Cardiopulmonary bypass during tetralogy of Fallot corrective surgery is associated with oxidative stress, and contributes to peri-operative problems. Curcumin has been known as a potent scavenger of reactive oxygen species, which enhances the activity of antioxidants and suppresses phosphorylation of transcription factors involved in inflamation and apoptosis.

To evaluate the effects of curcumin as an antioxidant by evaluating the concentrations of malondialdehyde and glutathione, activity of nuclear factor-kappa B, c-Jun N-terminal kinase, caspase-3, and post-operative clinical outcomes.

Tetralogy of Fallot patients for corrective surgery were randomised to receive curcumin (45 mg/day) or placebo orally for 14 days before surgery. Malondialdehyde and glutathione concentrations were evaluated during the pre-ischaemia, ischaemia, re-perfusion phases, and 6 hours after aortic clamping-off. Nuclear factor-kappa B, c-Jun N-terminal kinase, and caspase-3, taken from the infundibulum, were assessed during the pre-ischaemia, ischaemia, and re-perfusion phases. Haemodynamic parameters were monitored until day 5 after surgery.

In all the observation phases, malondialdehyde and glutathione concentrations were similar between groups. There was no significant difference in nuclear factor-kappa B activity between the groups for three observations; however, in the curcumin group, c-Jun N-terminal kinase significantly decreased from the pre-ischaemia to the re-perfusion phases, and caspase-3 expression was lower in the ischaemia phase. Patients in the curcumin group had lower temperature and better ventricular functions, but no significant differences were found in mechanical ventilation day or length of hospital stay in the two groups.

Cardioprotective effects of curcumin may include inhibition of the c-Jun N-terminal kinase pathway and caspase-3 in cardiomyocytes, particularly in the ischaemia phase.

IL-27, a member of the IL-12 family, has been involved in maternal tolerance to the foetus and successful pregnancy. Growing evidences indicate that IL-27 plays a crucial role in pregnancy.

We carried out the present study in order to investigate whether polymorphisms in the IL27 are associated with the risk for CHDs, including atrial septal defect and ventricular septal defect.

We conducted this case–control study among 247 atrial septal defect patients, 150 ventricular septal defect patients, and 368 healthy controls in a Chinese population using polymerase chain reaction-restriction fragment length polymorphism assay.

Significantly increased risk for atrial septal defect (p=0.001, OR=1.490, 95% CI=1.178–1.887) and ventricular septal defect (p=0.004, OR=1.502, 95% CI=1.139–1.976) was observed to be associated with the allele G of rs153109. In a dominant model, we have also observed that increased susceptibilities for atrial septal defect (p<0.01, OR=1.89, 95% CI=1.35–2.63) and ventricular septal defect (p<0.01, OR=2.50, 95% CI=1.67–3.85) were statistically associated with rs153109; however, no association was found between CHD risk and rs17855750 in the IL27 gene.

The 153109 of the IL27 gene may be associated with the susceptibility to CHD, including atrial septal defect and ventricular septal defect.

The purpose of this study was to determine whether a murmur detected on routine pre-discharge examination of asymptomatic newborn children in the first 48 hours of life warrants further investigation with echocardiography.

We conducted a retrospective review of all echocardiography studies of neonates born at Jordan University Hospital between August, 2007 and June, 2014. Findings on physical examination as well as the indication of the echocardiographic studies were reviewed. We included asymptomatic neonates for whom echocardiographic studies were carried out due to the sole indication of a heart murmur on routine pre-discharge neonatal physical examination.

Of 309 asymptomatic newborns with murmurs on pre-discharge examination, echocardiography revealed 68 (22%) cases of CHD, with 18 (6%) designated as significant heart disease with anticipated intervention during infancy or childhood. The most common abnormality was ventricular septal defect occurring in 36 cases. Critical heart diseases detected included hypoplastic left heart syndrome in two and aortic valve stenosis in four newborns.

Although most asymptomatic neonates with heart murmurs have normal hearts, a small percentage may have significant heart disease. The decision to refer an asymptomatic newborn with a murmur for echocardiography before discharge from the hospital remains controversial and must be supported by other evidence such as murmur characteristics and local trends in parental compliance with follow-up well-baby visits.

Different echocardiographic parameters have been studied and validated for assessing the severity of mitral stenosis; however, scant data are available for these markers in the context of balloon mitral valvuloplasty in juvenile age groups (ages ⩽20 years).

The aim of the present study was to find out the utility of echocardiographic parameters such as mitral valve separation index, left atrial volume, right ventricular systolic pressure, tricuspid annular plane systolic excursion, tricuspid annular systolic velocity, and right ventricular Tei index in predicting success of balloon mitral valvuloplasty and their relation to mitral valve area in juvenile mitral stenosis.

We carried out a prospective single-centre study involving 52 juvenile mitral stenosis patients undergoing elective valvuloplasty. Success was defined as an increase in mitral valve area ⩾50% or ⩾1.5 cm2. Echocardiographic measurements were taken before and 24 hours after the procedure and statistical analyses were carried out.

The mean age of the study population was 14.3 years (SD ±4.55), ranging from 7 to 20 years. Valvuloplasty was successful in 49 out of 52 patients. The mean valve area improved from 0.89 (SD ±0.16) to 1.73 (SD ±0.22) cm2/m2 (p<0.01), and the mean mitral valve gradient decreased from 19.87 (SD ±7.89) to 7.45 (SD ±2.07) (p=0.021). All the surrogate parameters improved favourably after valvuloplasty (p<0.01). The decrease in right ventricular systolic pressure was a better indicator of the success followed by the increase in valve separation index (area under the curve 0.81 and 0.76, respectively).

All the surrogate markers studied showed favourable improvement, and right ventricular systolic pressure reduction and improved mitral valve separation index were better indicators of successful valvuloplasty.

The peri-operative mortality of the arterial switch operation in neonates with transposition of the great arteries is considerably low; however, long-term outcomes of translocated coronary arteries still remain one of the most crucial issues.

A total of 110 neonates with transposition of the great arteries after arterial switch operation were evaluated; three (2.7%) late deaths occurred. The remaining 107 patients except for one underwent follow-up angiography. Angiography showed coronary artery stenosis in nine (8.4%), with right coronary artery lesions in two and left main trunk lesions in seven. In two patients, right coronary artery stenosis regressed during follow-up. In left main trunk lesions, the severity of stenosis improved in four, did not change in one, and progressed to total occlusion in two patients. In children with coronary artery stenosis, myocardial scintigraphy showed perfusion defects in five out of six (83%) with left main trunk with ⩾75% stenosis and in four out of four with left main trunk stenosis ⩾90%. In contrast, patients whose coronary artery stenosis disappeared during follow-up had no perfusion defects on scintigraphy.

Regression of ostial stenosis of the transplanted coronary artery on angiogram was observed. The stenosis regressed over time in six patients; two coronary arteries with 99% stenosis and delayed angiographic enhancement of the distal coronary artery resulted in total occlusion within 1 year after the arterial switch operation. Combination of angiography and myocardial scintigraphy could be useful to differentiate deceptive stenosis from progressive stenosis.

Remote monitoring is increasingly used in the follow-up of patients with cardiac implantable electronic devices. Data on paediatric populations are still lacking. The aim of our study was to follow-up young patients both in-hospital and remotely to enhance device surveillance.

This is an observational registry collecting data on consecutive patients followed-up with the CareLink system. Inclusion criteria were a Medtronic device implanted and patient’s willingness to receive CareLink. Patients were stratified according to age and presence of congenital/structural heart defects (CHD).

A total of 221 patients with a device – 200 pacemakers, 19 implantable cardioverter defibrillators, and two loop recorders – were enrolled (median age of 17 years, range 1–40); 58% of patients were younger than 18 years of age and 73% had CHD. During a follow-up of 12 months (range 4–18), 1361 transmissions (8.9% unscheduled) were reviewed by technicians. Time for review was 6±2 minutes (mean±standard deviation). Missed transmissions were 10.1%. Events were documented in 45% of transmissions, with 2.7% yellow alerts and 0.6% red alerts sent by wireless devices. No significant differences were found in transmission results according to age or presence of CHD. Physicians reviewed 6.3% of transmissions, 29 patients were contacted by phone, and 12 patients underwent unscheduled in-hospital visits. The event recognition with remote monitoring occurred 76 days (range 16–150) earlier than the next scheduled in-office follow-up.

Remote follow-up/monitoring with the CareLink system is useful to enhance device surveillance in young patients. The majority of events were not clinically relevant, and the remaining led to timely management of problems.

To analyse the relationship between skipping breakfast and haemodynamic, metabolic, inflammatory, and cardiovascular risk factors in adolescents.

A cross-sectional study was carried out with information from an ongoing cohort study in Presidente Prudente, São Paulo, Brazil. The sample comprised of 120 adolescents (11.7±0.8 years old) who met the following inclusion criteria: age between 11 and 14 years; enrolled in the school unit of elementary education; absence of any known disease; and no drug consumption. The parents or legal guardians of the patients signed a formal informed consent. Skipping breakfast was self-reported through face-to-face interviews. Blood pressure, intima-media thickness, trunk fatness, total and fractional cholesterol levels – high-density lipoprotein cholesterol and low-density lipoprotein cholesterol – triacylglycerol levels, and high-sensitivity C-reactive protein levels were measured.

In this study, 47.5% (95% CI: 38.5–56.4%) of the adolescents reported skipping breakfast at least 1 day/week. Adolescents who skipped breakfast had higher values of trunk fatness and systolic blood pressure. Breakfast frequency was negatively related to systolic blood pressure (β −1.99 [−3.67; −0.31]) and z score dyslipidaemia (β −0.46 [−0.90; −0.01]), but this relationship was mediated by trunk fatness.

Skipping breakfast is related to cardiovascular risk factors in adolescents, and this relationship was mainly mediated by trunk fatness.

The purpose of our study was to evaluate the association between insulin resistance and left ventricular size and function in obese children.

A total of 79 cases aged 10–16 years and diagnosed with obesity and 79 healthy and non-obese cases as controls were included in the study. Patient and control groups were divided into three groups in terms of age as group 1 (10–12 years), group 2 (12–14 years), and group 3 (14–16 years). Fasting blood glucose, lipid profile, and fasting insulin levels of the cases were assessed. Mitral valve E and A waves, left ventricular ejection fraction, fractional shortening, end-diastolic and end-systolic diameters, left atrium diameter, and septal wall thickness were measured using echocardiography.

Measurements of septal diastolic thicknesses, left atrium diameter, and left ventricular end-systolic diameter of all the three groups obtained by echocardiography were statistically higher compared with the controls. In all the patient groups, the mitral valve E/A ratio was >1. In groups 2 and 3, there was a positive correlation between fasting insulin levels and HOMA-IR and left ventricular end-systolic diameter, end-diastolic diameter, and septal systolic and diastolic wall thicknesses.

In paediatric obesity, identification of early cardiac changes will be significant in allowing early diagnosis and treatment of cardiovascular diseases.

Surgical correction was the treatment of choice for pulmonary stenosis until three decades ago, when balloon valvuloplasty was implemented. The natural history of surgically relieved pulmonary stenosis has been considered benign but is actually unknown, as is the need for re-intervention.

The objective of this study was to investigate the morbidity and mortality of patients with surgically treated pulmonary stenosis operated at Aarhus University Hospital between 1957 and 2000.

The total study population included 80 patients. In-hospital mortality was 2/80 (2.5%), and an additional four patients died after hospital discharge; therefore, the long-term mortality was 5%. The maximum follow-up period was 57 years, with a median of 33 years. In all, 16 patients (20%) required at least one re-intervention. Pulmonary valve replacement due to pulmonary regurgitation was the most common re-intervention (67%). Freedom from re-intervention decreased >20 years after the initial repair. In addition, 45% of patients had moderate/severe pulmonary regurgitation, 38% had some degree of right ventricular dilatation, and 40% had some degree of tricuspid regurgitation, which did not require re-intervention at the present stage.

Surgical relief for pulmonary stenosis is efficient in relieving outflow obstruction; however, this efficiency is achieved at the cost of pulmonary regurgitation, leading to right ventricular dilatation and tricuspid regurgitation. When required, pulmonary valve replacement is performed most frequently >20 years after the initial surgery. Lifelong follow-up of patients treated surgically for pulmonary stenosis is emphasised in this group of patients, who might otherwise consider themselves cured.

Our aim was to determine the optimal cut-off values, sensitivity, specificity, and diagnostic power of 12 echocardiographic parameters on the second day of life to predict subsequent ductal patency.

We evaluated preterm infants, born at ⩽32 weeks of gestation, starting on their second day of life, and they were evaluated every other day until ductal closure or until there were clinical signs of re-opening. We measured transductal diameter; pulmonary arterial diastolic flow; retrograde aortic diastolic flow; pulsatility index of the left pulmonary artery and descending aorta; left atrium and ventricle/aortic root ratio; left ventricular output; left ventricular flow velocity time integral; mitral early/late diastolic flow; and superior caval vein diameter and flow as well as performed receiver operating curve analysis.

Transductal diameter (>1.5 mm); pulmonary arterial diastolic flow (>25.6 cm/second); presence of retrograde aortic diastolic flow; ductal diameter by body weight (>1.07 mm/kg); left pulmonary arterial pulsatility index (⩽0.71); and left ventricle to aortic root ratio (>2.2) displayed high sensitivity and specificity (p<0.0001; area under the curve>0.9). Parameters with moderate sensitivity and specificity were as follows: left atrial to aortic root ratio; left ventricular output; left ventricular flow velocity time integral; and mitral early/late diastolic flow ratio (p<0.05; area under the curve 0.7–0.88). Descending aortic pulsatility index and superior caval vein diameter and flow (p>0.05) had low diagnostic value.

Left pulmonary arterial pulsatility index, left ventricle/aortic root ratio, and ductal diameter by body weight are useful adjuncts offering a broader outlook for predicting ductal patency.

Parental understanding of their children’s heart disease is inadequate, which may contribute to poor health outcomes. The purpose of this study was to determine what parental knowledge is important in the care of children with heart disease from the perspective of parents, nurses, and physicians.

Focus groups were formed with parents of children with single ventricle congenital heart disease (CHD), biventricular CHD, and heart transplantation, and with nurses and physicians who provide care for these children. A nominal group technique was used to identify and prioritise important parental knowledge items and themes. The voting data for each theme were reported by participant type – parent, nurse, and physician – and patient diagnosis – single ventricle CHD, biventricular CHD, and heart transplantation.

The following three themes were identified as important by all groups: recognition of and response to clinical deterioration, medications, and prognosis and plan. Additional themes that were unique to specific groups included the following: medical team members and interactions (parents), tests and labs (parents), neurodevelopmental outcomes and interventions (physicians), lifelong disease requiring lifelong follow-up (physicians and nurses), and diagnosis, physiology, and interventions (single ventricle and biventricular CHD).

Parents, nurses, and physicians have both common and unique views regarding what parents should know to effectively care for their children with single ventricle CHD, biventricular CHD, or heart transplantation. Specific targeted parental education that incorporates these findings should be provided to each group. Further development of questionnaires regarding parental knowledge with appropriate content validity is warranted.

The effect of Hybrid stage 1 palliation for hypoplastic left heart syndrome on right ventricular function is unknown. We sought to compare right ventricular function in normal neonates and those with hypoplastic left heart syndrome before Hybrid palliation and to assess the effect of Hybrid palliation on right ventricular function, using the right ventricular myocardial performance index and the ratio of systolic and diastolic durations.

We carried out a retrospective review of echocardiographic data on 23 infants with hypoplastic left heart syndrome who underwent Hybrid palliation and 35 normal controls. Data were acquired before Hybrid and after Hybrid palliation – post 1, 0–4 days; post 2, 1 week; post 3, 2–3 weeks; post 4, 1–1.5 months following Hybrid palliation.

Myocardial performance index and ratio of systolic and diastolic durations were higher in the pre-Hybrid hypoplastic left heart syndrome group (n=23) – 0.47±0.16 versus 0.25±0.07, p<0.001; 1.59±0.44 versus 1.09±0.14, p<0.0001 – compared with controls (n=35). There was no significant change in the myocardial performance index at any of the post-Hybrid time points. Ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid – post 3: 2.08±0.62 and post 4: 2.21±0.45 versus pre: 1.59±0.44, p=0.043 and 0.003. There were no significant differences in parameters between sub-groups of infants who died (n=10) and survivors (n=13).

Right ventricular myocardial performance index and ratio of systolic and diastolic durations were significantly higher in infants with hypoplastic left heart syndrome before intervention compared with controls. The ratio of systolic and diastolic durations increased significantly 2 weeks after Hybrid palliation. Our data suggest that infants with hypoplastic left heart syndrome have right ventricular dysfunction before intervention, which worsens over 2 weeks after Hybrid palliation.