic and in the sub-pulmonary position, to the failing univentricular heart, and to the assessment of diastolic function in children.

At present, there is significant interest in the morphology of the coronary arteries, not least due to the increasingly well-recognised association between anomalous origin of the arteries and sudden cardiac death. Much has also been learnt over the last decade regarding the embryology of the arteries. In this review, therefore, we provide a brief introduction into the recent findings regarding their development. In particular, we emphasise that new evidence, derived using the developing murine heart, points to the arterial stems growing out from the adjacent sinuses of the aortic root, rather than the arteries growing in, as is currently assumed. As we show, the concept of outgrowth provides an excellent explanation for several of the abnormal arrangements encountered in the clinical setting. Before summarising these abnormal features, we draw attention to the need to describe the heart in an attitudinally appropriate manner, following the basic rule of human anatomy, rather than describing the cardiac components with the heart in the “Valentine” orientation. We then show how the major abnormalities involving the coronary arteries in humans can be summarised in terms of abnormal origin from the pulmonary circulation, abnormal aortic origin, or fistulous communications between the coronary arteries and the cardiac cavities. In the case of abnormal aortic origin, we highlight those malformations known to be associated with sudden cardiac death.

Background: Transcatheter closure of coronary artery fistula has emerged as a safe and effective alternative to surgery. However, follow-up angiographic data after closure of the coronary artery fistula is extremely limited. We report our clinical and angiographic follow-up of children who underwent either transcatheter or surgical closure. Method: Clinical profile, echocardiography parameters, and closure technique were retrospectively reviewed from the hospital charts. Since 2007, 15 children have been intervened and followed up with electrocardiography, echocardiography, and angiography. Results: A total of 15 children (six girls), with mean age of 6.7±5.4 years and weighing 16.3±9.8 kg, underwent successful closure (transcatheter=13, surgical=2) without periprocedural complication. Coronary artery fistula arose from the right (n=7) and left coronary artery (n=8) and drained into the right atrium or the right ventricle. Transcatheter closure was carried out using a duct occluder. Of the patients, two underwent surgical closure of the fistula on a beating heart. At 31.8±18.7 months follow-up, all the children were asymptomatic and had no evidence of myocardial ischaemia or infarction. However, follow-up angiography revealed thrombotic occlusion of fistula with the patent parent coronary artery in those having branch coronary artery fistula, and five of seven patients with parent coronary artery fistula had near-complete occlusion of fistula extending into the native coronary artery. Conclusion: Follow-up angiography revealed a high incidence of parent artery occlusion when the fistula was arising from the native artery and not from one of its branches. Coronary artery fistula intervention of the parent coronary artery fistula always carries the potential risk of ischaemia, unless the distal-most exiting segment is the primary site of occlusion.

Introduction: A known comorbidity of congenital cardiovascular malformations is low birth weight, but the reasons for this association remain obscure. This retrospective study examines the relationship between congenital cardiovascular malformations and the birth weight of singletons, taking into account differences in gestational age and other factors. Methods: Using data from the retrospective, population-based Baltimore–Washington Infant Study, six types of congenital cardiovascular malformations were investigated in comparison with controls (n=3519) through linear regression: d-transposition of the great arteries (n=187), other conotruncal heart defects (n=361), endocardial cushion defects (n=281), left heart obstructive lesions (n=507), atrial septal defects (n=281), and membranous ventricular septal defects (n=622). Results: Infants with conotruncal heart defects (−218 g), endocardial cushion defects with Down syndrome (−265 g), endocardial cushion defects without Down syndrome (−194 g), left heart obstructive lesions (−143 g), atrial septal defects (−150 g), and membranous ventricular septal defects (−127 g) showed significant birth weight deficits, adjusting for gestational age, and other covariates. Infants with d-transposition of the great arteries (−67 g) did not show significant birth weight deficits compared with the control group. Discussion: The degree of birth weight decrement appears to be highly related to the specific type of congenital cardiovascular malformation. As a whole, these infants do not exhibit low birth weights solely because of being premature, and thus other mechanisms must underlie these associations.