COVID-19 has markedly impacted the provision of neurodevelopmental care. In response, the Cardiac Neurodevelopmental Outcome Collaborative established a Task Force to assess the telehealth practices of cardiac neurodevelopmental programmes during COVID-19, including adaptation of services, test protocols and interventions, and perceived obstacles, disparities, successes, and training needs.

A 47-item online survey was sent to 42 Cardiac Neurodevelopmental Outcome Collaborative member sites across North America within a 3-week timeframe (22 July to 11 August 2020) to collect cross-sectional data on practices.

Of the 30 participating sites (71.4% response rate), all were providing at least some clinical services at the time of the survey and 24 sites (80%) reported using telehealth. All but one of these sites were offering new telehealth services in response to COVID-19, with the most striking change being the capacity to offer new intervention services for children and their caregivers. Only a third of sites were able to carry out standardised, performance-based, neurodevelopmental testing with children and adolescents using telehealth, and none had completed comparable testing with infants and toddlers. Barriers associated with language, child ability, and access to technology were identified as contributing to disparities in telehealth access.

Telehealth has enabled continuation of at least some cardiac neurodevelopmental services during COVID-19, despite the challenges experienced by providers, children, families, and health systems. The Cardiac Neurodevelopmental Outcome Collaborative provides a unique platform for sharing challenges and successes across sites, as we continue to shape an evidence-based, efficient, and consistent approach to the care of individuals with CHD.

The fate of right ventricular dimensions after surgical closure of secundum-type atrial septal defects remains unclear. The objectives of this study were to assess ventricular dimensions, exercise capability, and arrhythmias of patients operated for secundum-type atrial septal defect and compare the results with those in healthy references.

A total of 78 consecutive patients underwent surgical closure for a secundum-type atrial septal defect between 1990 and 1995. In all, 42 patients were included and underwent a cross-sectional evaluation including echocardiography, magnetic resonance imaging, exercise testing, and 24-hour ambulatory electrocardiography. Patients were matched with healthy controls for gender, body surface area, and age.

The mean age at surgery was 4.6 plus or minus 2.8 years, and the mean age at follow-up was 16 plus or minus 3 years. There were no residual intracardiac lesions. The mean right ventricular end-systolic volume was significantly larger in patients (142 ± 26 millilitres) than in references (137 ± 28 millilitres; p = 0.04). In 25% of the patients, right ventricular end-systolic volume was larger than the 95th percentile for references. No relevant arrhythmias were detected. Exercise testing did not reveal differences with healthy references: maximal power (169 ± 43 Watt patients versus 172 ± 53 controls; p = 0.8), maximal oxygen uptake (38 ± 8 versus 41 ± 13 millilitres per minute per kilogram; p = 0.1).

After surgical closure of secundum-type atrial septal defect, right ventricular end-systolic volume is increased. These findings have no impact on rhythm status or exercise capacity at this stage of follow-up, but may have implications for the timing of surgery or the technique of closure if confirmed in longer follow-up.

The physiological importance of the lateral tunnel stenosis in the Fontan pathway for children with single ventricle physiology can be difficult to determine. The impact of the stenosis and stent implantation on total cavopulmonary connection resistance has not been characteriszed, and there are no clear guidelines for intervention.

A computational framework for haemodynamic assessment of stent implantation in patients with lateral tunnel stenosis was developed. Cardiac magnetic resonances images were reconstructed to obtain total cavopulmonary connection anatomies before stent implantation. Stents with 2-mm diameter increments were virtually implanted in each patient to understand the impact of stent diameter. Numerical simulations were performed in all geometries with patient-specific flow rates. Exercise conditions were simulated by doubling and tripling the lateral tunnel flow rate. The resulting total cavopulmonary connection vascular resistances were computed. A total of six patients (age: 14.4±3.1 years) with lateral tunnel stenosis were included for preliminary analysis. The mean baseline resistance was 1.54±1.08 WU·m2 and dependent on the stenosis diameter. It was further exacerbated during exercise. It was observed that utilising a stent with a larger diameter lowered the resistance, but the resistance reduction diminished at larger diameters.

Using a computational framework to assess the severity of lateral tunnel stenosis and the haemodynamic impact of stent implantation, it was observed that stenosis in the lateral tunnel pathway was associated with higher total cavopulmonary connection resistance than unobstructed pathways, which was exacerbated during exercise. Stent implantation could reduce the resistance, but the improvement was specific to the minimum diameter.

To improve the prenatal diagnosis for anomalous origin of pulmonary artery branches by comparing and analyzing different types of fetal echocardiography features.

Between June 2012 and December 2018, fetal echocardiographic features were analyzed retrospectively from fetuses with a prenatal diagnosis of anomalous origin of pulmonary artery branch. The main points of identification were summarized.

A total of 12 fetuses were diagnosed, including anomalous origin of a pulmonary artery branch from the innominate artery and six cases with unilateral absence of pulmonary artery. The shared characteristic sonographic finding was the lack of confluence at the bifurcation of the main pulmonary artery. The differences between the two conditions are highlighted by the origin of the anomalous vessel. In fetuses with anomalous origin of one pulmonary artery branch, the affected pulmonary artery arose from the posterior wall of the ascending aorta as noted on three vessels and trachea view as well as the long axis of the left ventricular outflow tract. This is in contrast to fetuses with unilateral absence of pulmonary artery, where the origin of affected pulmonary artery arises from the base of the innominate artery via the ipsilateral patent arterial duct as evident on the three vessels and trachea view and the coronal view of innominate (brachiocephalic) artery.

(1) The main similarity is an absence of a confluence at the bifurcation of the main pulmonary artery. (2) The main distinguishing feature is the origin of the anomalous vessel from either the subclavian or directly from the aorta.

To investigate the feasibility of critical congenital heart disease (CCHD) screening test by pulse oximetry in four geographical regions of Turkey with different altitudes, before implementation of a nationwide screening program.

It was a prospective multi-centre study performed in four centres, between December, 2015 and May, 2017. Pre- and post-ductal oxygen saturations and perfusion indices (PI) were measured using Masimo Radical-7 at early postnatal days. The results were evaluated according to the algorithm recommended by the American Academy of Pediatrics. Additionally, a PI value <0.7 was accepted to be significant.

In 4888 newborns, the mean screening time was 31.5 ± 12.1 hours. At first attempt, the mean values of pre- and post-ductal measurements were: saturation 97.3 ± 1.8%, PI 2.8 ± 2.0, versus saturation 97.7 ± 1.8%, PI 2.3±1.3, respectively. Pre-ductal saturations and PI and post-ductal saturations were the lowest in Centre 4 with the highest altitude. Overall test positivity rate was 0.85% (n = 42). CCHD was detected in six babies (0.12%). Of them, right hand (91 ± 6.3) and foot saturations (92.1 ± 4.3%) were lower compared to ones with non-CCHD and normal variants (p <0.05, for all comparisons). Sensitivity, specificity, positive and negative predictive values, and likelihood ratio of the test were: 83.3%, 99.9%, 11.9%, 99.9%, and 99.2%, respectively.

This study concluded that pulse oximetry screening is an effective screening tool for congenital heart disease in newborns at different altitudes. We support the implementation of a national screening program with consideration of altitude differences for our country.

Significant inter-centre variability in the intensity of endomyocardial biopsy surveillance for rejection following paediatric cardiac transplantation has been reported. Our aim was to determine if low-intensity biopsy surveillance with two scheduled biopsies in the first year would produce outcomes similar to published registry outcomes.

A retrospective study of paediatric recipients transplanted between 2008 and 2014 using a low-intensity biopsy protocol consisting of two surveillance biopsies at 3 and 12–13 months in the first post-transplant year, then annually thereafter. Additional biopsies were performed based on echocardiographic and clinical surveillance. Excluded were recipients that were re-transplanted or multi-organ transplanted or were followed at another institution.

A total of 81 recipients in the first 13 months after transplant underwent an average of 2 (SD ± 1.3) biopsies, 24 ± 6.8 echocardiograms, and 17 ± 4.4 clinic visits per recipient. During the 13-month period, 19 recipients had 24 treated rejection episodes, with the first at an average of 2.8 months post-transplant. The 3-, 12-, 36-, and 60-month conditional on discharge graft survival were 100%, 98.8%, 98.8%, and 90.4%, respectively, comparable to reported figures in major paediatric registries. At a mean follow-up of 4.7 ± 2.1 years, four patients (4.9%) developed cardiac allograft vasculopathy, three (3.7%) developed a malignancy, and seven (8.6%) suffered graft loss.

Rejection surveillance with a low-intensity biopsy protocol demonstrated similar intermediate-term outcomes and safety measures as international registries up to 5 years post-transplant.

Some centres have proposed creating the bidirectional cavopulmonary anastomosis without cardiopulmonary bypass, while others continue to use deep hypothermic circulatory arrest. The purpose of this review is to evaluate the results of using continuous cardiopulmonary bypass with moderate hypothermia, perhaps the most commonly used of the three techniques for this procedure.

Between 1990 and 2005, 114 patients, having a mean age of 1.58 years, with a median age of 8 months, and ranging from 3 months to 16 years, underwent creation of either a unilateral cavopulmonary anastomosis, in 94 cases, or bilateral anastomoses in 20 cases. All had continuous cardiopulmonary bypass with moderate hypothermia at 32 degrees Celsius, with 24 also having aortic cross-clamping with cardioplegia for simultaneous intracardiac procedures. Interrupted absorbable sutures were used to create the anastomosis in 105 patients.

Perioperative mortality was 5%, with 6 of the patients dying. The mean period of cardiopulmonary bypass for an isolated anastomosis was 91 minutes, with a range from 44 to 160 minutes. In 10 patients (8.8%), it was necessary to place a graft to augment the anastomosis. The average postoperative length of stay was 7.9 days for those undergoing an isolated unilateral anastomosis, and 16.4 days for patients undergoing combined cardiac operations. We have now created the Fontan circulation in 79 of the patients, at an average interval from the bidirectional cavopulmonary anastomosis of 2.1 plus or minus 1.14 years. In 76 patients, we performed postoperative angiograms, and none revealed any stenoses.

The bidirectional cavopulmonary anastomosis can be performed successfully with continuous cardiopulmonary bypass and moderate hypothermia with a beating heart, avoiding circulatory arrest. The use of interrupted and absorbable sutures was not associated with any late anastomotic stenosis.

Reduced long-axis shortening despite enhanced global function has been reported in aortic stenosis. We sought to improve the understanding of this phenomenon using multi-dimensional strain analysis in conjunction with the evaluation of left ventricular rotation and twist – ventricular torsion – using tissue Doppler techniques.

A total of 57 patients with variable severity of aortic stenosis, aortic regurgitation, or mixed aortic valve disease, subdivided into six groups, were studied. Ventricular morphology was assessed using long-axis/short-axis and mass/volume ratios, afterload using end-systolic meridional wall stress, and global performance using ejection fraction. The circumferential and longitudinal strain was measured from two-dimensional images, and left ventricular rotation and twist were estimated as the difference in rotation between the base and apex of the ventricle.

Aortic stenosis was associated with higher mass/volume, ejection fraction, circumferential strain and left ventricular rotation and twist, significantly lower end-systolic wall stress, and a trend towards lower longitudinal strain compared with normal. Myocardial mechanics in aortic regurgitation were normal despite ventricular dilation. Mixed aortic valve disease showed findings similar to aortic stenosis. Left ventricular rotation and twist correlated with midwall circumferential strain (r = 0.62 and p < 0.0001), endocardial circumferential strain (r = 0.61 and p < 0.0001), and end-systolic wall stress (r = 0.48 and p < 0.0001), but not with longitudinal strain (r = 0.18 and p > 0.05).

Myocardial mechanics are normal in patients with aortic regurgitation, independent of abnormalities in cardiac geometry. Conversely, in aortic stenosis and mixed aortic valve disease, significant alterations in the patterns of fibre shortening are found. The effects of stenosis on cardiac function seem to dominate the effect of ventricular remodelling.

Technical developments in paediatric cardiology over the last few decades have increased expectations on professionals, demanding of them more emotional competence and communicative ability. The aim of this study was to examine the approach of paediatric cardiologists in informing and communicating with the family of the patient.

A qualitative interview method was first tested in a pilot study with two paediatric cardiologists. There were nine subsequent semi-structured interviews that were carried out with paediatric cardiologists. A researcher performed all the interviews, which were taped, transcribed, decoded, and analysed.

Among paediatric cardiologists, how to break bad news to the family is an important concern, evident in findings regarding the significance of trust and confidence, the use of different emotional positions, and a common ambition to achieve skills to handle the situation. There is a need for reflection, education, and sharing of experiences. The cardiologists desire further development of teamwork and of skills in medical students and residents for delivering bad news.

Doctors are expected to cope with the complexities of diagnoses and decisions, while simultaneously being sensitive to the feelings of the parents, aware of their own emotions, and able to keep it all under control in the context of breaking the bad news to the parents and keeping them informed. These conflicting demands create a need to expand the professional role of the doctor by including more training in emotional competence and communicative ability, beginning in medical school and continuing through consultancy.

There is no standard dose or protocol for beta-blocker administration as preconditioning in children undergoing coronary CT angiography.

A total of 63 consecutive patients, with a mean age of 10.0±3.1 years, who underwent coronary CT angiography to assess possible coronary complications were enrolled in a single-centre, retrospective study. All patients were given an oral beta-blocker 1 hour before coronary CT angiography. Additional oral beta-blocker or intravenous beta-blocker was given to those with a high heart rate. We compared image quality, radiation exposure, and adverse events among the patients without additional beta-blocker, with additional oral beta-blocker, and with additional intravenous beta-blocker.

There were no significant differences in image quality or radiation exposure among the groups. The heart rate just before scanning was significantly correlated with image quality (p<0.001, r=−0.533) but was not correlated with radiation exposure (p=0.45, r=0.096). There were no adverse events related to any allergic reaction, thereby showing the effectiveness of the beta-blocker.

Initial oral beta-blocker administration (0.8 mg/kg/dose) should be administered to all children undergoing coronary CT angiography. Additional intravenous beta-blocker should be given to those with poor heart rate control to improve image quality without increasing radiation exposure or allowing adverse events.