Multicentre research databases can provide insights into healthcare processes to improve outcomes and make practice recommendations for novel approaches. Effective audits can establish a framework for reporting research efforts, ensuring accurate reporting, and spearheading quality improvement. Although a variety of data auditing models and standards exist, barriers to effective auditing including costs, regulatory requirements, travel, and design complexity must be considered.

The Congenital Cardiac Research Collaborative conducted a virtual data training initiative and remote source data verification audit on a retrospective multicentre dataset. CCRC investigators across nine institutions were trained to extract and enter data into a robust dataset on patients with tetralogy of Fallot who required neonatal intervention. Centres provided de-identified source files for a randomised 10% patient sample audit. Key auditing variables, discrepancy types, and severity levels were analysed across two study groups, primary repair and staged repair.

Of the total 572 study patients, data from 58 patients (31 staged repairs and 27 primary repairs) were source data verified. Amongst the 1790 variables audited, 45 discrepancies were discovered, resulting in an overall accuracy rate of 97.5%. High accuracy rates were consistent across all CCRC institutions ranging from 94.6% to 99.4% and were reported for both minor (1.5%) and major discrepancies type classifications (1.1%).

Findings indicate that implementing a virtual multicentre training initiative and remote source data verification audit can identify data quality concerns and produce a reliable, high-quality dataset. Remote auditing capacity is especially important during the current COVID-19 pandemic.

Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology.

Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established.

Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups.

In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups.

While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.

Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely identified.

All patients who underwent bidirectional Glenn operation operation at our centre between January 2007 and March 2017 were retrospectively identified and patient variables were extracted. For patients who had undergone Fontan operation at the time of this study, post-Fontan patient variables were also extracted.

One-hundred patients were included with a median age at pre-bidirectional Glenn operation catheterisation of 4.5 months. In total, 71 (71%) patients had a systemic right ventricle. At the pre-bidirectional Glenn operation catheterisation, the mean systemic ventricular end-diastolic pressure was higher amongst those with systemic right ventricle compared to left ventricle (9.1 mmHg ± 2.1 versus 7.7 ± 2.7 mmHg, p < 0.01). On univariate analysis, pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01), history of recoarctation (p = 0.03), history of Norwood operation (p = 0.04), and ventricular systolic pressure (p < 0.01). On multivariate analysis, systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01) and ventricular systolic pressure (p < 0.01). Amongst those who had undergone Fontan operation at the time of study (n = 49), those with a higher pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure were more likely to have experienced death, transplantation, or listed for transplantation (p = 0.02) and more likely to have had heart failure symptoms (p = 0.04) at a mean time from Fontan of 5.2 years ± 1.3.

In patients undergoing bidirectional Glenn operation operation, the volume-loaded, pre-bidirectional Glenn operation state may expose diastolic dysfunction that has prognostic value.

Arrhythmias are common in the post-operative course of patients with hypoplastic left heart syndrome. We sought to determine the types, incidence, risk factors, and impact of arrhythmias in patients with HLHS and anatomic variants.

We performed a retrospective chart review of 120 consecutive patients with HLHS and anatomical variants, who had single-ventricle palliation at our institution from January, 2006 to December, 2016.

A total of thirty-one patients (26%) had 37 episodes of arrhythmias over a median follow-up period of 3.5 years. Of the 37 episodes, 12 (32.4%) were ectopic atrial tachycardia, 9 (24.3%) were paroxysmal supraventricular tachycardia, 4 (10.8%) were junctional ectopic tachycardia, 5 (13.6%) were sinus node dysfunction, 3 (8.1%) were heart block, 2 (5.4%) were atrial flutter, and 2 (5.4%) were ventricular tachycardia. Twenty-four (65%) of the arrhythmias occurred at post-stage 1 surgery. Most (64.8%) of the arrhythmias were resolved. Arrhythmias that occurred at post-stage 1 surgery were more likely to resolve compared to post-stages 2 or 3 (p = 0.006). No anatomical, surgical, or clinical variables were associated with arrhythmia except for age (OR per unit decrease in age at stage 1 palliation: 1.12 (95% CI 1.003, 1.250); p = 0.0439). Arrhythmias were not associated with length of hospital stay or mortality.

Arrhythmias are common in patients with HLHS and anatomic variants, with EAT and PSVT being the most common types. Arrhythmias were associated with younger age at surgery, but did not affect mortality or length of hospital stay.

Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.

Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.

Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.

NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.

Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences.

This is a 30-year (1988–2018) retrospective study of patients with pulmonary atresia intact ventricular septum.

Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock–Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001).

Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

The aim of this study was to evaluate the safety and efficacy of the Cardi-O-Fix plug used for the treatment of muscular ventricular septal defects.

We retrospectively reviewed the medical records of five patients with muscular ventricular septal defects who underwent transcatheter closure using the Cardi-O-Fix Plug, from November 2017 to August 2019. The median age was 5.1 years (range: 3.2–6.5). Their median body weight was 18.1 kg (range: 13.4–21.8). All the patients underwent detailed two-dimensional Doppler and colour flow imaging by transthoracic echocardiography. The left ventricular median defect size of the muscular ventricular septal defects was 5.6 mm (range: 5.3–7.0). The right ventricular median defect size of the muscular ventricular septal defects was 3.9 mm (range: 3.3–4.7). All the procedures were performed on beating hearts.

All the patients underwent successful device implantation with no displacement or detachment, they have complete echocardiographic closure at the 1-year follow-up. There were no occluder-related arrhythmia, chordae tendineae rupture, tricuspid insufficiency, aortic regurgitation, haemolysis, or embolisation.

Application of the Cardi-O-Fix plug appears to be a feasible, safe, and effective treatment option for patients with muscular ventricular septal defects. Longer follow-up periods are warranted to prove the conclusion for long-term outcomes.

Patent ductus arteriosus stenting in duct-dependent pulmonary circulation is a challenging procedure. Percutaneous carotid artery access for ductal stenting has proven to be feasible; however, comparison with femoral artery access in terms of procedure details and complications either immediate or late is scarce. Therefore, we evaluated carotid artery access in comparison with femoral artery for stenting of patent ductus arteriosus.

Forty neonates were reviewed, 20 were stented via carotid artery access, and 20 via the traditional femoral artery access. Comparison variables were neonatal demographics at the procedure, angiographic ductal anatomy, procedure details, and immediate complications. Follow-up Doppler ultrasound on access site was performed to document late complications.

Median age of included cases was 10.5 (3–28) days with complex ductal anatomy more frequently accessed via carotid artery than femoral. Immediate access-related complications were significantly higher with femoral than carotid artery access; 9 (45%) versus 3 (15%) respectively, p = 0.038. With carotid access, we had only one case with small pseudoaneurysm and acute hemiparesis 3 days after the procedure. Delayed local complications were more common with femoral access (15%) than carotid access (5%), mild stenosis in one case, and severe in another with femoral access; while with transcarotid arterial access, only one case had mild narrowing.

Percutaneous carotid artery access in neonates is a more convenient approach for patent ductus arteriosus stenting especially with complex ductal anatomy. Moreover, local complications are limited and vascular patency is better preserved, in comparison with trans-femoral arterial access. However, the potential for neurological adverse events should not be overlooked.

Three-dimensional printing is increasingly utilised for congenital heart defect procedural planning. CT or MR datasets are typically used for printing, but similar datasets can be obtained from three-dimensional rotational angiography. We sought to assess the feasibility and accuracy of printing three-dimensional models of CHD from rotational angiography datasets.

Retrospective review of CHD catheterisations using rotational angiography was performed, and patient and procedural details were collected. Imaging data from rotational angiography were segmented, cleaned, and printed with polylactic acid on a Dremel® 3D Idea Builder (Dremel, Mount Prospect, IL, USA). Printing time and materials’ costs were captured. CT scans of printed models were compared objectively to the original virtual models. Two independent, non-interventional paediatric cardiologists provided subjective ratings of the quality and accuracy of the printed models.

Rotational angiography data from 15 catheterisations on vascular structures were printed. Median print time was 3.83 hours, and material costs were $2.84. The CT scans of the printed models highly matched with the original digital models (root mean square for Hausdorff distance 0.013 ± 0.003 mesh units). Independent reviewers correctly described 80 and 87% of the models (p = 0.334) and reported high quality and accuracy (5 versus 5, p = NS; κ = 0.615).

Imaging data from rotational angiography can be converted into accurate three-dimensional-printed models of CHD. The cost of printing the models was negligible, but the print time was prohibitive for real-time use. As the speed of three-dimensional printing technology increases, novel future applications may allow for printing patient-specific devices based on rotational angiography datasets.

To evaluate the reliability of balloon coronary compression testing during percutaneous pulmonary valve implantation.

Despite the widespread use of the ‘balloon coronary test’ as the preferable method to rule out the risk of coronary compression, this adverse event has been described after pulmonary valve implantation where coronary balloon test suggested no risk or low risk, calling into question the accuracy of the test.

We performed a retrospective chart review of 84 patients who underwent pulmonary valve implantation between January 2018 and December 2019 and selected 36 patients whose archived imaging was suitable to perform quantitative analysis of the ‘balloon coronary test’. We focused on the spatial disparity between the right ventricular outflow tract position defined by the inflated testing balloon and the eventual implanted valve position, to classify the test as inaccurate or accurate.

In total, 36.1% of cases were classified as having an inaccurate coronary balloon test. Among the baseline characteristics, right ventricular outflow tract substrate was identified as a significant predictor of test accuracy. Related to this characteristic, the type of testing balloon used and the size of the eventually implanted valve were found to be associated with test accuracy.

Based on our findings, balloon coronary testing is not an accurate method of predicting final valve position with respect to fixed structures in the thorax. This may translate to a high false positive rate for the likelihood of coronary compression in pulmonary valve implantation.

To assess the efficacy and safety of captopril, simvastatin, and L-carnitine as cardioprotective drugs in children with type 1 diabetes mellitus on different echocardiographic parameters, electrocardiographic parameter, lipid profile, and carotid intima–media thickness.

This randomised controlled trial was conducted on 100 children with type 1 diabetes mellitus for more than 3 years during the period from September 2018 to June 2020. Fifty healthy children of matched age and sex served as a control group. The patients were randomly assigned into four groups (25 children each): no-treatment group who received no cardioprotective drug, simvastatin group who received simvastatin (10–20 mg/day), captopril group who received captopril (0.2 mg/kg/day), and L-carnitine group who received L-carnitine (50 mg/kg/day) for 4 months. Lipid profile, serum troponin I, carotid intima–media thickness, and echocardiographic examinations were performed on all included children before and after the treatment.

Total cholesterol and low-density lipoprotein were significantly decreased in children who received simvastatin or L-carnitine. Triglycerides significantly decreased only in children who received simvastatin. High-density lipoprotein significantly increased in simvastatin and L-carnitine groups only. Serum troponin I decreased significantly in all the three treatment groups. Carotid intima–media thickness showed no significant change in all treatment groups. Echocardiographic parameters significantly improved in simvastatin, L-carnitine, and captopril groups.

Captopril, simvastatin, and L-carnitine have a significant beneficial effect on cardiac functions in children with type 1 diabetes mellitus. However, only simvastatin and L-carnitine have a beneficial effect on the lipid profile. The drugs were safe and well tolerated.

Clinical trial registration: The clinical trial was registered at www.clinicaltrial.gov (NCT03660293).