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.

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.

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.

Interatrial septal anomalies, which include atrial septal defect, patent foramen ovale, and atrial septal aneurysm, are common disorders among adult patients. Early detection of interatrial septal anomalies is important in order to prevent haemodynamic consequences and/or thromboembolic events. Electrocardiogram offers some clues that should serve as hints for detection of interatrial abnormalities. The aim of our study was to analyse the interatrial septum by transoesophageal echocardiography in patients with electrocardiogram signs of right bundle branch block and in those without right bundle branch block.

In a prospective study, 87 adult patients were included, that is, 41 with electrocardiogram signs of right bundle branch block forming the first group and 46 without right bundle branch block forming the second group. Interatrial septal anomalies were present in 80.5% of the patients with right bundle branch block, with patent foramen ovale (39.02%) being the most prevalent disorder, followed by atrial septal aneurysm (21.9%) and atrial septal defect (19.5%). Interatrial septal abnormalities were significantly more frequent in the first group compared with the second group (80.5% versus 6.5%, p value less than 0.001). Independently, patent foramen ovale was significantly more prevalent in patients with right bundle branch block (39.02% versus 4.3%, p value less than 0.001), as were atrial septal aneurysm (21.9% versus 2.2%, p value equal 0.01) and atrial septal defect (19.5% versus 0%, p value equal 0.004).

Right bundle branch block should serve as a valuable indicator to motivate a detailed search for interatrial septal abnormalities.

The objective of this study was to compare three patient-based New York Heart Association assessments with cardiologist assessments in outpatients with congenital cardiac disease.

Consecutive adult outpatients completed three questionnaires in a random order: a patient-based translation of the New York Heart Association classes, a self-constructed questionnaire based on the New York Heart Association classes, and the Specific Activity Scale. The treating cardiologist assessed the New York Heart Association class on the same day. Patient–cardiologist agreement was assessed by calculating percent agreement and weighted kappa. We also explored the level of agreement for patients without co-morbidity.

In all, 86 adults – with a median age of 35.8 years – including 46 women participated. An agreement of 75.6% (weighted kappa is 0.43; probability is smaller than 0.01), 70.6% (weighted kappa is 0.44; probability is smaller than 0.01), and 74.4% (weighted kappa is 0.28; probability is smaller than 0.01) was found between the cardiologist assessment and the patient-based translation, self-constructed questionnaire, and the Specific Activity Scale, respectively. The patient-based translation equally over- and underestimated the New York Heart Association class, whereas the self-constructed questionnaire overestimated and the Specific Activity Scale underestimated the New York Heart Association class. Agreement levels for patients without co-morbidity were higher than agreement levels for the total group.

The patient-based translation yielded adequate agreement with cardiologist-assessed New York Heart Association class, showed equal over- and underestimation, and was easy to complete. The patient-based translation with the instruction to only consider functional impairments caused by the congenital cardiac defect is recommended in future studies of outpatients with congenital cardiac disease.

To objectively evaluate and describe physical activity levels in children with a stable congenital heart defect and compare those levels with children who do not have a congenital heart defect.

We matched 21 pairs of children for gender and grade in school and gave them an accelerometer-based motion sensor to wear for 7 consecutive days.

Physical activity levels did not differ between children with and without a congenital heart defect. During the 7 days of monitoring, children in this study spent most of their time in sedentary behaviours, that is, 6.7 hours of the 13 monitored hours, 54 minutes in moderate-intensity physical activity, and 12 minutes in vigorous-intensity physical activity. Less than one-fifth of all participants, with or without a congenital heart defect, accumulated sufficient physical activity to meet current physical activity recommendations for children and adolescents.

Children with a stable congenital heart defect have activity behaviours that are similar to children without a congenital heart defect. Habitual physical activity in children with a congenital heart defect should be encouraged early on in life to develop strong physical activity habits that will hopefully follow them across their lifespan.

Achievement of adequate oral nutrition is a challenging task after early neonatal cardiac surgery. This study aims to describe predictors of oral feeding outcomes for neonates after early surgical interventions.

A retrospective review of neonates admitted with congenital cardiac disease over a period of 1 year. We analysed predictors of the need for a feeding tube at discharge and the amount taken at each feeding. Multilevel modelling was used to look at individual change over time predicting oral amount at each feeding.

We identified 56 neonates. Diagnoses were heterogeneous; 23% of the infants had associated genetic syndromes and 45% required pre-operative mechanical ventilation. The median time from birth to surgery was 8.4 days, with 29 infants fed orally before surgery. The mean time from surgery to first oral feeding attempt was 12 hours. Time from surgery to oral feeding, the amount taken with first feeding, and cross-clamp times were significant predictors of oral feeding success, whereas the presence of a comorbidity – genetic abnormality – and longer ventilator dependency predicted failure. Almost half of the neonates required a feeding tube upon discharge, and no infant discharged was solely breastfed. Discharge with a feeding tube was associated with greater weight gain at that time.

Neonates with congenital cardiac disease face significant barriers to successfully achieving oral feeding on hospital discharge. Enteral feeding guidelines focus on physiological stabilisation and do not always address the developmental milestones necessary to support oral feeding. Future prospective studies are necessary to identify multimodal strategies to optimise early feeding.

Hyperglycaemia has been identified as a risk factor for adverse outcomes in critically ill patients, including those who have undergone cardiopulmonary bypass. Tight glucose control with insulin therapy has been shown to improve outcomes, but is not common practice for children following cardiopulmonary bypass. We examined the relationship between blood glucose level and systemic and cerebral oxygen transport in a uniform group of neonates after the Norwood procedure.

Systemic oxygen consumption was measured using respiratory mass spectrometry in 17 neonates for 72 hours postoperatively. Cardiac output, systemic and total pulmonary vascular resistances – including the Blalock–Taussig shunt, systemic oxygen delivery and oxygen extraction ratio, as well as arterial lactate and glucose, were measured at 2- to 4-hour intervals. Cerebral oxygen saturation was measured by near-infrared spectroscopy.

Blood glucose levels ranged from 2.8 to 24.6 millimoles per litre. Elevated glucose level showed a significant negative correlation with cardiac output (p = 0.02) and cerebral oxygen saturation (p = 0.03), and a positive correlation with oxygen extraction ratio (p = 0.03). It tended to correlate positively with systemic vascular resistance (p = 0.09) and negatively with oxygen delivery (p = 0.09), but did not correlate with oxygen consumption (p = 0.13).

Hyperglycaemia is negatively associated with systemic haemodynamics, oxygen transport, and cerebral oxygenation status in neonates after the Norwood procedure. Further study is warranted to examine tight glucose control with insulin therapy on postoperative systemic and cerebral oxygen transport and functional outcomes in neonates after cardiopulmonary bypass.

Our animal model suggests that quantification of ductal flow from colour Doppler pixels is possible. We aimed to clarify whether this method can be used to determine a clinically significant ductal shunt in children.

We retrospectively quantified ductal flow from saved images from 20 children who had been admitted for device occlusion of patent ductus arteriosus. Colour Doppler images over the main stem of the pulmonary artery were obtained in longitudinal cross-sections. The colour pixel percentages during diastole, representing ductal flow, were correlated with the documented shunt, measured invasively according to Fick's principle.

The ratio of pulmonary to systemic flow correlated best with the sum of the percentages of green colour pixels (r = 0.73, r2 = 0.54, p < 0.001). When the shunt was 1.5:1 or more, 12 out of 13 infants had 50% or more of the region of interest covered with green pixels – sensitivity 92%, specificity 71%. The correlation between ductal diameter and pulmonary-to-systemic flow ratio was less significant (r = 0.6, r2 = 0.37, p < 0.03).

We conclude that clinically significant shunts with pulmonary-to-systemic flow ratio over 1.5 can be diagnosed with this method where neither the size of the patient nor echocardiographic settings seem to be critical. The method could be used to provide an objective indication for ductal closure, but further prospective studies in children are needed to verify the power of the method.

There are concerns over the outcome of device closure of secundum atrial septal defect with special reference to erosions and aortic regurgitation.

To assess the medium-term outcome of device closure of atrial septal defects with special reference to complications.

A total of 205 patients with secundum atrial septal defects underwent transcatheter closure from October, 1999 to April, 2009. The median age was 18 (1.4–55) years. Amplatzer Septal Occluder was used in all the patients. Medium-term follow-up was available in 176 of 200 (88%) patients.

Device closure was successful in 200 out of 205 (98%) patients. The device embolised in four patients and was associated with short inferior caval vein margin (p = 0.003). Balloon sizing in 71 patients (35%) resulted in implantation of a larger device (p = 0.002). Early complications included pericardial effusion, 2:1 heart block, and infective endocarditis (1 patient each). There were eight patients who reported migraine (3.9%). At median follow-up of 5.8 (0.6–10.3) years, complete closure occurred in 197 out of 200 patients. Short superior caval vein margin was associated with a residual shunt (p < 0.001). There were two patients who developed mild aortic regurgitation (1%), which correlated with a device-to-defect ratio of >1.3:1 (p = 0.001). There were no erosions, late embolisation, or thromboembolism. Atrial fibrillation occurred in three adults (1.5%).

Device closure of secundum atrial septal defects using Amplatzer Septal Occluder is safe and effective in the medium term. Short inferior caval vein margin correlates with increased risk of embolisation and short superior caval vein margin with a residual shunt. The risk of developing aortic regurgitation is low and correlates with increased device-to-defect ratio.

Cardiovascular complications and vascular changes are common in patients with beta-thalassaemia major. The aim of this study is to investigate the common carotid artery intima-media thickness in children.

The study population was consisted of 33 thalassaemic children (22 boys and 11 girls, with a median age of 8 years) and 30 healthy children for control (12 girls and 18 boys, with a median age of 8 years) who were matched for age and gender. Common carotid artery intima-media thickness of the children was measured.

The patients’ age at diagnosis ranged from 0.25 to 2 years, with a median of 0.6 months. Their disease duration ranged from 4 to 13.75 years, with a median of 6.5 years. The patients’ median common carotid artery intima-media thickness was significantly higher than controls – that is, 0.87 versus 0.74, with a p-value less than 0.005. The mean common carotid artery intima-media thickness value was positively correlated with disease duration (r = 0.535), with a p-value less than 0.01 and ferritin level (r = 0.501), with a p-value less than 0.01.

Owing to the nature of the disease, patients with beta-thalassaemia major should be considered to have an increased risk of early vascular alteration and atherosclerosis. For this reason, common carotid artery intima-media thickness measurement can be recommended as a non-invasive and early diagnostic method.