Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.

We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.

A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).

The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Population-based registries report 95% 5-year survival for children undergoing surgery for CHD. This study investigated paediatric cardiac surgical outcomes in the Australian indigenous population.

All children who underwent cardiac surgery between May, 2008 and August, 2014 were studied. Demographic information including socio-economic status, diagnoses and co-morbidities, and treatment and outcome data were collected at time of surgery and at last follow-up.

A total of 1528 children with a mean age 3.4±4.6 years were studied. Among them, 123 (8.1%) children were identified as indigenous, and 52.7% (62) of indigenous patients were in the lowest third of the socio-economic index compared with 28.2% (456) of non-indigenous patients (p⩽0.001). The indigenous sample had a significantly higher Comprehensive Aristotle Complexity score (indigenous 9.4±4.2 versus non-indigenous 8.7±3.9, p=0.04). The probability of having long-term follow-up did not differ between groups (indigenous 93.8% versus non-indigenous 95.6%, p=0.17). No difference was noted in 30-day mortality (indigenous 3.2% versus non-indigenous 1.4%, p=0.13). The 6-year survival for the entire cohort was 95.9%. The Cox survival analysis demonstrated higher 6-year mortality in the indigenous group – indigenous 8.1% versus non-indigenous 5.0%; hazard ratio (HR)=2.1; 95% confidence intervals (CI): 1.1, 4.2; p=0.03. Freedom from surgical re-intervention was 79%, and was not significantly associated with the indigenous status (HR=1.4; 95% CI: 0.9, 1.9; p=0.11). When long-term survival was adjusted for the Comprehensive Aristotle Complexity score, no difference in outcomes between the populations was demonstrated (HR=1.6; 95% CI: 0.8, 3.2; p=0.19).

The indigenous population experienced higher late mortality. This apparent relationship is explained by increased patient complexity, which may reflect negative social and environmental factors.

The use of ultrasound for assessing diaphragmatic dysfunction after paediatric cardiac surgery may be under-utilised. This study aimed to evaluate the role of bedside ultrasound performed by an intensivist to diagnose diaphragmatic dysfunction and the need for plication after paediatric cardiac surgery.

We carried out a retrospective cohort study on prospectively collected data of postoperative children admitted to the paediatric cardiac ICU during 2013. Diaphragmatic dysfunction was suspected based on difficulties in weaning from positive pressure ventilation or chest X-ray findings. Ultrasound studies were performed by the paediatric cardiac ICU intensivist and confirmed by a qualified radiologist.

Out of 344 postoperative patients, 32 needed diaphragm ultrasound for suspected dysfunction. Ultrasound studies confirmed diaphragmatic dysfunction in 17/32 (53%) patients with an average age and weight of 10.8±3.8 months and 6±1 kg, respectively. The incidence rate of diaphragmatic dysfunction was 4.9% in relation to the whole population. Diaphragmatic plication was needed in 9/17 cases (53%), with a rate of 2.6% in postoperative cardiac children. The mean plication time was 15.1±1.3 days after surgery. All patients who underwent plication were under 4 months of age. After plication, they were discharged with mean paediatric cardiac ICU and hospital stay of 19±3.5 and 42±8 days, respectively.

Critical-care ultrasound assessment of diaphragmatic movement is a useful and practical bedside tool that can be performed by a trained paediatric cardiac ICU intensivist. It may help in the early detection and management of diaphragmatic dysfunction after paediatric cardiac surgery through a decision-making algorithm that may have potential positive effects on morbidity and outcome.

The present study aims to identify the role of inflammatory markers such as C-reactive protein, interleukin-6, and fractalkine in CHD-associated pulmonary hypertension in children.

This is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children.

Serum C-reactive protein and interleukin-6 levels were significantly higher in the children with CHD-related pulmonary hypertension (respectively, p=0.049 and 0.026). Serum C-reactive protein concentrations correlated negatively with ejection fraction (r=−0.609, p=0.001) and fractional shortening (r=−0.452, p=0.007) in the pulmonary hypertension group. Serum fractalkine concentrations correlated negatively with ejection fraction (r=−0.522, p=0.002) and fractional shortening (r=−0.395, p=0.021) in the children with pulmonary hypertension. Serum interleukin-6 concentrations also correlated negatively with Qs (r=−0.572, p=0.021), positively with Rs (r=0.774, p=0.001), and positively with pulmonary wedge pressure (r=0.796, p=0.006) in the pulmonary hypertension group. A cut-off value of 2.2 IU/L for C-reactive protein was able to predict pulmonary hypertension with 77.5% sensitivity and 77.5% specificity. When the cut-off point for interleukin-6 concentration was 57.5 pg/ml, pulmonary hypertension could be predicted with 80% sensitivity and 75% specificity.

Inflammation is associated with the pathophysiology of pulmonary hypertension. The inflammatory markers C-reactive protein and interleukin-6 may have a role in the clinical evaluation of paediatric pulmonary hypertension related to CHDs.

In Kawasaki disease, although coronary dilatation is attributed to vasculitis, the effect of myocardial inflammation is underestimated. Coronary dilatations are determined by Z-scores, which do not take into account dominance. The aim of the present study was to describe the impact of coronary dominance on dilatation in Kawasaki disease.

We performed a retrospective analysis of coronary dilatations according to angiography categorisation of dominance.

Of 28 patients (2.6 [0.2–10.1] years), right dominance was present in 15 patients and left in 13. Early dilatation was present in all patients, of whom 11 were ipsilateral to the dominant segment and 17 contralateral. Ipsilateral dilatations were present at diagnosis (9/11 versus 6/17, p=0.02) compared with contralateral dilatations, which developed 2 weeks after diagnosis (9/11 versus 16/17, p=0.29). Coronary artery Z-scores of patients with contralateral dilatation increased at 2 weeks, before returning to baseline values (2.0±2.2 at diagnosis, 4.1±1.8 at 2 weeks, 1.8±1.2 at 3–6 months, p=0.001), compared with patients with ipsilateral dilatation in whom Z-scores were maximal at diagnosis and remained stable (3.0±0.9, 2.7±1.1 and 2.6±1.5, respectively, p=0.13). Dominant coronary artery Z-scores were higher compared with non-dominant segments at diagnosis (3.0±0.9 versus 1.0±0.8, p<0.001) and at late follow-up (2.6±1.5 versus 0.4±1.4, p=0.002) in patients with ipsilateral dilatation.

Progression of coronary dilatation after diagnosis may be a sign of dilatation secondary to vasculitis, as opposed to regression of Z-scores in ipsilateral dilatations, probably related to physiological vasodilatation in response to carditis. This needs to be validated in larger studies against vasculitic and myocardial inflammatory markers.

The prevalence of overweight in children with CHD is about 26.9%. Increase in adipose tissue is related to the secretion of proinflammatory markers such as C-reactive protein. Assuming that children with CHD are exposed to other inherent risk factors for heart disease, our objective was to evaluate the correlation between levels of C-reactive protein and body mass index in children and adolescents with CHD.

A cross-sectional study with 377 children and adolescents with CHD in a clinical setting of a reference hospital was carried out. C-reactive protein data were collected after 12 hours of fasting. Nutritional status was classified according to body mass index. The patients were divided into three groups: cyanotic, acyanotic, and minimal heart defects (controls).

The mean age was 9.9±4.2 years, and 53.6% of the sample included males. The cyanotic group represented 22.3%, acyanotic 42.2%, and minimal defects 35.5% of the sample. The average body mass index percentile was 57.23±32.06. The median values of C-reactive protein were as follows: cyanotic 0.340, acyanotic with clinical repercussion 0.203, and minimal defects 0.128. There was a significant difference between the minimal defects and the cyanotic groups (p=0.023). There was a significant correlation between C-reactive protein and body mass index percentile (r=0.293, p<0.01). C-reactive protein levels were higher in girls (p=0.034). There were no significant correlations between C-reactive protein and age or birth weight.

The correlation between body mass index percentile and C-reactive protein was confirmed in this population. The prevention of overweight is paramount to avoid overlapping modifiable risk factors to those already inherent to the CHD.

The prevalence of CHD has been well described worldwide except in Tibet. This study aimed to illustrate the prevalence and composition of CHD in Tibetan children according to altitude.

In the first part, we prospectively recruited 7088 unselected Tibetan children (4–17 years) from south-west Tibet. The total prevalence of CHD increased from 4.6/1000 below 4200 m to 13.4/1000 above 4700 m, with a female-to-male ratio of 1.3:3.1. The total prevalence and female prevalence of patent ductus arteriosus increased more than 10-fold. Females living above 4700 m had exceptionally high prevalence of patent ductus arteriosus (14.9/1000). The prevalence of atrial septal defect was comparable among different altitudes (3.3–3.8/1000). The prevalence of ventricular septal defect was 1.3/1000 below 4700 m, and no cases were found above this altitude. In the second part, we retrospectively reviewed the clinical data of 383 CHD children in Tibet and 73 children at lower altitudes. The percentage of isolated ventricular septal defect decreased from 54.8 to 3.1%, and the percentage of isolated patent ductus arteriosus increased from 8.2 to 68.4% with elevation. Children living below 4200 m (10.4–13.7%) had a larger proportion of complex CHD than those above this altitude (2.0–3.1%). Of the 20 Tibetan children with complex CHD, 14 (70.0%) lived below 4200 m.

A wide variation in CHD prevalence and composition existed in Tibetan children among different altitudes.

There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions.

A three-dimensional Fontan model was constructed on the basis of CT images, and a 4-mm-diameter fenestration was located between the extracardiac Fontan conduit and the right atrium at three positions: superior, middle, and inferior part of the conduit. Haemodynamics in the Fontan route were analysed using a three-dimensional computational fluid dynamic model in realistic physiological conditions, which were predicted using a lumped parameter model of the cardiovascular system. The respiratory effect of the caval flow was taken into account. The flow rate through the fenestration, the effect of lowering the central venous pressure, and wall shear stress in the Fontan circuit were evaluated under central venous pressures of 10, 15, and 20 mmHg. The pulse power index and pulsatile energy loss index were calculated as energy loss indices.

Under all central venous pressures, the middle-part fenestration demonstrated the most significant effect on enhancing the flow rate through the fenestration while lowering the central venous pressure. The middle-part fenestration produced the highest time-averaged wall shear stress, pressure pulse index, and pulsatile energy loss index.

Despite slightly elevated energy loss, the middle-part fenestration most significantly increased cardiac output and lowered central venous pressure under respiration in the Fontan circulation.

Fontan survivors have depressed cardiac index that worsens over time. Serum biomarker measurement is minimally invasive, rapid, widely available, and may be useful for serial monitoring. The purpose of this study was to identify biomarkers that correlate with lower cardiac index in Fontan patients.

This study was a multi-centre case series assessing the correlations between biomarkers and cardiac magnetic resonance-derived cardiac index in Fontan patients ⩾6 years of age with biochemical and haematopoietic biomarkers obtained ±12 months from cardiac magnetic resonance. Medical history and biomarker values were obtained by chart review. Spearman’s Rank correlation assessed associations between biomarker z-scores and cardiac index. Biomarkers with significant correlations had receiver operating characteristic curves and area under the curve estimated. In total, 97 cardiac magnetic resonances in 87 patients met inclusion criteria: median age at cardiac magnetic resonance was 15 (6–33) years. Significant correlations were found between cardiac index and total alkaline phosphatase (−0.26, p=0.04), estimated creatinine clearance (0.26, p=0.02), and mean corpuscular volume (−0.32, p<0.01). Area under the curve for the three individual biomarkers was 0.63–0.69. Area under the curve for the three-biomarker panel was 0.75. Comparison of cardiac index above and below the receiver operating characteristic curve-identified cut-off points revealed significant differences for each biomarker (p<0.01) and for the composite panel [median cardiac index for higher-risk group=2.17 L/minute/m2 versus lower-risk group=2.96 L/minute/m2, (p<0.01)].

Higher total alkaline phosphatase and mean corpuscular volume as well as lower estimated creatinine clearance identify Fontan patients with lower cardiac index. Using biomarkers to monitor haemodynamics and organ-specific effects warrants prospective investigation.

Hypertension following primary coarctation repair affects up to a third of subjects. A number of studies suggest that future hypertension risk is reduced if primary repair is performed at a younger age.

The objective of this study was to evaluate the risk of future medical treatment for hypertension depending on age of primary coarctation repair.

This study was carried out at a tertiary paediatric cardiology referral centre. Retrospective database evaluation of children aged <16 years undergoing primary surgical coarctation repair between October, 2005 and October, 2014 was carried out. Patients with complex heart diseases were excluded. The following age groups were considered: neonate (⩽28 days), infant (>28 days and ⩽12 months), and children (>12 months). Main outcome measure is the need for long-term anti-hypertensive medication. The risk for re-coarctation was also evaluated.

A total of 87 patients were analysed: 60 neonates, 17 infants, 10 children. Among them, 6.7% neonates, 29.4% infants, and 40% children required long-term anti-hypertensive medications. Group differences were statistically significant (p=0.004). After adjustment for type of repair, the risk of long-term anti-hypertensive therapy was 4.5 (95% confidence interval 1.2–16.9, p=0.025) and 10.5 times (95% confidence interval 2.6–42.3, p=0.001) higher if primary repair was carried out in infancy and childhood, respectively, compared with neonates. Among all, 13 patients developed re-coarctation: 21.7% in the neonatal group, 5.9% in the infant group, and 20% in the child group. We could not demonstrate a significant difference between these proportions or calculate a reliable risk for developing re-coarctation.

Risk of medical treatment for hypertension was lowest when primary repair was carried out during the neonatal period, rising 10-fold if first operated on as a child. Knowing the likelihood of hypertension development depending on age of primary repair is useful for long-term surveillance and counselling.

In 2003, work-hour regulations were implemented by the Accreditation Council for Graduate Medical Education. Much has been published regarding resident rest and quality of life as well as patient safety. There has been no examination on the effect of work-hour restrictions on academic productivity of fellows in training. Paediatric subspecialty fellows have a scholarly requirement mandated by the American Board of Pediatrics. We have examined the impact of work-hour restrictions on the scholarly productivity of paediatric cardiology fellows during their fellowship.

We conducted a literature search for all paediatric cardiology fellows between 1998 and 2007 at a single academic institution as first or senior authors on papers published during their 3-year fellowship and 3 years after completion of their categorical fellowship (n=63, 30 fellows before 2003 and 33 fellows after 2003). The numbers of first- or senior-author fellow publications before and after 2003 were compared. We also collected data on final paediatric cardiology subspecialty career choice.

There was no difference in the number of fellow first-author publications before and after 2003. Before work-hour restrictions, the mean number of publications per fellow was 2.1 (±2.2), and after work-hour restrictions it was 2.0 (±1.8), (p=0.89). By subspecialty career choice, fellows who select electrophysiology, preventative cardiology, and heart failure always published within the 6-year time period.

Since the implementation of work-hour regulations, total number of fellow first-authored publications has not changed. The role of subspecialty choice may play a role in academic productivity of fellows in training.

Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings.

An international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology.

Palivizumab prophylaxis was recommended for children with the following conditions: <2 years with unoperated haemodynamically significant CHD, who are cyanotic, who have pulmonary hypertension, or symptomatic airway abnormalities; <1 year with cardiomyopathies requiring treatment; in the 1st year of life with surgically operated CHD with haemodynamically significant residual problems or aged 1–2 years up to 6 months postoperatively; and on heart transplant waiting lists or in their 1st year after heart transplant. Unanimous consensus was not reached for use of immunoprophylaxis in children with asymptomatic CHD and other co-morbid factors such as arrhythmias, Down syndrome, or immunodeficiency, or during a nosocomial outbreak. Challenges to effective immunoprophylaxis included the following: multidisciplinary variations in identifying candidates with CHD and prophylaxis compliance; limited awareness of severe disease risks/burden; and limited knowledge of respiratory syncytial virus seasonal patterns in subtropical/tropical regions.

Evidence-based immunoprophylaxis recommendations were formulated for subgroups of children with CHD, but more data are needed to guide use in tropical/subtropical countries and in children with certain co-morbidities.

The aim of this study was to describe previously unrecognised or under-recognised adverse events associated with Melody® valve implantation.

In rare diseases and conditions, it is typically not feasible to conduct large-scale safety trials before drug or device approval. Therefore, post-market surveillance mechanisms are necessary to detect rare but potentially serious adverse events.

We reviewed the United States Food and Drug Administration’s Manufacturer and User Facility Device Experience (MAUDE) database and conducted a structured literature review to evaluate adverse events associated with on- and off-label Melody® valve implantation. Adverse events were compared with those described in the prospective Investigational Device Exemption and Post-Market Approval Melody® transcatheter pulmonary valve trials.

We identified 631 adverse events associated with “on-label” Melody® valve implants and 84 adverse events associated with “off-label” implants. The most frequent “on-label” adverse events were similar to those described in the prospective trials including stent fracture (n=210) and endocarditis (n=104). Previously unrecognised or under-recognised adverse events included stent fragment embolisation (n=5), device erosion (n=4), immediate post-implant severe valvar insufficiency (n=2), and late coronary compression (n=2 cases at 5 days and 3 months after implantation). Under-recognised adverse events associated with off-label implantation included early valve failure due to insufficiency when implanted in the tricuspid position (n=7) and embolisation with percutaneous implantation in the mitral position (n=5).

Post-market passive surveillance does not demonstrate a high frequency of previously unrecognised serious adverse events with “on-label” Melody® valve implantation. Further study is needed to evaluate safety of “off-label” uses.