Haemodynamically unstable patients can experience potentially hazardous changes in vital signs related to the exchange of depleted syringes of epinephrine to full syringes. The purpose was to determine the measured effects of epinephrine syringe exchanges on the magnitude, duration, and frequency of haemodynamic disturbances in the hour after an exchange event (study) relative to the hours before (control).

Beat-to-beat vital signs recorded every 2 seconds from bedside monitors for patients admitted to the paediatric cardiovascular ICU of Texas Children’s Hospital were collected between 1 January, 2013 and 30 June, 2015. Epinephrine syringe exchanges without dose/flow change were obtained from electronic records. Time, magnitude, and duration of changes in systolic blood pressure and heart rate were characterised using Matlab. Significant haemodynamic events were identified and compared with control data.

In all, 1042 syringe exchange events were found and 850 (81.6%) had uncorrupted data for analysis. A total of 744 (87.5%) exchanges had at least 1 associated haemodynamic perturbation including 2958 systolic blood pressure and 1747 heart-rate changes. Heart-rate perturbations occurred 37% before exchange and 63% after exchange, and 37% of systolic blood pressure perturbations happened before syringe exchange, whereas 63% occurred after syringe exchange with significant differences found in systolic blood pressure frequency (p<0.001), duration (p<0.001), and amplitude (p<0.001) compared with control data.

This novel data collection and signal processing analysis showed a significant increase in frequency, duration, and magnitude of systolic blood pressure perturbations surrounding epinephrine syringe exchange events.

Although interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative, variation across centres persists. It remains unclear whether centres with lower interstage mortality have lower-risk patients or whether differences in care may explain this variation. We examined previously established risk factors across National Pediatric Cardiology Quality Improvement Collaborative centres with lower and higher interstage mortality rates.

Lower-mortality centres were defined as those with >25 consecutive interstage survivors. Higher-mortality centres were defined as those with cumulative interstage mortality rates >10%, which is a collaborative historic baseline rate. Baseline risk factors and perioperative characteristics were compared.

Seven lower-mortality centres were identified (n=331 patients) and had an interstage mortality rate of 2.7%, as compared with 13.3% in the four higher-mortality centres (n=173 patients, p<0.0001). Of all baseline risk factors examined, the only factor that differed between the lower- and higher-mortality centres was postnatal diagnosis (18.4 versus 31.8%, p=0.001). In multivariable analysis, there remained a significant mortality difference between the two groups of centres after adjusting for this variable: adjusted mortality rate was 2.8% in lower-mortality centres compared with 12.6% in higher-mortality centres, p=0.003. Secondary analyses identified multiple differences between groups in perioperative practices and other variables.

Variation in interstage mortality rates between these two groups of centres does not appear to be explained by differences in baseline risk factors. Further study is necessary to evaluate variation in care practices to identify targets for improvement efforts.

The aim of this study was to explore perinatal and early postnatal outcomes in fetuses with prenatally diagnosed d-transposition of the great arteries and impacts of standardised prenatal consultation.

All fetuses with prenatally diagnosed d-transposition of the great arteries prospectively enrolled at South China cardiac centre from 2011 to 2015. Standardised prenatal consultation was introduced in 2013 and comprehensive measures were implemented, such as establishing fetal CHD Outpatient Consultation Service, performing standard prenatal consultation according to specifications, and establishing a multidisciplinary team with senior specialists performing in-person consultations. Continuous follow-up investigation was conducted. Perinatal and postnatal outcomes were compared before and after consultation including live birth, elective termination of pregnancy, spontaneous fetal death, stillbirths, referral for surgery, and survival.

In all, 146 fetuses were enrolled with 41 (28%) lost to follow-up. Among 105 remaining fetuses, 29 (28%) were live births and 76 (72%) were terminated. After consultation, live birth rate was higher (50 versus 33%) and termination rate was lower (50 versus 76%), although there was no statistical significance. Excluding three live births without postnatal d-transposition of the great arteries, 65% (17/26) underwent arterial switch operation within 30 days. A total of three in-hospital deaths occurred and during the 10-month follow-up period, one death was observed. In one case, the switch procedure was performed at 13 months and the infant survived. Out of eight infants without arterial switch operation, two died.

Live birth rate increased after consultation; however, termination remained high. Combining termination, patients without arterial switch operation, and operative mortality, outcomes of d-transposition of the great arteries infants can be improved. Standard consultation, multidisciplinary collaboration, and improved perinatal care are important to improve outcomes.

Owing to massive improvements in the diagnostics and surgery of children with CHD, fatality has decreased substantially. As more children with CHD survive from infancy into later childhood, more will need medication for chronic heart failure. However, surprisingly little is actually known about which drugs are being used to treat children with CHD, and whether prescription rates and CHD prevalence have changed over time.

The objective of this study was to assess the total prescription of cardiovascular drugs to children during an 18-year period and to assess concomitant CHD prevalence.

All prescription data of cardiovascular drugs to children aged 0–19 years were extracted from publicly available databases in Norway and Denmark from 1999 to 2016. This was coupled with data on CHD prevalence and birth rates.

The number of defined daily doses of cardiovascular drugs prescribed to children doubled in the study period. This was because of an increased use of beta blockers, angiotensin-converting-enzyme inhibitors/angiotensin receptor blockers, and anti-arrhythmic agents. The use of some classes of drugs was significantly reduced over time. The prevalence of CHD remained constant in both countries – 80 per 10,000 births.

We show that there is an increase in the overall prescription of cardiovascular drugs to children. Beta blockers, angiotensin-converting enzyme/angiotensin receptor blockers, and anti-arrhythmics account for the largest increase. Birth rates decreased or remained constant together with CHD prevalence, suggesting that the increased use of cardiovascular drugs reflected increased prescription per patient, rather than more patients receiving a constant amount of drugs.

This study aimed to develop a method for retrieval of the new meshed nitinol atrial septal defect occluders – Ceraflex and Occlutech.

The newly developed atrial septal defect occluders have potential benefits considering implantation, but concerns regarding their removal in case of embolisation have been raised.

Over 21 years, 1449 patients underwent interventional atrial septal defect occlusion in our institution. We reviewed the cases of embolisation of the device, developed a strategy for device removal, and tested it on the benchside and in animal tests.

In 11 patients (0.8%), the intended atrial septal defect occlusion was complicated by an embolisation of the device. In contrast to the Amplatzer septal occluders, retrieval of Occlutech devices larger than 16 mm with snare techniques was impossible. In benchside tests, this was confirmed and a new method for removal of large meshed devices was developed. This involved the commercially available Maslanka® biopsy forceps. The feasibility of this technique in vivo was tested in a pig model. During animal tests, using the Maslanka biopsy forceps it was possible to interventionally retrieve embolised Ceraflex and Occlutech devices of different sizes – 10, 16, 30, and 40 mm – into a 12-F sheath.

It was impossible to retrieve Occlutech and Ceraflex devices larger than 16 mm into a large sheath in vivo and during benchside tests. However, this was feasible on the bench and in vivo using the Maslanka biopsy forceps even with the largest available devices.

Recently, mean platelet volume-to-lymphocyte ratio has emerged as a novel parameter of inflammation. No study has investigated the role of mean platelet volume-to-lymphocyte ratio in children with Kawasaki disease. We aimed to evaluate the relationship between mean platelet volume-to-lymphocyte ratio and coronary artery abnormalities in Kawasaki disease.

Between January 2008 and January 2017, a total of 58 children with Kawasaki disease and 42 healthy subjects matched for sex and age were enrolled. Before the treatment, transthoracic echocardiography for all children was performed. Clinical and laboratory results including mean platelet volume, platelet distribution width, red blood cell distribution width, and counts of platelets, neutrophils, lymphocytes, and white blood cells, erythrocyte sedimentation rate, and C-reactive protein levels were measured. Mean platelet volume-to-lymphocyte ratio was calculated as mean platelet volume divided by lymphocyte count.

Compared with healthy controls, mean platelet volume-to-lymphocyte ratio was significantly lower in the children with Kawasaki disease (p<0.01). A total of 14 patients (24.1%) had incomplete Kawasaki disease and 15 (25.8%) children with Kawasaki disease had coronary involvement. Mean platelet volume-to-lymphocyte ratio was significantly lower in patients with coronary artery abnormalities (p<0.01). According to receiver operating characteristic curve analysis performed for the prediction of coronary artery abnormalities, the best cut-off point for mean platelet volume-to-lymphocyte ratio was 2.5 (area under curve=0.593, sensitivity 53.3%, specificity 51.1%).

It was first shown that the children with Kawasaki disease have lower mean platelet volume-to-lymphocyte ratio compared with control subjects. Mean platelet volume-to-lymphocyte ratio may be helpful in predicting coronary artery lesions in patients with Kawasaki disease.

Enlargement of the left atrium is a non-invasive marker of diastolic dysfunction of the left ventricle, a determinant of prognosis in children with cardiomyopathy. Similarly, N-terminal prohormone brain natriuretic peptide is a useful marker in the management of children with cardiomyopathy and heart failure. The aim of this study is to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy.

This was a retrospective study reviewing the medical records of patients <18 years of age, who were diagnosed with cardiomyopathy or acute myocarditis with eventual development of cardiomyopathy. Left atrial volume by transthoracic echocardiogram and pulmonary capillary wedge pressure, a surrogate of left atrial pressure, obtained by means of cardiac catheterisation were analysed. In addition, N-terminal prohormone brain natriuretic peptide levels obtained at the time of the cardiac catheterisation were also reviewed. Statistical analysis was performed to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide levels.

There was a linear correlation of left atrial pressure estimated in the cardiac catheterisation with indexed left atrial volume (r=0.63; p<0.001) and left atrial volume z-scores (r=0.59; p<0.001). We found no statistically significant association between the left atrial pressure and N-terminal prohormone brain natriuretic peptide levels.

Left atrial volume measured non-invasively by echocardiography can be used as a surrogate for left atrial pressure in assessing diastolic dysfunction of the left ventricle in children with cardiomyopathy. The larger the size of the left atrium, worse is the diastolic function of the left ventricle.

Optimal management strategy for native aortic coarctation in neonates and young infants is still a matter of debate. The surgical procedure, histopathologic research, and clinical outcome in 15 neonates who underwent surgery after successful balloon angioplasty is the basis of this study.

Between 01 October, 2014 and 01 August, 2017, we enrolled 15 patients with native aortic coarctation for this study. These patients had complications regarding recoarctation, following balloon angioplasty intervention at our institute and other centres. Surgically extracted parts were examined histopathologically and patient’s data were collected retrospectively.

The reasons for recurrence of recoarctation after balloon angioplasty are as follows: patients with higher preoperative echocardiographic gradients had recoarctation earlier, neointimal proliferation, aortic intimal fibrosis at the region of ductal insertion, and ductal residual tissue debris after balloon angioplasty. No repeat intervention was required in the 15 patients who underwent surgery followed by balloon angioplasty. Early mortality was seen in one patient after surgery. Postoperative complication in the surgical group occurred in the form of chylothorax in one patient.

In centres in which the neonatal ICU is inexperienced, balloon angioplasty is particularly recommended. In developing neonatal clinics, balloon angioplasty, when performed on patients at their earliest possible age, delays actual corrective operation to a later date, which in turn provides less risky surgical outcomes in infants who are gaining weight, growing, and do not have any haemodynamic complaints.

The objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.

Adult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient.

This is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6–12 months after procedures.

There was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70–85%) and post-procedure mean was 83 (SD 2.52)% (range 78–87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06–20.28 g/L 6 months post procedure (range 18.1–24.4 to 13–23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2–2.2 post procedure. Left atrial volume for four of the cases did not change (22.6–76.6 ml [mean 48.4 ml] to 29.6–72.9 ml [mean 52 ml], p value: 0.83).

Stenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans.

The aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection.

We conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, <6 months (n=33), and older group, >6 months (n=67).

The proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044).

Early bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.

We propose a new dynamic flow imaging using 320-detector row CT, and investigate the assessment of coronary flow in aneurysms of Kawasaki disease in adulthood.

Six patients with Kawasaki disease and coronary aneurysms associated (26.7 years old) and six controls were enrolled. Dynamic coronary CT angiography with 320-row CT was continuously performed at mid-diastole throughout 15–25 cardiac cycles with prospective Electrocardiogram gating after injection of contrast media. Dynamic data sets of 15–25 cycles were computed into 90–100 data sets by motion coherence image processing. Next, time–density curves for coronary arteries were calculated for all the phases. On the basis of the maximum slope method, coronary flow index was defined as the ratio of the maximum upslope of the attenuation of coronary arteries to the upslope of the attenuation of ascending aorta on the time–density curves. Coronary flow indexes for the proximal and distal sites of coronary arteries and intra-aneurysm were measured.

The aims of this study were to investigate risk factors for the development of postoperative chylothorax following paediatric congenital heart surgery and to investigate the impact of a management guideline on management strategies and patient outcome.

All patients with chylothorax following cardiac surgery at the Royal Children’s Hospital, Melbourne, over a 48-month period beginning in January 2008 were identified. A control group, matched for age, date of surgery, and sex, was identified. To investigate potential risk factors, univariable and multivariable logistic regression models were constructed with paired analysis. To examine the effect of a standardised management protocol, data before and after the implementation of the guideline were compared.

In total, 121 cases of chylothorax were identified, with 121 controls, matched for age at surgery, date of surgery, and sex. The incidence of chylothorax was 5.23%. Increasing surgical complexity (univariable OR 0.17 for the least complex versus the most complex group, p=0.02), closed-heart surgeries (OR 0.07 for open versus closed, p<0.001), and redo chest incisions (OR 10.0 for redo versus virgin, p<0.001) were significantly associated with chylothorax. The standardised management protocol had no significant impact on either drainage duration or management strategy.

We have replicated the previously reported association between surgical complexity and chylothorax risk, and have shown, for the first time, that redo chest openings are also associated with a significantly increased risk. The implementation of a standardised management protocol in our institution did not result in a significant change in either chylothorax drainage duration or management strategy.

We investigated dimensions and elasticity of whole aorta in patients with bicuspid aortic valve and influence of valve phenotype.

The study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal – fusion between right and left coronary cusps – and vertical – fusion between right–non-coronary cusps; according to age they were divided into younger (5–10 years) and older patients (11–16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic–diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending–descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied.

z-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn−1.10−6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group.

Aortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.

SCN5A encodes sodium-channel α-subunit Nav1.5. The mutations of SCN5A can lead to hereditary cardiac arrhythmias such as the long-QT syndrome type 3 and Brugada syndrome. Here we sought to identify novel mutations in a family with arrhythmia.

Genomic DNA was isolated from blood of the proband, who was diagnosed with atrial flutter. Illumina Hiseq 2000 whole-exome sequencing was performed and an arrhythmia-related gene-filtering strategy was used to analyse the pathogenic genes. Sanger sequencing was applied to verify the mutation co-segregated in the family.

A novel missense mutation in SCN5A (C335R) was identified, and this mutation co-segregated within the affected family members. This missense mutation was predicted to result in amplitude reduction in peak Na+ current, further leading to channel protein dysfunction. Our study expands the spectrum of SCN5A mutations and contributes to genetic counselling of families with arrhythmia.

The objective of this study was to prospectively validate the “Brief Developmental Assessment”, which is a new early recognition tool for neurodevelopmental abnormalities in children with heart disease that was developed for use by cardiac teams.

This was a prospective validation study among a representative sample of 960 pre-school children with heart disease from three United Kingdom tertiary cardiac centres who were analysed grouped into five separate age bands.

The “Brief Developmental Assessment” was successfully validated in the older four age bands, but not in the youngest representing infants under the age of 4 months, as pre-set validation thresholds were met – lower 95% confidence limit for the correlation coefficient above 0.75 – in terms of agreement of scores between two raters and with an external measure the “Mullen Scales of Early Learning”. On the basis of American Association of Pediatrics Guidelines, which state that the sensitivity and specificity of a developmental screening tool should fall between 70 and 80%, “Brief Developmental Assessment” outcome of Red meets this threshold for detection of Mullen scores >2 standard deviations below the mean.

The “Brief Developmental Assessment” may be used to improve the quality of assessment of children with heart disease. This will require a training package for users and a guide to action for abnormal results. Further research is needed to determine how best to deploy the “Brief Developmental Assessment” at different time points in children with heart disease and to determine the management strategy in infants younger than 4 months old.