We sought to systematically study determinants of “clinical status at arrival after transport” of neonates with CHD and its impact on clinical outcomes in a low- and middle-income country environment.

Consecutive neonates with CHD (n = 138) transported (median distance 138 km; 5–425 km) to a paediatric cardiac programme in Southern India were studied prospectively. Among 138 neonatal transports, 134 were in ambulances. Four neonates were transported by family in private vehicles; 60% with duct-dependent circulation (n = 57) were transported without prostaglandin E1. Clinical status at arrival after transport was assessed using California modification of TRIPS Score (Ca-TRIPS), evidence of end-organ injury and metabolic insult.

Upon arrival, 42% had end-organ injury, 24% had metabolic insult and 36% had Ca-TRIPS Score >25. Prior to surgery or catheter intervention, prolonged ICU stay (>48 hours), prolonged ventilation (>48 hours), blood stream sepsis, and death occurred in 48, 15, 19, and 3.6%, respectively. Ca-TRIPS Score >25 was significantly associated with mortality (p = 0.005), sepsis (p = 0.035), and prolonged ventilation (p < 0.001); end-organ injury with prolonged ICU stay (p = 0.031) and ventilation (p = 0.045); metabolic insult with mortality (p = 0.012) and sepsis (p = 0.015).

Fifteen babies needed only medical management, 10 received comfort care (due to severe end-organ injury in 3), 107 underwent cardiac surgery (n = 83) or catheter intervention (n = 24), with a mortality of 6.5%. Clinical status at arrival after transport did not impact post-procedure outcomes.

Neonates with CHD often arrive in suboptimal status after transport in low- and middle-income countries resulting in adverse clinical outcomes. Robust transport systems need to be integrated in plans to develop newborn heart surgery in low- and middle-income countries.

Patients with Turner’s syndrome are at risk of aortic dilation and dissection. Currently, it is not known whether such dilation is related to associated cardiovascular abnormalities, or to the genetic anomaly itself.

We studied echocardiographically 107 patients with genetically proven Turner’s syndrome, with heterogeneous underlying karyotypes, and without associated cardiac lesions. Their average age was 19.6 plus or minus 8.4 years. We compared the finding with those from 71 age-matched healthy female volunteers. The diameter of the aorta was measured at the level of the basal attachments of the aortic valvar leaflets, the sinuses of Valsalva, the sinutubular junction, and its ascending component.

Compared to control subjects, the patients with Turner’s syndrome had larger diameters of the aorta at the level of the sinuses of Valsalva, at 23.4+/−4.8 versus 25.5+/−4.1 millimetres (p = 0.0014), the sinutubular junction, at 19.9+/−3.8 versus 23.3+/−4.1 millimetres (p < 0.0001), and the ascending aorta, at 22.3+/−4.9 versus 24.6+/−4.4millimetres (p = 0.0011). Dilation of the sinutubular junction, found in just over one-quarter of the patients, was more common than dilation of the ascending aorta, the latter found in less than one-tenth. The patients with Turner’s syndrome, therefore, presented with remodelling of the aortic root, with relative dilation of the sinutubular junction. The underlying karyotype influenced both the dimensions of the sinutubular junction (p = 0.0054), and the ascending aorta (p = 0.0064), so that patients with the karyotype 45X had larger aortas. The karyotype was the strongest predictor by multivariate analysis for dilation at both these sites (p = 0.0138 and 0.0085, respectively).

Dilation at the sinutubular junction is frequent in patients with Turner’s syndrome, and is more common than dilation of the ascending aorta. The syndrome is associated with a remodelling of the aortic root, with prominent dilation of the sinutubular junction. There seems to be a relation between aortic dilation and the underlying genotype.

Neonates and infants with congenital and acquired cardiac disease often require placement of central venous lines for extended intravenous therapy. It may be advantageous to avoid the larger venous vessels of the head and neck and lower extremities in order to preserve these for future interventions and therapies. We evaluated the results of a nursing led peripherally inserted central catheter team in our congenital cardiac centre.

Bedside peripherally inserted central catheter the insertion procedures were evaluated for success, complications, and completion of therapy.

A total of 125 peripherally inserted central catheters were successfully placed in 105 patients. The mean age at the time of placement was 13.5 plus or minus 19.1 days; median age was 7 days; mean weight was 3.5 plus or minus 1.1 kilogram. Cyanotic cardiac disease accounted for 76% of the diagnoses. Central placement of these lines was successful in 78% of patients. Complications during insertions were limited to inadvertent arterial access in five (3%) infants and oxygen desaturations during sedation for the procedure in two (1%) patients. None of the infants suffered long-term compromise from arterial access; none required intubation for the desaturations. The team was able to respond to 90% of their requests within 24 hours. Median catheter dwell time was 14 days.

In a population of infants with cardiac disease, a nursing staffed peripherally inserted central catheter team achieved an insertion rate of 78% with few complications and a rapid response time. Reliance on bedside insertion permits continuous critical care monitoring and may eliminate the need for fluoroscopy.

To investigate QT dispersion in the surface electrocardiogram of children with rheumatic carditis.

QT dispersion was quantitatively evaluated in 33 children with acute rheumatic carditis. As a control group, we studied 33 healthy children free of any disease. The children were eligible for participation if the following criteria were met: diagnosis of acute rheumatic fever based on the revised Jone’s criteria and suffering from their first attack of carditis. The echo Doppler cardiogram was performed in all children, within 48–72 hours of hospitalisation.

Patients with carditis had a greater QT dispersion than the control group. When we analyse the QT dispersion according to the severity of the carditis, we observed that the dispersion tended to be greater in those with more severe valvar lesion. The sensitivity and specificity of the measurements of the QT dispersion in predicting acute carditis were estimated by using receiver operating characteristic curves. A QT dispersion greater than 40 milliseconds had a sensitivity of 63.6% and a specificity of 93.9% in predicting acute rheumatic carditis.

The lengthening of QT dispersion may reflect on cardiac involvement in rheumatic fever and be a new important parameter in the diagnosis and therapeutic decision for rheumatic carditis.

We aimed to compare the procedural and mid-term performance of a specifically designed self-expanding stent with balloon-expandable stents in patients undergoing hybrid palliation for hypoplastic left heart syndrome and its variants.

The lack of specifically designed stents has led to off-label use of coronary, biliary, or peripheral stents in the neonatal ductus arteriosus. Recently, a self-expanding stent, specifically designed for use in hypoplastic left heart syndrome, has become available.

We carried out a retrospective cohort comparison of 69 neonates who underwent hybrid ductal stenting with balloon-expandable and self-expanding stents from December, 2005 to July, 2014.

In total, 43 balloon-expandable stents were implanted in 41 neonates and more recently 47 self-expanding stents in 28 neonates. In the balloon-expandable stents group, stent-related complications occurred in nine patients (22%), compared with one patient in the self-expanding stent group (4%). During follow-up, percutaneous re-intervention related to the ductal stent was performed in five patients (17%) in the balloon-expandable stent group and seven patients (28%) in self-expanding stents group.

Hybrid ductal stenting with self-expanding stents produced favourable results when compared with the results obtained with balloon-expandable stents. Immediate additional interventions and follow-up re-interventions were similar in both groups with complications more common in those with balloon-expandable stents.

Children with congenital heart disease (CHD) have complex unique post-operative care needs. Limited data assess parents’ hospital discharge preparedness and education quality following cardiac surgery. The goals were to identify knowledge gaps in discharge preparedness after congenital heart surgery and to assess the acceptability of an educational mobile application to improve discharge preparedness.

Telephonic interviews with parents of children with two-ventricle physiology who underwent cardiac surgery 5–7 days post-discharge and in-person interviews with clinicians were conducted. We collected parent and clinician demographics, parent health literacy information and patient clinical data. We analysed interview transcripts using summative content analysis.

We interviewed 26 parents and 6 clinicians. Twenty-two of the 26 (85%) parents felt ready for discharge; 4 of the 6 (67%) clinicians did not feel most parents were ready for discharge. Fifteen of the 26 parents (58%) reported receiving the majority of discharge teaching on the day of discharge. Eight parents did not feel like all of their questions were answered. Most parents (14/26, 54%) preferred visual educational learning aids and could accurately describe important aspects of care. Most parents (23/26, 88%) and all 6 clinicians felt a mobile application for post-operative care education would be helpful.

Most parents received education on the day of discharge and could describe the information they received prior to discharge, although there were some preparedness gaps identified after discharge. Clinicians and parents varied in their perceptions of the readiness for discharge. Most responses suggest that a mobile application for discharge education may be helpful for transition to home.

Cardiac rehabilitation programmes for paediatric patients with congenital heart disease (CHD) have been shown to promote emotional and physical health without any associated adverse events. While prior studies have demonstrated the effectiveness of these types of interventions, there has been limited research into how the inclusion of psychological interventions as part of the programme impacts parent-reported and patient-reported quality of life.

Patients between the ages of 7 and 24 years with CHD completed a cardiac rehabilitation programme that followed a flexible structure of four in person-visits with various multidisciplinary team members, including paediatric psychologists. Changes in scores from the earliest to the latest session were assessed regarding exercise capacity, patient functioning (social, emotional, school, psychosocial), patient general and cardiac-related quality of life, patient self-concept, and patient behavioural/emotional problems.

From their baseline to final session, patients exhibited significant improvement in exercise capacity (p = 0.00009). Parents reported improvement in the patient’s emotional functioning, social functioning, school functioning, psychosocial functioning, cognitive functioning, communication, and overall quality of life. While patients did not report improvement in these above areas, they did report perceived improvement in certain aspects of cardiac-related quality of life and self-concept.

This paediatric cardiac rehabilitation programme, which included regular consultations with paediatric psychologists, was associated with divergent perceptions by parents and patients on improvement related to quality of life and other aspects of functioning despite improvement in exercise capacity. Further investigation is recommended to identify underlying factors associated with the differing perceptions of parents and patients.

To describe the motor proficiency of 5-year-old children who underwent early infant cardiac surgery and had atypical infant gross motor development. To identify risk factors for motor dysfunction at 5 years of age.

A total of 33 children (80.5% participation rate) were re-assessed by a physiotherapist blinded to the diagnosis and previous clinical course, using standardised motor assessment tools.

Motor proficiency was categorised as below average or well below average in 41% of the study patients. Approximately 30% of the cohort had balance deficits. Motor abilities at 4 months and 2 years of age were associated with motor proficiency at age 5; however, atypical motor development in infancy was not predictive of below-average or well below-average scores at age 5. Risk factors associated with motor ability at age 5 included respiratory support and intensive care length of stay in the 1st year of life, asymmetrical crawling in infancy, and cyanotic CHD at age 5.

Despite differences from other reported studies in terms of cohort diagnoses and age at surgery, the rate of motor dysfunction was similar, with rates much higher than expected in typical children. Further assessment is needed in later childhood to determine the significance of these findings.

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.

Electrical injury can result in a variety of cardiac abnormalities. We evaluate the cardiac effects in patients injured by electric shock and treated in our medical centre.

We reviewed retrospectively the findings in 52 children, aged from 7 months to 17 years, with a mean age of 10.1 ± 5.1 years, all evaluated and treated for accidental electric shock from January, 1992, through July, 2004. Relevant data regarding clinical presentation, electrocardiogram recording and cardiac enzymes was compiled. We also evaluated the echocardiographic findings, clinical course, treatment, and outcome.

Syncope had been the presenting symptom in 17 children (33%), asystole in 1 patient, and ventricular fibrillation or tachycardia in 2 patients. Characteristic changes of acute ischaemia of the anterior wall on the basis of changes in the ST segments were noted in 2 patients. Total creatine phosphokinase was measured in 33 children (63%), and was elevated in 20. Creatine phosphokinase-MB was measured in 11 patients, and was abnormal in six (54%). Troponin was measured in three children, and was significantly high in one (33%). Cardiopulmonary resuscitation and mechanical ventilation for a significant period was necessary in 5 patients, of whom 4 (80%) survived. None of the survivors was left with any cardiac disability following the acute event.

Significant cardiac damage and complications are rare in children and young adults who survive incidental electrocution. Most of the cardiac events are observed during the acute phase and immediately subsequent to electrocution. No delayed complications are anticipated.