CHDs are the most common type of birth defect. One in four newborns with a heart defect has a critical CHD. In Mexico, there is a lack of data available to determine its prevalence. Pulse oximetry screening programmes have been implemented worldwide, reporting opportunity areas in algorithm interpretation and data management. Our study aims to share preliminary results of a 3-year experience of a multicentre pulse oximetry screening programme that addresses critical challenges.

This retrospective study examined the reports of newborns screened from February 2016 to July 2019 from five hospitals. Two algorithms –the New Jersey and the American Academy of Pediatrics– were implemented over consecutive periods. The algorithms’ impact was assessed through the calculation of the false-positive rate in an eligible population.

A total of 8960 newborns were eligible for the study; from it, 32.27% were screened under the New Jersey and 67.72% under the American Academy of Pediatrics algorithm – false-positive rate: 1% (CI 95: ± 0.36%) and 0.71% (CI 95: ± 0.21%), respectively. Seventy-nine newborns were referred, six were diagnosed with critical CHD, and six with CHD. The critical CHD estimated prevalence was 6.69:10,000 newborns (CI 95: ± 5.36). Our results showed that the algorithm was not related to the observable false-positive rate reduction.

Other factors may play a role in decreasing the false-positive rate. Our experience implementing this programme was that a systematic screening process led to more confident results, newborn’s report interpretation, and follow-up.

Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia.

A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro–Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described.

Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3 years (interquartile range 4.3–6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12 mmHg (interquartile range 10–15), the Nakata index 272 mm2/m2 (interquartile range 204–327), and the McGoon index (interquartile range 1.86–2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19 days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%.

The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.

Case reports of the development of perimyocarditis in adolescents and young adults after BNT162b2 messenger RNA (mRNA) COVID-19 vaccination have raised concerns about the cardiac side effects of the vaccine. The aim of the study was to evaluate clinical follow-up and subclinical myocardial function after mRNA COVID-19 vaccine in adolescents with chronic heart disease.

Forty-one adolescents aged 12–18 who were followed up at paediatric cardiology clinic between December 2021 and May 2022, and who had received two doses of the Pfizer-BioNTech COVID-19 mRNA vaccine were included in the study. The patients were evaluated five times in total – before the vaccination, one week after receiving the first dose, one month after receiving the first dose, one week after receiving the second dose, and one month after receiving the second dose. Cardiac assessment for all patients included an electrocardiogram, transthoracic echocardiography, and two-dimensional speckle-tracking strain echocardiography for left ventricular subclinical myocardial function.

The mean age of the adolescents was 16.2 ± 1.5 years, and 56% (n = 23) were male. There was no statistically significant difference in patients' echocardiographic measurements including left ventricular global longitudinal strain and electrocardiogram parameters including PR, QRS, and QTc intervals through the follow-up. Seven patients reported cardiac complaints at post-vaccination follow-up visits, but laboratory and echocardiographic evidence of cardiac involvement was not observed.

Based on the results of our study, the mRNA COVID-19 vaccine did not cause impairment in subclinical myocardial function assessed by speckle-tracking echocardiography in adolescents with chronic heart disease.

In single-ventricle patients undergoing staged-bidirectional Glenn, 36–59% have aorto-pulmonary collateral flow, but risk factors and clinical outcomes are unknown. We hypothesise that shunt type and catheter haemodynamics may predict pre-bidirectional Glenn aorto-pulmonary collateral burden, which may predict death/transplantation, pulmonary artery or aorto-pulmonary collateral intervention.

Retrospective cohort study of patients undergoing a Norwood procedure for single-ventricle anatomy. Covariates included clinical and haemodynamic characteristics up to/including pre-bidirectional Glenn catheterisation and aorto-pulmonary collateral burden at pre-bidirectional Glenn catheterisation. Multivariable models used to evaluate relationships between risk factors and outcomes.

From January 2011 to March 2016, 104 patients underwent Norwood intervention. Male sex (odds ratio 3.36, 95% confidence interval 1.17–11.4), age at pre-bidirectional Glenn assessment (2.12, 1.33–3.39 per month), and pulmonary to systemic flow ratio (1.23, 1.08–1.41 per 0.1 unit) were associated with aorto-pulmonary collateral burden. Aorto-pulmonary collateral burden was not associated with death/transplantation (hazard ratio 1.19, 95% confidence interval 0.37–3.85), pulmonary artery (sub-hazard ratio 1.38, 0.32–2.61), or aorto-pulmonary collateral interventions (sub-hazard ratio 1.11, 0.21–5.76). Longer post-Norwood length of stay was associated with greater risk of death/transplantation (hazard ratio 1.22 per week, 95% confidence interval 1.08–1.38), but lower risk of aorto-pulmonary collateral intervention (sub-hazard ratio 0.86 per week, 95% confidence interval 0.75–0.98). Time to pre-bidirectional Glenn catheterisation was associated with lower risk of pulmonary artery (sub-hazard ratio 0.80 per month, 95% confidence interval 0.65–0.98) and aorto-pulmonary collateral intervention (sub-hazard ratio 0.79, 0.63–0.99). Probability of moderate/severe aorto-pulmonary collateral burden increased with left-to-right shunt (22.5% at <1.0, 57.6% at >1.4) and the age at pre-bidirectional Glenn catheterisation (10.6% at <2 months, 56.9% at >5 months).

Aorto-pulmonary collateral burden is common after Norwood procedure and increases as age at bidirectional Glenn increases. As expected, higher pulmonary to systemic flow ratio is a marker for greater aorto-pulmonary collateral burden pre-bi-directional Glenn; aorto-pulmonary collateral burden does not confer risk of death/transplantation or pulmonary artery intervention.

Patient- and proxy-reported outcomes (PROs) are an important indicator of healthcare quality and can be used to inform treatment. Despite the widescale use of PROs in adult cardiology, they are underutilised in paediatric cardiac care. This study describes a six-center feasibility and pilot experience implementing PROs in the paediatric and young adult ventricular assist device population.

The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a collaborative learning network comprised of 55 centres focused on improving clinical outcomes and the patient/family experience for children with heart failure and those supported by ventricular assist devices. The development of ACTION’s PRO programme via engagement with patient and parent stakeholders is described. Pilot feasibility, patient/parent and clinician feedback, and initial PRO findings of patients and families receiving paediatric ventricular assist support across six centres are detailed.

Thirty of the thirty-five eligible patients (85.7%) were enrolled in the PRO programme during the pilot study period. Clinicians and participating patients/parents reported positive experiences with the PRO pilot programme. The most common symptoms reported by patients/parents in the first month post-implant period included limitations in activities, dressing change distress, and post-operative pain. Poor sleep, dressing change distress, sadness, and fatigue were the most common symptoms endorsed >30 days post-implant. Parental sadness and worry were notable throughout the entirety of the post-implant experience.

This multi-center ACTION learning network-based PRO programme demonstrated initial success in this six-center pilot study experience and yields important next steps for larger-scale PRO collection, research, and clinical intervention.

Although pulmonary artery banding remains a useful palliation in bi-ventricular shunting lesions, single-stage repair holds several advantages. We investigate outcomes of the former approach in high-risk patients.

Retrospective cohort study including all pulmonary artery banding procedures over 9 years, excluding single ventricle physiology and left ventricular training.

Banding was performed in 125 patients at a median age of 41 days (2–294) and weight of 3.4 kg (1.8–7.32). Staged repair was undertaken for significant co-morbidity in 81 (64.8%) and anatomical complexity in 44 (35.2%). The median hospital stay was 14 days (interquartile range 8–33.5) and 14 patients (11.2%) required anatomical repair before discharge. Nine patients died during the initial admission (hospital mortality 7.2 %) and five following discharge (inter-stage mortality 4.8%). Of 105 banded patients who survived, 19 (18.1%) needed inter-stage re-admission and 18 (14.4%) required unplanned re-intervention. Full repair was performed in 93 (74.4%) at a median age of 13 months (3.1–49.9) and weight of 8.5 kg (3.08–16.8). Prior banding, 54% were below the 0.4th weight centile, but only 28% remained so at repair. Post-repair, 5/93 (5.4%) developed heart block requiring permanent pacemaker, and 11/93 (11.8%) required unplanned re-intervention. The post-repair mortality (including repairs during the initial admission) was 6/93 (6.5%), with overall mortality of the staged approach 13.6% (17/125).

In a cohort with a high incidence of co-morbidity, pulmonary artery banding is associated with a significant risk of re-intervention and mortality. Weight gain improves after banding, but heart block, re-intervention, and mortality remain frequent following repair.

The primary objective was to evaluate the trend of blood sirolimus concentrations in neonates following ductal stenting. The long-term outcomes and incidence of infections were also evaluated.

Prospective open-label observational study in a tertiary referral centre over a 1-year period. Serum sirolimus levels were estimated at 1 hour and 24hrs post-stent insertion followed by 7 days in neonates who underwent ductal stenting. The trend in sirolimus levels, incidence of infections, complications and outcomes following ductal stenting were studied.

Seven neonates with duct-dependent pulmonary circulation underwent ductal stenting at median age of 8.5 days and weight of 2.83kg. The average stent size was 3.5±0.4 mm, and average stent length was 16.3±5.1 mm. The mean sirolimus concentrations at 1 hour, 24 hours and 7 days were 41.3±6.9ng/ml, 15.4±7.1ng/ml and 3.1±0.85ng/ml respectively. Levels fell below therapeutic range for all patients by 7 days. Three patients had sepsis or necrotising enterocolitis, but responded well to antibiotics; 1 patient had aspiration related sudden death. There were no further events at a mean follow-up of 207 days, and 4 patients underwent elective surgery at 238 ± 81 days after ductal stenting.

This study demonstrates applicability of drug-eluting stents for ductal stenting in newborns. Drug-eluting stents with abluminal drug delivery are associated with high sirolimus levels in initial hours but rapidly taper to negligible levels within a week of implantation. Neonates with high pre-procedure likelihood of infection developed sepsis but responded well to conservative management. The patency of drug-eluting ductal stents is preserved over long-term follow-up.

This study aimed to evaluate the efficacy and safety of high-frequency oscillation ventilation combined with intermittent mandatory ventilation in infants with acute respiratory distress syndrome after congenital heart surgery.

We retrospectively analysed the clinical data of 32 infants who were ventilated due to acute respiratory distress syndrome after congenital heart surgery between January, 2020 and January, 2022. We adopted high-frequency oscillation ventilation combined with intermittent mandatory ventilation as the rescue ventilation mode for infants who were failing conventional mechanical ventilation.

After rescue high-frequency oscillation ventilation combined with intermittent mandatory ventilation, the dynamic compliance (Cdyn), PaO2 and PaO2/FiO2 ratio of the infants improved compared with conventional mechanical ventilation (p < 0.05). Moreover, high-frequency oscillation ventilation combined with intermittent mandatory ventilation resulted in a significant decrease in arterial-alveolar oxygen difference (AaDO2), FiO2, and oxygenation index (p < 0.05). No significant effect on haemodynamic parameters was observed. Moreover, no serious complications occurred in the two groups.

Rescue high-frequency oscillation ventilation combined with intermittent mandatory ventilation significantly improved oxygenation in infants who failed conventional mechanical ventilation for acute respiratory distress syndrome after congenital heart surgery. Thus, this strategy is considered safe and feasible. However, further studies must be conducted to confirm the efficacy and safety of high-frequency oscillation ventilation combined with intermittent mandatory ventilation as a rescue perioperative respiratory support strategy for CHD.

Patent ductus arteriosus is an important cause of morbidity and mortality, especially in very low birth weight infants. The aim of the study is to report our single-centre short-term results of preterm patients who underwent ligation through left anterior mini-thoracotomy .

Data of 27 preterm infants operated by the same surgeon who underwent Patent ductus arteriosus (PDA) closure with left anterior mini-thoracotomy technique between November 2020 and January 2022 at a single institution were reviewed. The patients were divided into two groups according to their weight at the time of surgery. Data on early postoperative outcomes and survival rates after discharge were collected.

Twenty-seven patients with a mean (±SD) gestational age of 25.8 (±2.0) weeks and a mean birth weight of 1027 (±423) g were operated using left anterior mini-thoracotomy technique. The lowest body weight was 480 g. Complications such as bleeding, abnormal healing of incision, or pneumothorax were not seen. There were 8 mortalities after the operation (29,6 %). The causes of the deaths were sepsis, necrotising enterocolitis, hydrops fetalis, hepatoblastoma, and intracranial bleeding. There was no statistically significant difference in the rates of complication between the groups.

Left anterior mini-thoracotomy technique can be performed as the first choice when transcatheter intervention cannot be applied in preterm infants. It provides easy access to the PDA, a good exposure, minimal contact with the lungs, good cosmetic results in early and mid-term and shortens the operation time, especially in very low birth weight preterm babies. However, early ligation may be helpful to minimise the complications related to PDA.

Anomalous origin of pulmonary artery branch from ascending aorta (APA) in the presence of two separate semilunar valves is an uncommon entity necessitating early diagnosis and surgery to prevent development of irreversible pulmonary vascular disease. We evaluated our experience with the technique and outcome of 11 patients with this condition.

Between January 2000 and December 2019, 11 patients were diagnosed with APA. Echocardiographic data were collected from the records, including the site of origin of the anomalous pulmonary artery, additional defects, pulmonary artery pressures, and biventricular function. Intraoperative charts were reviewed for the details of the surgical procedure and cardiopulmonary bypass. Post-operative data included survival, ventilatory support, and duration of hospital stay. After discharge, children were reviewed at 1 month, 3 months, 6 months, and then at yearly intervals.

Of the 11 patients, females were more than males (7:4) with a median age of 6 months (15 days–28 years) and median weight of 5.7 kg (1.8–40 kg). Nine patients underwent direct re-implantation of anomalous pulmonary artery branch to main pulmonary artery. The survival rate was 88.8% in our series. On follow-up, no re-operations or re-interventions were required and all surgically corrected patients were in stable clinical condition.

Early- and midterm outcomes of children who underwent surgery for APA is convincing. Early direct re-implantation of the anomalous branch pulmonary artery to main pulmonary artery without any graft material is the optimal surgical strategy for these patients.

To describe the epidemiology of severe bleeding in the immediate post-operative period in children who undergo cardiopulmonary bypass surgery using the Bleeding Assessment Scale for critically Ill Children (BASIC).

Retrospective cohort study in a paediatric ICU from 2015 to 2020.

356 children were enrolled; 59% were male with median (IQR) age 2.1 (0.5–8) years. Fifty-seven patients (16%) had severe bleeding in the first 24 hours post-operatively. Severe bleeding was observed more frequently in younger and smaller children with longer bypass and cross-clamp times (p-values <0.001), in addition to higher surgical complexity (p = 0.048). Those with severe bleeding received significantly more red blood cells, platelets, plasma, and cryoprecipitate in the paediatric ICU following surgery (all p-values <0.001). No laboratory values obtained on paediatric ICU admission were able to predict severe post-operative bleeding. Those with severe bleeding had significantly less paediatric ICU-free days (p = 0.010) and mechanical ventilation-free days (p = 0.013) as compared to those without severe bleeding.

Applying the BASIC definition to our cohort, severe bleeding occurred in 16% of children in the first day following cardiopulmonary bypass. Severe bleeding was associated with worse clinical outcomes. Standard laboratory assays do not predict bleeding warranting further study of available laboratory tests.

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.

Low-profile stent implantation remains a rescue treatment for aortic coarctation and branch pulmonary arteries stenosis in small children. Stent re-expansion to cope with vascular growth remains problematic.

To evaluate ex vivo feasibility and mechanical behaviour of over-dilating BeSmooth peripheral stents (Bentley InnoMed, Germany).

Three BeSmooth peripheral stents in diameters of 7, 8, and 10 mm were dilated to nominal pressure and then 13 atm. BeSmooth Ø7 × 23 mm was sequentially post-dilated using 12, 14, and 16 mm high-pressure balloons. BeSmooth Ø10 × 57 mm was post-dilated with a 14 mm balloon and then with a 48 mm bare-metal Optimus XXL stent hand-mounted on a 14 mm balloon (stent-in-stent). BeSmooth Ø8 × 57 mm was directly post-dilated with a 48 mm bare-metal Optimus XXL stent hand-mounted on a 16 mm balloon (stent-in-stent). The stents’ diameter and length were measured. Digital inflation pressure was noted. Balloon rupture and stent fracture patterns were closely evaluated.

At 20atm pressure, BeSmooth Ø7 × 23 mm shortened to 2 mm forming a 12 mm diameter solid ring circle and the woven balloon ruptured radially. At 10 atm pressure, BeSmooth Ø10 × 57 mm fractured longitudinally in various dispatched breaking points at a diameter of 13 mm without shortening and ruptured the balloon with multiple pinholes. At 10 atm pressure, BeSmooth Ø8 × 57 mm fractured centrally at three different points at a diameter of 11.5 mm without shortening and the balloon broke radially in half.

In our benchmark tests, extreme shortening, severe balloon rupture, or unpredictable stent fracture patterns at small balloon diameters limits safe post-dilation of BeSmooth stents beyond 13 mm. BeSmooth stents are not ideal candidates for off-label stent interventions in smaller patients.

Balloon valvuloplasty is the primary treatment for congenital aortic valve stenosis in our centre. We sought to determine independent predictors of reintervention (surgical repair or repeated balloon dilation) after primary valvuloplasty.

We retrospectively studied patients with congenital aortic valve stenosis who underwent balloon valvuloplasty during 2004–2018. The following risk factors were analysed: aortic valve insufficiency after balloon valvuloplasty >+1/4, post-procedural gradient across the aortic valve ≥35 mmHg, pre-interventional gradient across the valve, annulus size, use of rapid pacing, and balloon/annulus ratio. Primary outcome was aortic valve reintervention.

In total, 99 patients (median age 4 years, range 1 day to 26 years) underwent balloon valvuloplasty for congenital aortic valve stenosis. After a mean follow-up of 4.0 years, 30% had reintervention. Adjusted risks for reintervention were significantly increased in patients with post-procedural aortic insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg (HR 2.55, 95% CI 1.13–5.75, p = 0.024). Pre-interventional gradient, annulus size, rapid pacing, and balloon/annulus ratio were not associated with outcome.

Post-procedural aortic valve insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg in patients undergoing balloon valvuloplasty for congenital aortic valve stenosis confers an increased risk for reintervention in mid-term follow-up.

To better understand parents’ accounts of their prenatal and postnatal experience after prenatal diagnosis of CHD – particularly emotional processing and coping mechanisms – to identify strategies to improve support.

This single-centre, longitudinal qualitative study included pregnant mothers and their support persons seen in Fetal Cardiology Clinic at Vanderbilt Children’s Hospital from May through August 2019 for probable complex CHD. Twenty-seven individuals from 17 families participated in 62 phone interviews during pregnancy and postpartum: 27 conducted after the initial prenatal cardiology consultation, 15 after a follow-up prenatal visit, and 20 after birth. Applied thematic analysis approach was used to code and analyse transcribed interviews. Coding and codebook revisions occurred iteratively; intercoder reliability was >80%.

Patients included mothers (16 [59%]), fathers (8 [30%]), and other support persons (3 [11%]). Initial fetal diagnoses included a range of moderate to severe CHD. Prenatally, parents sought to maintain hope while understanding the diagnosis; planning for the future rather than focusing on day-to-day was more common if prognoses were better. Postnatally, with confirmation of prenatal diagnoses, parents’ sense of control expanded, and they desired more active engagement in clinical decision making.

To enhance effective communication and support, understanding how parents conceptualise hope in relation to diagnosis and how that may evolve over time is critical. Expectant parents whose child has a significant risk of mortality may demonstrate hope by focusing on positivity. As prognostic uncertainty diminishes postpartum, the parental role on the team may shift, requiring clinicians to provide different support.

Congenitally corrected transposition of the great arteries is a complex pathology characterised by atrioventricular and ventriculo-arterial discordance. Optimal surgical approaches are still a matter of debate.

To evaluate the outcomes of different surgical treatments in a single centre.

Between 1998 and 2020, 89 patients were studied. The cohort was divided into three groups: physiologic, anatomic, and univentricular repair.

Physiologic correction (56.18%) was associated with significant tricuspid valve regurgitation progress (42%) and complete AV block (30%) compared to anatomic repair. Right ventricular systolic dysfunction was developed in 14%. Instead, anatomic correction (30.34%) (double switch 59% and Rastelli type 40.7%) presented moderate to severe aortic regurgitation (4%) and left ventricular systolic dysfunction (11%). Complete AV block was developed in 14.8%. Rate of reintervention was 34% for physiologic and 26% for anatomic. Univentricular palliation (13.8%) presented no complications or late mortality during the follow-up. The overall survival at 5 and 10 years, respectively, was 80% (95% CI 69, 87) and 75% (95% CI 62, 84). There was no statistically significant difference in mortality between the groups (p log-rank = 0.5752).

Management of congenitally corrected transposition of the great arteries remains a challenge. In this cohort, outcomes after physiologic repair were satisfactory in spite of the progression of tricuspid regurgitation and the high incidence of AV block. Anatomic repair improved tricuspid regurgitation but increased the risk of aortic regurgitation and left ventricular systolic dysfunction. The Fontan group showed the lowest incidence of complications.