Although chylothorax is an uncommon complication following paediatric cardiothoracic surgery, it has significant associated morbidities and increased in-hospital mortality, as well as results in higher costs. A lack of prospective evidence or consensus guidelines for management of chylothorax further hinders optimal management. The aim of this survey was to characterise variations in practice in the management of chylothorax and to identify areas for future research.

A descriptive, observational survey investigating conservative management practices of chylothorax was distributed internationally to health-care professionals in paediatric intensive care and cardiology units. The survey investigated five domains: the first providing general information about health-care professionals and four domains focusing on clinical practice including diet composition and duration.

In total, sixty-four health-care professionals completed the survey, representing 38 organisations from 16 countries. The respondents were dietitians (80%), physicians (19%), and nurses (1%). In Australia and New Zealand, management was most commonly directed by physicians’ preference (67%) as compared to unit protocols in Europe (67%), United States of America (67%), and Other regions (55%). Dietitians in Australia/New Zealand, United Kingdom, and Ireland followed the most restrictive diet therapy recommending <5 g long chain triglyceride fat per day (p < 0.00001). The duration of diet therapy significantly varied between regions: Australia/New Zealand: 4 weeks (36%) and 6 weeks (43%); Europe: 4 weeks (25%) and 6 weeks (57%); and North America: 4 weeks (18%) and 6 weeks (75%) (p < 0.00001).

This survey highlights international variations in practice in the management of chylothorax, particularly with respect to treatment duration and dietary fat restriction. Future research should include a multi-centre randomised controlled trial to inform evidence-based practice and reduce morbidity, particularly poor growth.

Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate.

We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus–Kaye–Stansel procedure over the past decade at a single national cardiology centre.

Of 114 children who underwent Norwood procedure or Damus–Kaye–Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2–82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10–70mmHg) to 2mmHg (range 0–20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2–7.9 mm) to 8.6 mm (range 6.2–10.9 mm). The median coarctation index increased by 0.49 (range = 0.24–0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications.

The prevalence of recurrent aortic arch obstruction following Norwood/Damus–Kaye–Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.

Understanding perceptions of family caregivers’ roles and responsibilities regarding their child with complex cardiac needs has potential to help care teams better support parents. Paternal experience has been under-explored in pediatric cardiac cohorts.

Ten fathers of children undergoing cardiac surgery completed quantitative surveys on their knowledge needs and preferred format of communication. In face-to-face recorded interviews, they responded to open-ended questions about the definition of being a good father to a child with a complex cardiac condition, perceived paternal responsibilities, personal growth as a parent to a child with a complex heart condition, support needs, and recommendations to medical staff for paternal inclusion. Semantic content analysis was utilised. The study reports strictly followed COnsolidated criteria for REporting Qualitative research guidelines.

The fathers reported high preference for knowledge about the child’s heart condition, communication about the treatment plan, and desire for inclusion in the care of their child. Paternal role was defined thematically as: providing a supportive presence, being there, offering bonded insight, serving as strong provider, and acting as an informed advocate. The fathers revealed that their responsibilities sometimes conflicted as they strove to serve as an emotional and economic stabiliser for their family, while also wanting to be foundationally present for their child perioperatively.

This study provides insight into paternal experience and strategies for paternal inclusion. This summary of the self-defined experience of the fathers of pediatric cardiac patients offers constructive and specific advice for medical teams.

Assessment of right ventricular size and function is an important part of the clinical cardiac evaluation; however, these quantitative measures are challenging by echocardiography. Automated software could be useful in place of manual measurements and qualitative assessment. This study evaluates a semi-automated software by comparing automated to manual measures in normal children.

Neonates to adolescents with normal echocardiograms were prospectively enrolled. Measurements were performed using manual techniques and semi-automated software (EchoInsight®, Epsilon Imaging, Ann Arbor, Michigan, United States of America). Right ventricular measurements included end-diastolic and end-systolic area, fractional area change, chamber dimensions, and tricuspid annular plane systolic excursion. Agreement between manual and semi-automated measures was compared.

Echocardiograms for 233 patients were included in the analysis. Intra- and inter-observer reliabilities for semi-automated measures were good with intraclass correlation coefficients all over 0.9 and 0.85, respectively. There was very strong correlation between manual and semi-automated methods for areas and dimensions (r = 0.93–0.99) and low bias (1.4–10.8%). For functional measures, tricuspid annular plane systolic excursion measures correlated well (r = 0.84), but fractional area change did not (r = 0.50). Both demonstrated significant bias (33.5–43.0%). The semi-automated method consistently underestimated fractional area change with a mean of 26.6% versus a manual mean of 36.1%.

The semi-automated software is capable of generating quantitative right ventricular measures in children with good reliability. The software demonstrates very good correlation and low bias when compared to manual methods for right ventricular areas and dimensions. There is a significant difference between manual and semi-automated techniques for the functional measures.

Limited data exist for management of hyperuricemia in non-oncologic patients, particularly in paediatric cardiac patients. Hyperuricemia is a risk factor for acute kidney injury and may prompt treatment in critically ill patients. The primary objective was to determine if rasburicase use was associated with greater probability normalisation of serum uric acid compared to allopurinol. Secondary outcomes included percent reduction in uric acid, changes in serum creatinine, and cost of therapy.

A single-centre retrospective chart review.

A 20-bed quaternary cardiovascular ICU in a university-based paediatric hospital in California.

Patients admitted to cardiovascular ICU who received rasburicase or intravenous allopurinol between 2015 and 2016.

None.

Data from a cohort of 14 patients receiving rasburicase were compared to 7 patients receiving IV allopurinol. Patients who were administered rasburicase for hyperuricemia were more likely to have a post-treatment uric acid level less than 8 mg/dl as compared to IV allopurinol (100 versus 43%; p = 0.0058). Patients who received rasburicase had a greater absolute reduction in post-treatment day 1 uric acid (−9 mg/dl versus −1.9 mg/dl; p = 0.002). There were no differences in post-treatment day 3 or day 7 serum creatinine or time to normalisation of serum creatinine. The cost of therapy normalised to a 20 kg patient was greater in the allopurinol group ($18,720 versus $1928; p = 0.001).

In a limited paediatric cardiac cohort, the use of rasburicase was associated with a greater reduction in uric acid levels and associated with a lower cost compared to IV allopurinol.

Infants with CHD often experience growth failure. Ensuring optimal growth before surgery is associated with improved outcomes and has emerged as a significant cause of parental stress. Parents have reported a perceived lack of accessible feeding information for infants with CHD. To address this gap, the aim of this study was to develop feeding information to better support parents.

A search for existing material on six electronic databases and an internet search for unpublished (grey) literature on feeding information for infants with CHD were carried out. Following the development of feeding information, semi-structured interview(s) with parents/health-care professionals were completed, focusing on whether the information was easy to understand, relevant, provided sufficient information around feeding/feeding difficulties, and whether there were any information gaps. Iterative changes were made to the information following each interview. The process was completed until thematic saturation was achieved.

A total of 23 unique articles were identified of which 5 studies were included. From the grey literature, four web pages were reviewed. A total of 22 parents and 25 health-care professionals were interviewed. All parents/health-care professionals felt that the feeding information developed provided sufficient information; however, many wanted information on how to introduce complementary food, particularly if weaning was delayed.

This study describes the development of feeding information for infants with CHD. From parent interviews, gaps identified focused on the introduction of complementary foods and uncertainty regarding the feeding journey beyond surgery.

Children with congenital heart disease and their families are at risk of psychosocial problems. Emotional and behavioural problems, impaired school functioning, and reduced exercise capacity often occur. To prevent and decrease these problems, we modified and extended the previously established Congenital Heart Disease Intervention Program (CHIP)–School, thereby creating CHIP-Family. CHIP-Family is the first psychosocial intervention with a module for children with congenital heart disease. Through a randomised controlled trial, we examined the effectiveness of CHIP-Family.

Ninety-three children with congenital heart disease (age M = 5.34 years, SD = 1.27) were randomised to CHIP-Family (n = 49) or care as usual (no psychosocial care; n = 44). CHIP-Family consisted of a 1-day group workshop for parents, children, and siblings and an individual follow-up session for parents. CHIP-Family was delivered by psychologists, paediatric cardiologists, and physiotherapists. At baseline and 6-month follow-up, mothers, fathers, teachers, and the child completed questionnaires to assess psychosocial problems, school functioning, and sports enjoyment. Moreover, at 6-month follow-up, parents completed program satisfaction assessments.

Although small improvements in child outcomes were observed in the CHIP-Family group, no statistically significant differences were found between outcomes of the CHIP-Family and care-as-usual group. Mean parent satisfaction ratings ranged from 7.4 to 8.1 (range 0–10).

CHIP-Family yielded high program acceptability ratings. However, compared to care as usual, CHIP-Family did not find the same extent of statistically significant outcomes as CHIP-School. Replication of promising psychological interventions, and examination of when different outcomes are found, is recommended for refining interventions in the future.

Dutch Trial Registry number NTR6063, https://www.trialregister.nl/trial/5780.

This study evaluated cardiac function using tissue Doppler echocardiography and assessed electrocardiographic findings in children diagnosed with Wilson’s disease.

Asymptomatic patients with a diagnosis of Wilson’s disease (n = 43) were compared to healthy controls (n = 37) that were age and gender matched.

The standard electrocardiographic and conventional echocardiographic examinations were similar in both groups. The left ventricular ejection fraction, shortening fraction, and diastolic function were not significantly different between the two groups. The Tei index for mitral lateral, mitral septal, tricuspid lateral, tricuspid septal, and inter-ventricular septum on tissue Doppler echocardiography was higher in the patient group, yet it did not reach statistical significance. Mitral lateral and septal systolic annular velocity values were significantly lower in the patient group when compared to the control group (p = 0.02 and 0.04, respectively). Also, mitral lateral and septal isovolumetric contraction time values were higher in the patient group (p = 0.04). Although the left ventricular values were not significantly different, relative left ventricular wall thickness was higher in the patient group when compared to the control group, and concentric remodelling in the left ventricle was found in 7 (16%) of 42 patients. QT interval (p = 0.02) and P-wave dispersion values (p = 0.04) were significantly higher in the patient group compared to the control group, and these tend to predict arrhythmias.

Our study based on the tissue Doppler echocardiography assessment indicated a subclinical systolic, rather than diastolic, dysfunction in the myocardium with increased QT interval and P-wave dispersion, despite the young age of the patients and short disease duration.

There are several agents used for conscious sedation by various routes in children. The aim of this prospective randomised study is to compare the effectiveness of three commonly used sedatives: intranasal ketamine, intranasal midazolam, and oral chloral hydrate for children undergoing transthoracic echocardiography.

Children who were referred to paediatric cardiology due to a heart murmur for transthoracic echocardiography were prospectively randomised into three groups. Seventy-three children received intranasal midazolam (0.2 mg/kg), 72 children received intranasal ketamine (4 mg/kg), and 72 children received oral chloral hydrate (50 mg/kg) for conscious sedation. The effects of three agents were evaluated in terms of intensity, onset, and duration of sedation. Obtaining high-quality transthoracic echocardiography images (i.e. absence of artefacts) were regarded as successful sedation. Side effects due to medications were also noted.

There was no statistical difference in terms of sedation success rates between three groups (95.9, 95.9, and 94.5%, respectively). The median onset of sedation in the midazolam, ketamine, and chloral hydrate was 14 minutes (range 7–65), 34 minutes (range 12–56), and 40 minutes (range 25–57), respectively (p < 0.001 for all). However, the median duration of sedation in study groups was 68 minutes (range 20–75), 55 minutes (range 25–75), and 61 minutes (range 34–78), respectively (p = 0.023, 0.712, and 0.045). Gastrointestinal side effects such as nausea and vomiting were significantly higher in the chloral hydrate group (11.7 versus 0% for midazolam and 2.8% for ketamine, respectively, p = 0.002).

Results of our prospectively randomised study indicate that all three agents provide adequate sedation for successful transthoracic echocardiography. When compared the three sedatives, intranasal midazolam has a more rapid onset of sedation while intranasal ketamine has a shorter duration of sedation. Intranasal ketamine can be used safely with fewer side effects in children undergoing transthoracic echocardiography.

In this study, we applied ultra-short time series of interbeat intervals (RR-intervals) to evaluate heart rate variability through default chaotic global techniques with the purpose of discriminating obese youths from non-obese youth patients.

Chaotic global analysis of the RR-intervals from the electrocardiogram and pre-processing adjustments was undertaken. The effect of cubic spline interpolations was assessed, while the spectral parameters remained fixed. Exactly, 125 RR-intervals of data were recorded.

CFP1, CFP3, and CFP6 were the only significant combinations of chaotic globals when the standard conditions were enforced and at the level p<0.01 (or <1%). These significances were acheived via Kruskal–Wallis and Cohen’s ds effects sizes tests of significance after Anderson–Darling and Lilliefors statistical tests indicated non-normal distributions in the majority of cases. Adjustments of the cubic spline interpolation from 1 to 13 Hz were revealed to be inconsequential when measured by Kruskal–Wallis and Cohen’s ds, regarding the outcome between the two datasets.

Chaotic global analysis was offered as a robust technique to distinguish autonomic dysfunction in obese youths. It can discriminate the two different groups using ultra-short data lengths, and no cubic spline interpolations need be applied.