Balloon atrial septostomy is a common palliative procedure in neonates with cyanotic congenital heart disease with restricted interatrial blood flow. Despite its advantages, balloon atrial septostomy is not a risk-free procedure and can be associated with numerous complications. The objective of this study is to determine whether the performance of this procedure out-of-hours has a significant impact on the incidence of adverse outcome measures.

A total of 106 neonates who underwent balloon atrial septostomy between 2004 and 2010 were studied retrospectively. In all, 64 infants had the procedure performed within routine hours (9 am to 6 pm), whereas 42 neonates underwent the procedure out-of-hours (6:01 pm to 8:59 am). Procedure-related complications occurred in 32 infants (30.2%), which included 12 out of 64 (18.8%) infants in the routine-hours group and 20 out of 42 (47.6%) in the out-of-hours group. During further follow-up after surgery and including both major and minor adverse events, seven more infants (10.9%) suffered complications after balloon atrial septostomy in the routine-hours group and four more infants (9.5%) suffered complications in the out-of-hours group. This totalled the complication rate in the routine-hours group to 19 infants (29.7%) and 24 infants (57.1%) in the out-of-hours group (p = 0.001). A higher overall mortality rate was also noted in the out-of-hours group.

Balloon atrial septostomy performed out-of-hours produced higher complication rates as opposed to balloon atrial septostomy performed during routine hours. Only essential cases should be undertaken at night, and all other cases should be deferred to the daytime to limit unnecessary adverse complication.

Surgical ligation of patent ductus arteriosus is considered when medical treatment fails or is contraindicated. This study aims to determine the mortality and morbidity of preterm neonates referred for patent ductus arteriosus ligation.

A prospective study was conducted in the East of England to follow the outcome of premature infants under 37 weeks’ gestation undergoing patent ductus arteriosus ligation. A standardised proforma was used to collect information before and after the procedure.

A total of 102 premature infants were recruited, and patent ductus arteriosus ligation was performed in 92. Surgical complications occurred in 8.7% (8/92), which included pneumothorax (5/8), recurrent laryngeal nerve palsy (2/8), and chylothorax (1/8). Morbidity outcome data were not available for all infants. The incidence of chronic lung disease was 88% (88/99); intraventricular haemorrhage was 49% (49/100); necrotising enterocolitis 39% (39/99), and retinopathy of prematurity 42% (41/97). The overall mortality rate in our study was 7.8% (8/102). Mortality rate in infants who had patent ductus arteriosus ligation was 4.3% (4/92). The 30-day survival rate after ligation was 99% (91/92). Beyond 30 days post-ligation, three infants died from other causes that were not directly related to surgery.

Patent ductus arteriosus ligation in premature infants is associated with low mortality and complication rates; however, there is a high incidence of neonatal morbidity. Surgical capacity for patent ductus arteriosus ligation needs to be carefully planned nationally as the duration of “waiting time” and transport to another surgical centre could adversely affect outcomes in this high-risk population.

Patients with a congenitally corrected transposition of the great arteries show an increasing incidence of cardiac failure with age. In other systemic right ventricles, such as in dextro-transposition after atrial switch, excessive hypertrophy is a potential risk factor for impaired systolic function. In this trial, we sought to compare systemic function and volumes between patients with congenitally corrected transposition and those with dextro-transposition after atrial switch by using cardiac magnetic resonance imaging.

A total of 19 patients (nine male) with congenitally corrected transposition and 31 patients (21 male) with dextro-transposition after atrial switch were studied using a 1.5-Tesla scanner. Cine steady-state free-precession sequences in standard orientations were acquired for volumetric and functional imaging. Patient parameters were compared with those of a group of 25 healthy volunteers. Although patients with congenitally corrected transposition were older, they presented with higher right ventricular ejection fractions (p = 0.04) compared with patients with dextro-transposition. Patients with congenitally corrected transposition showed a weak negative correlation between age at examination and systemic ejection fraction (r = −0.18, p = 0.04) but no correlation between right ventricular myocardial mass index and ejection fraction. There was no significant difference in the right ventricular end-diastolic volumes between both patient groups.

Although patients with congenitally corrected transposition had a longer pressure load of the systemic right ventricle, ventricular function was better compared with that in patients with dextro-transposition after atrial switch. The results suggest that the systemic ventricles might have partly different physiologies. One difference could be the post-operative situation after atrial switch, which results in impaired atrial contribution to ventricular filling.

Necrotising enterocolitis is a rare, though catastrophic complication that may occur in term newborns with congenital heart disease. There is considerable controversy regarding the factors that lead to necrotising enterocolitis in this population. We sought to determine the incidence of necrotising enterocolitis among term and near-term newborns with congenital heart disease, focusing on the relationship of enteral feeding to this complication.

In this retrospective study, we identified the incidence of necrotising enterocolitis among 1551 newborns admitted to our cardiac intensive care unit between July 1, 2002 and July 1, 2010. In order to understand the impact of enteral feeding upon the development of necrotising enterocolitis, we undertook a nested 2:1 matched case–control analysis to compare feeding patterns in an age- and lesion-matched control population.

Necrotising enterocolitis developed in 45 term or near-term infants (3%). The majority of these cases, 27 (60%), occurred in the post-operative period after the introduction of enteral feeds. This subgroup was used for matched analysis. There were no differences in enteral feeding patterns among the patients who developed necrotising enterocolitis and their matched controls. The overall mortality rate for patients who developed necrotising enterocolitis was 24.4% (11 out of 45).

Despite numerous advances in the care of infants with congenital heart disease, necrotising enterocolitis remains a significant source of morbidity and mortality. In these infants, there is no clear relationship between enteral feeding patterns and the development of necrotising enterocolitis in the post-operative period. The benefits of graduated feeding advancements to avoid the development of necrotising enterocolitis remain unproven.

Delay and impairment of motor development is reported in patients with congenital heart disease. This pilot study addressed the feasibility and effect of a low-dose motor training programme of 60 min once per week on motor ability in preschool children with congenital heart disease.

In all, 14 children – including four girls, in the age group of 4–6 years – with various types of congenital heart disease performed the motor developmental test MOT 4–6 before and after 3 months of a playful exercise programme of 60 min once a week.

At baseline, the motor quotient ranged from normal to slightly impaired (median 92.0; Quartile 1: 83.75; Quartile 3: 101.25). After intervention, motor quotient did not change significantly for the entire group (95.0 (88.0, 102.5); p = 0.141). However, in the subgroup of nine children with retarded motor development at baseline (motor quotient lower 100), seven children had an improved motor quotient after 3 months of intervention. In this subgroup, motor quotient increased significantly (p = 0.020) by 5%.

Overall, a short intervention programme of 60 min only once a week does not improve motor ability in all children with congenital heart disease. However, those with retarded motor development profit significantly from this low-dose intervention.

Shunt occlusion is a major cause of death in children with single ventricle. We evaluated whether one daily measurement of oxygen saturation at home could detect life-threatening shunt dysfunction.

A total of 28 infants were included. Parents were instructed to measure saturation once daily and if less than or equal to 70% repeat the measurement. Home monitoring was defined as positive when a patient was admitted to Queen Silvia Children's Hospital because of saturation less than or equal to 70% on repeated measurement at home. A shunt complication was defined as arterial desaturation and a narrowing of the shunt that resulted in an intervention to relieve the obstruction or in death. Parents’ attitude towards the method was investigated using a questionnaire.

A shunt complication occurred out of hospital eight times in eight patients. Home monitoring was positive in five out of eight patients. In two patients, home monitoring was probably life saving; in one of them, the shunt was replaced the same day and the other had an emergency balloon dilatation of the shunt. In three out of eight patients, home monitoring was negative; one had an earlier stage II and survived, but two died suddenly at home from thrombotic shunt occlusion. On seven occasions in three patients home monitoring was positive but there was no shunt complication. The method was well accepted by the parents according to the results of the questionnaire.

Home monitoring of oxygen saturation has the potential to detect some of the life-threatening shunt obstructions between stages I and II in infants with single-ventricle physiology.

Identify trends of enrolment and key challenges when recruiting infants with complex cardiac diseases into a multi-centre, randomised, placebo-controlled drug trial and assess the impact of efforts to share successful strategies on enrolment of subjects.

Rates of screening, eligibility, consent, and randomisation were determined for three consecutive periods of time. Sites collectively addressed barriers to recruitment and shared successful strategies resulting in the Inventory of Best Recruiting Practices. Study teams detailed institutional practices of recruitment in post-trial surveys that were compared with strategies of enrolment initially proposed in the Inventory.

The number of screened patients increased by 30% between the Initial Period and the Intermediate Period (p = 0.007), whereas eligibility decreased slightly by 7%. Of those eligible for entry into the study, the rate of consent increased by 42% (p = 0.025) and randomisation increased by 71% (p = 0.10). During the Final Period, after launch of a competing trial, fewer patients were screened (−14%, p = 0.06), consented (−19%, p = 0.12), and randomised (−34%, p = 0.012). Practices of recruitment in the post-trial survey closely mirrored those in the Inventory.

Early identification and sharing of best strategies of recruitment among all recruiting sites can be effective in increasing recruitment of critically ill infants with congenital cardiac disease and possibly other populations. Strategies of recruitment should focus on those that build relationships with families and create partnerships with the medical providers who care for them. Competing studies pose challenges for enrolment in trials, but fostering trusting relationships with families can result in successful enrolment into multiple studies.

To describe the epidemiology of chromosomal and non-chromosomal cases of atrioventricular septal defects in Europe.

Data were obtained from EUROCAT, a European network of population-based registries collecting data on congenital anomalies. Data from 13 registries for the period 2000–2008 were included.

There was a total of 993 cases of atrioventricular septal defects, with a total prevalence of 5.3 per 10,000 births (95% confidence interval 4.1 to 6.5). Of the total cases, 250 were isolated cardiac lesions, 583 were chromosomal cases, 79 had multiple anomalies, 58 had heterotaxia sequence, and 23 had a monogenic syndrome. The total prevalence of chromosomal cases was 3.1 per 10,000 (95% confidence interval 1.9 to 4.3), with a large variation between registers. Of the 993 cases, 639 cases were live births, 45 were stillbirths, and 309 were terminations of pregnancy owing to foetal anomaly. Among the groups, additional associated cardiac anomalies were most frequent in heterotaxia cases (38%) and least frequent in chromosomal cases (8%). Coarctation of the aorta was the most common associated cardiac defect. The 1-week survival rate for live births was 94%.

Of all cases, three-quarters were associated with other anomalies, both chromosomal and non-chromosomal. For infants with atrioventricular septal defects and no chromosomal anomalies, cardiac defects were often more complex compared with infants with atrioventricular septal defects and a chromosomal anomaly. Clinical outcomes for atrioventricular septal defects varied between regions. The proportion of termination of pregnancy for foetal anomaly was higher for cases with multiple anomalies, chromosomal anomalies, and heterotaxia sequence.

Recent evidence in adults suggests that left ventricular mass measured as left ventricular mass/height1.7 predicts cardiovascular morbidity and mortality better than the two widely used indices, left ventricular mass/body surface area and left ventricular mass/height2.7. Standards of left ventricular mass/height1.7 have not been reported in children, for whom, owing to lack of significant cardiovascular morbidity and mortality, body mass index has traditionally been used as a potential cardiovascular risk factor.

In this retrospective study, 692 clinically normal children aged 1 day to 18 years underwent detailed echocardiographic assessment to assess whether any of the left ventricular mass indices – left ventricular mass/height1.7, left ventricular mass/body surface area, and left ventricular mass/height2.7 – are associated with obesity as measured by body mass index. Correlations, t-tests, and linear regressions were used for statistical testing.

Left ventricular mass/height1.7 was better correlated (R2 = 0.36) with body mass index than left ventricular mass/body surface area (R2 = 0.179) and left ventricular mass/height2.7 (R2 = 0.006), although all three dependent variables show a significant correlation (p < 0.035). In addition, a higher percentage of obese patients were noted to have elevated left ventricular mass as measured by left ventricular mass/height1.7 than by the other two methods.

Left ventricular mass/height1.7 is a reliable indicator of obesity-associated left ventricular hypertrophy. Left ventricular mass/height1.7 can be used conveniently during transitions from youth to adults for long-term follow-up. These findings support the importance of including left ventricular mass/height1.7 in future studies of cardiovascular risks and preventive strategies in children and adolescents.

Providing care for children with cardiac disease remains a challenge in developing countries.

Since 2001, a team of paediatric cardiologists has been visiting Mongolia to perform children's cardiac catheterisation, as well as screening children for cardiac disease. Considering the limited medical resources, as well as cost, risk, and benefit, we focused our activities on diagnostic consultation by echocardiography, transcatheter closure of persistent arterial duct, balloon dilation of valvar pulmonary stenosis and aortic coarctation, and diagnostic catheterisation.

Up to 2011, we have completed echocardiography in 1200 patients; diagnostic catheterisations in 59 patients; and catheter interventions in 255 patients, including 224 for persistent arterial duct, 23 for valvar pulmonary stenosis, 6 for aortic coarctation, and a few others. We have visited 14 rural areas to screen for children's cardiac diseases. A total of 131 persistent ducts were closed with coils and a further 93 with a duct occluder. Migration of the coil or occluder to the pulmonary artery occurred once for each device. Pulmonary valvuloplasty was successful in 23 patients and a coarctation was effectively dilated in six patients.

The limited healthcare resources in developing countries such as Mongolia make catheter interventional procedures an attractive alternative to surgery in treating children with simple but critical congenital heart diseases. Introduction of the duct occluder extended the application of transcatheter occlusion of persistent arterial duct in Mongolia to larger vessels and avoided the expense of coil occlusion using multiple coils.

Although survival to hospital discharge among children requiring extracorporeal membrane oxygenation support for medical and surgical cardio-circulatory failure has been reported in international registries, extended survival and re-hospitalisation rates have not been well described in the literature.

This is a single-institution, retrospective review of all paediatric patients receiving extracorporeal membrane oxygenation for primary cardiac dysfunction over a 5-year period.

A total of 74 extracorporeal membrane oxygenation runs in 68 patients were identified, with a median follow-up of 5.4 years from hospital discharge. Overall, 66% of patients were decannulated alive and 25 patients (37%) survived to discharge. There were three late deaths at 5 months, 20 months, and 6.8 years from discharge. Of the hospital survivors, 88% required re-hospitalisation, with 63% of re-admissions for cardiac indications. The median number of hospitalisations per patient per year was 0.62, with the first re-admission occurring at a mean time of 9 months after discharge from the index hospitalisation. In all, 38% of patients required further cardiac surgery.

Extended survival rates for paediatric hospital survivors of cardiac extracorporeal membrane oxygenation support for medical and post-surgical indications are encouraging. However, re-hospitalisation within the first year following hospital discharge is common, and many patients require further cardiac surgery. Although re-admission hospital mortality is low, longer-term follow-up of quality-of-life indicators is required.