To perform a systematic review and a meta-analysis of the effects of balloon atrial septostomy on peri-operative brain injury in neonates with transposition of the great arteries.

We conduct a systematic review of the literature to identify all observational studies that included neonates born with transposition of the great arteries who had peri-operative evidence of brain injury.

The search strategy produced three prospective and two retrospective cohort studies investigating the association between balloon atrial septostomy and brain injury totalling 10,108 patients. In two studies, the outcome was represented by the presence of a coded diagnosis of a clinically evident stroke at discharge, whereas in three studies the outcome was represented by the finding of pre-operative brain injury identified by magnetic resonance scans.

The overall brain injury rate for neonates who underwent balloon atrial septostomy versus control patients was 60 of 2273 (2.6%) versus 45 of 7835 (0.5%; pooled odds ratio, 1.90; 95% confidence intervals, 0.93–3.89; p = 0.08). A subgroup analysis of the three studies that used pre-operative brain injury as the primary outcome found no significant association between balloon atrial septostomy and brain injury (pooled odds ratio, 2.70; 95% confidence intervals, 0.64–11.33; p = 0.17). Balloon atrial septostomy frequency was 22.4% (2273 of 10,108), with reported rates ranging from 20% to 75%.

Our analysis shows that balloon atrial septostomy is not associated with increased odds for peri-operative brain injury. Balloon atrial septostomy should still be used for those patients with significant hypoxaemia, haemodynamic instability, or both.

The benefits of early enteral feeding in neonates are well known and yet the optimal pre-operative nutrition of prostaglandin-dependent infants with congenital cardiac disease remains ill-defined. This survey delineates international nutritional practices and trends with this population.

Paediatric practitioners responded to an Internet-based survey that explored assessment of feeding practices, criteria for feeding readiness, medication dosing, concurrent feeding with umbilical catheters, and the observed incidence of feeding intolerance. Documented nutritional strategies were not correlated with patient outcomes.

A total of 200 caregivers responded to the survey. Fewer United States caregivers (56%) reported routine pre-operative enteral feeding in prostaglandin-dependent infants when compared with caregivers outside the United States of America (93%). Of those respondents willing to feed, approximately two-thirds did not base their decision on the ductal flow direction. Numerous and heterogeneous parameters were reported to assess feeding readiness. Many caregivers report scepticism with regard to enteral feeding safety in neonates with an umbilical artery catheter, and to a lesser extent in the presence of an umbilical venous catheter. In summary, there is a prevailing lack of consensus regarding pre-operative enteral nutrition to prostaglandin-dependent neonates.

This survey demonstrates noticeable variations in pre-operative nutritional practices between providers from around the world. Arguments that support or refute this practice have little support in the medical literature. Future studies should aim to demonstrate the safety of such practice and compare the outcomes of prostaglandin-dependent neonates who were pre-operatively enterally fed with those who were not.

Advances in medical and surgical care have contributed to an important increase in the survival rates of children with congenital heart disease. However, survivors often have decreased exercise capacity and health-related issues that affect their quality of life. Cardiac Rehabilitation Programmes have been extensively studied in adults with acquired heart disease. In contrast, studies of children with congenital heart disease have been few and of limited scope. We therefore undertook a systematic review of the literature on cardiac rehabilitation in children with congenital heart disease to systematically assess the current evidence regarding the use, efficacy, benefits, and risks associated with this therapy and to identify the components of a successful programme.

We included studies that incorporated a cardiac rehabilitation programme with an exercise training component published between January, 1981 and November, 2010 in patients under 18 years of age.

A total of 16 clinical studies were found and were the focus of this review. Heterogeneous methodology and variable quality was observed. Aerobic and resistance training was the core component of most studies. Diverse variables were used to quantify outcomes. No adverse events were reported.

Cardiac Rehabilitation Programmes in the paediatric population are greatly underutilised, and clinical research on this promising form of therapy has been limited. Questions remain regarding the optimal structure and efficacy of the programmes. The complex needs of this unique population also mandate that additional outcome measures, beyond serial cardiopulmonary exercise testing, be identified and studied.

To investigate the effect of pulsatility of venous flow waveform in the inferior and superior caval vessels on the performance of functional and “failing” Fontan patients based on two primary performance measures – the conduit power loss and the distribution of inferior caval flow (hepatic factors) to the lungs.

Doppler angiography flows were acquired from two typical extra-cardiac conduit “failing” Fontan patients, aged 13 and 25 years, with ventricle dysfunction. Using computational fluid dynamics, haemodynamic efficiencies of “failing”, functional, and in vitro-generated mechanically assisted venous flow waveforms were evaluated inside an idealised total cavopulmonary connection with a caval offset. To investigate the effect of venous pulsatility alone, cardiac output was normalised to 3 litres per minute in all cases. To quantify the pulsatile behaviour of venous flows, two new performance indices were suggested.

Variations in the pulsatile content of venous waveforms altered the conduit efficiency notably. High-frequency and low-amplitude oscillations lowered the pulsatile component of the power losses in “failing” Fontan flow waveforms. Owing to the offset geometry, hepatic flow distribution depended strongly on the ratio of time-dependent caval flows and the pulsatility content rather than mixing at the junction. “Failing” Fontan flow waveforms exhibited less balanced hepatic flow distribution to lungs.

The haemodynamic efficiency of single-ventricle circulation depends strongly on the pulsatility of venous flow waveforms. The proposed performance indices can be calculated easily in the clinical setting in efforts to better quantify the energy efficiency of Fontan venous waveforms in pulsatile settings.

Parental suspicions that the child may possibly take narcotics may be substantiated by hair analysis, which may not only identify the narcotic abuse profile, but also define the minimum period of narcotic usage.

A toxicological hair analysis aiming at detecting the presence of amphetamines and cannabinoids in children with cardiac complaints of unclear aetiology, with a view of establishing a diagnosis.

The authors analysed hair samples collected from the occipital region of three adolescent patients for the presence of substances of abuse – amphetamines and cannabinoids. Hair strands were subjected to segmentation and chemical processing and subsequently analysed by liquid chromatography–mass detection method.

Indicating the presence of amphetamines and cannabinoids in hair samples, the toxicological analysis allowed for explaining cardiac complaints and personality changes in adolescent patients, with the said changes being associated with addiction to the above xenobiotics extending over some period.

Despite the increasing utilisation of interventional electrophysiology in adults and older children with arrhythmias, there are few data reflecting the safety and efficacy of this procedure in the age group under 2 years.

We describe our experience in assessing the efficacy and safety with this group of children.

We undertook a retrospective review of all infants under 2 years of age who underwent an interventional electrophysiology procedure between 1995 and 2009 to determine indications, procedural details, short- and long-term success, and complication rate.

A total of 23 interventional electrophysiology procedures were performed in 17 patients initially under 2 years of age. Of these, three patients had congenital heart disease. The most common indication was arrhythmia resistant to pharmacological agents (59%), with the remaining cases being arrhythmia complicated by cardiovascular instability (41%). There was initial success in 15 patients after the first procedure, with early recurrence in four. Following six repeat procedures, there was long-term success in 15 patients (88%), with three repeat procedures being performed after 2 years of age. There was one non-procedural death related to persisting arrhythmia. There were three minor complications. In one patient, cryotherapy was used successfully.

The interventional electrophysiology procedure is a viable therapeutic option in infants under 2 years with arrhythmia resistant to other conventional medical management.

Interventional closure of patent arterial duct has become an accepted alternative to surgical closure. Clinical trial with “Nit-Occlud® PDA-R”.

To assess the safety and efficacy of the device, we performed a prospective clinical study between June, 2009 and December, 2010 in La Paz, Bolivia. In all, 29 – 22 female patients and 7 male patients – out of 59 patients were selected on the basis of inclusion criteria. The procedures were performed under sedation at an age and weight of 5.7 years and 22.7 kilograms, respectively, with 4–6 French arterial sheaths and 5–7 French venous sheaths. The minimal diameter of the duct was 3.5 millimetres. The procedure, fluoroscopy, and hospitalisation times were 96.4 minutes (55 to 145), 13.1 minutes (3 to 25.2), and 24 hours, respectively. The “Nit-Occlud® PDA-R” was successfully deployed in all patients. Immediate, 24-hour, 1-, 3-, and 6-month closure rates were 65.5%, 79.3%, 96.5%, and 100%, respectively. The systolic pulmonary pressure diminished from 37 millimetres of mercury (21 to 57) before the intervention to 31 millimetres of mercury (21 to 45) after the intervention. No early or late embolisation, haemolysis, left pulmonary artery, or descending aorta obstruction occurred.

We conclude that the “Nit-Occlud® PDA-R” device is safe and effective in closing patent arterial duct up to a diameter of 8 millimetres.

To evaluate the growth of children after repair of Tetralogy of Fallot, as well as the influence of residual lesions and socio-economic status.

A total of 17 children, including 10 boys with a median age of 16 months at surgery, were enrolled in a retrospective cohort, in a tertiary care university hospital. Anthropometric (as z-scores), clinical, nutritional, and social data were collected.

Weight-for-age and weight-for-height z-scores decreased pre-operatively and recovered post-operatively in almost all patients, most markedly weight for age. Weight-for-height z-scores improved, but were still lower than birth values in the long term. Long-term height-for-age z-scores were higher than those at birth, surgery, and 3 months post-operatively. Most patients showed catch-up growth for height for age (70%), weight for age (82%), and weight for height (70%). Post-operative residual lesions (76%) influenced weight-for-age z-scores. Despite the fact that most patients (70%) were from low-income families, energy intake was above the estimated requirement for age and gender in all but one patient. There was no influence of socio-economic status on pre- and post-operative growth. Bone age was delayed and long-term-predicted height was within mid-parental height limits in 16 children (93%).

Children submitted to Tetralogy of Fallot repair had pre-operative acute growth restriction and showed post-operative catch-up growth for weight and height. Acute growth restriction could still be present in the long term.

To present the long-term follow-up of children hospitalised for severe rheumatic carditis who were treated with corticosteroids.

This is a retrospective analysis of the outcome of 242 patients with severe rheumatic carditis after discharge from two public hospitals in Niteroi, Brazil. We followed up 118 patients for 4 years or more, with an average of 7.7 years. They were treated with antibiotics to accomplish bacterial eradication and either intravenous methylprednisolone – 40 cases – or oral prednisone – 78 patients – to treat carditis. They were followed up in outpatient clinic.

Cardiac failure was categorised as classes III and IV according to the New York Heart Association classification. In the intravenous corticosteroid group, 21 cases (52.5%) had isolated mitral valve regurgitation, 12 (30%) had mitral plus aortic involvement, and seven (17.5%) had aortic lesion only. In the oral prednisone group, 45 (58%) had mitral valve regurgitation only, 27 (34%) had mitral plus aortic involvement, and six (8%) had aortic lesion only. A total of 28 children were in their first disease attack, of whom 19 (68%) had a rupture of chordae tendineae. A total of 58 patients (49%) sustained recurrence of carditis because of neglected secondary prophylaxis. In all, 19 cases (16%) underwent cardiac surgery – valve replacement or valvuloplasty. In 33% of the cases, the outcome was favourable – asymptomatic at follow-up. The overall mortality rate was 6.8%.

Many critically ill patients who complied with secondary prophylaxis were left with minor injuries, whereas those who neglected it or abandoned it had serious sequelae. The rate of abandonment and loss to follow-up was very high. Many cases (49%) were re-hospitalised because of carditis recurrence.

To evaluate the efficacy of flecainide and digoxin combination in foetal supraventricular tachycardia.

This study was carried out in a tertiary referral centre.

We conducted a retrospective review of 29 patients diagnosed with supraventricular foetal tachycardia between 2001 and 2009. Mode of presentation, foetal cardiac function, maternal anti-arrhythmic serum levels, drug tolerance, and maternal electrocardiogram recordings were assessed. The postnatal outcome of each infant was also evaluated for tachycardia recurrence.

In all, 27 foetuses were treated with digoxin and flecainide combination, and two foetuses were delivered without any treatment. Of the 27 foetuses, seven had atrial flutter and the remaining 22 had atrioventricular re-entry tachycardia. There were eight foetuses with hydrops (27%), of whom three had atrial flutter and five had atrioventricular re-entry tachycardia; 26 foetuses (96%) responded to flecainide and digoxin combination, with restoration of sinus rhythm in 22 (81.4%) and rate control in the other four. In one severely hydropic foetus, there was no response to treatment. In all, 26 treated infants were delivered alive, but one pregnancy was terminated for non-cardiac causes when the foetus was in sinus rhythm. There was no intrauterine death due to tachycardia. Although there were minor side effects to anti-arrhythmic medications, none of the pregnant women developed proarrhythmia.

Flecainide and digoxin combination treatment offers a safe and effective treatment for foetal supraventricular tachycardia with fast restoration of sinus rhythm.

To anglicise an American – that is, English language – disease-specific health-related quality of life measure, using the Paediatric Cardiac Quality of Life Inventory, for children in the age group of 8–12 years and adolescents in the age group of 13–18 years with cardiac disease, and to assess conceptual equivalence of the American and British versions.

A process of forward and backward translation of the measure was undertaken before focus groups and individual interviews with 40 participants – that is, 20 children/adolescents with cardiac disease and 20 parents of children/adolescents with cardiac disease – to determine their understanding of the meaning of the questions.

Interviews established that participants understood the meaning of the questions, although some found it difficult to explain the meaning of questions in which the language was explicit and wanted instead to answer the individual questions as they applied to them/their child. There was agreement that all versions of the questionnaire were relevant and comprehensive, and that the length of the questionnaires was acceptable and practical.

The anglicised version of the Paediatric Cardiac Quality of Life Inventory appears to be a linguistically valid measure of health-related quality of life for children and adolescents with cardiac disease. The psychometric properties of the anglicised Paediatric Cardiac Quality of Life Inventory are now being tested in a multi-centre study in the United Kingdom.