To summarise the characteristics and postoperative outcomes in paediatric patients with coronary sinus septal defect.

This retrospective study recruited paediatric patients diagnosed with coronary sinus septal defect from the Guangdong Cardiovascular Institute between 2011 and 2023. Clinical characteristics, echocardiographic parameters, surgical procedures, and postoperative outcomes were collected from electronic health records.

Among the 68 patients, 50% were male, with a median age of 1.0 years. Four cases (5.9%) were diagnosed during the prenatal period. The proportions of patients with type I, II, III, and IV coronary sinus septal defect were 51.5%, 5.9%, 16.1%, and 26.5%, respectively. The most common coexisting cardiac anomalies were persistent left superior caval vein. Twenty-seven cases were either missed or misdiagnosed by echocardiogram, accounting for 39.7% of the overall cases, with type I being the most frequently missed diagnosis. Fifty-four patients underwent surgery, two patients received transcutaneous intervention, while the remaining patients did not undergo any surgery or intervention. At follow-up, two patients with type I coronary sinus septal defect died from multiorgan dysfunction, and one patient underwent reoperation due to narrowing of the extracardiac tunnel. The remaining patients did not experience any major events and recovered well.

Paediatric patients with coronary sinus septal defect often do not exhibit specific clinical manifestations. Enhancing our understanding of the anatomic and haemodynamic characteristics of coronary sinus septal defect can improve the diagnostic accuracy of echocardiography. If diagnosis is suspected, confirmation can be obtained by cardiac CT and cardiac magnetic resonance. Accurate preoperative and intraoperative diagnosis of coronary sinus septal defect contributes to high surgical success rates and favourable treatment outcomes.

Cardiac catheterisation in the postoperative period emerges as a primary tool, providing effectiveness and safety in diagnosis, treatment guidance, and resolution of major residual lesions.

This is a retrospective case-control study. We collected the clinical records of patients who underwent cardiac catheterisation between January 2003 and December 2022 within the initial 72 hours after surgery in the pediatric cardiac ICU of a national referral hospital in Mexico City. Descriptive, univariate, and multivariate analyses were performed.

A total of 6,243 surgeries were performed, of which 264 were cardiac catheterizations carried out within the first 72 hours of the postoperative period;these included 73 diagnostic procedures and 191 interventional procedures. One hundred and thirty-five (135) catheterisations targeted recent suture intervention sites. The primary indications for cardiac catheterisation included low cardiac output and the suspicion of major residual lesions. Approximately 65% of interventions occurred within the first 24 hours and solved 426 residual lesions. No significant associations were found between mortality, complications, and the need for surgical reintervention in patients who underwent interventional catheterisation at recent suture sites (OR 1.93;95% CI:0.94–4.07:p = 0.076). Seventeen patients (17) were extubated in the initial 24 hours post-catheterisation. Two major complications were identified: rupture of the systemic-to-pulmonary shunt in the anastomosis, and a pulmonary artery laceration requiring emergency surgery. One patient died.

Cardiac catheterisation has evolved into a vital instrument to diagnose and resolve abnormalities and significant residual lesions without increasing the morbidity and mortality risks.

Malnutrition is a relevant prognostic factor in cardiovascular disease. However, it has not been studied in adults with CHD and Fontan circulation.

Retrospective, single-centre cohort study including all consecutive adults with Fontan circulation. Objectives: 1. To evaluate the prevalence of malnutrition, defined according to Controlling Nutritional Status score, which includes albumin, lymphocytes, and cholesterol and 2. To assess its utility as a prognostic marker.

We included 93 patients (55.9% male) with a mean age of 32.7 ± 8.3 years. After a median follow-up of 5.5 years (interquartile range 2.2 – 10.6), 14 patients met the combined primary outcome of death or heart transplant (15.1%). Moderate or severe malnutrition (Controlling Nutritional Status score ≥ 5) was detected in 18.3%. Overweight was found in 21.5% of patients, obesity in 4.3%, and low weight in 8.6%, with no significant differences in malnutrition parameters across weight categories. Patients with malnutrition had worse functional capacity (58.8% in New York Heart Association—NYHA-class III–IV, vs. 33.3% in patients without malnutrition, p = 0.05).

In univariate analysis, malnutrition was associated with a worse prognosis (death or heart transplant) with a hazard ratio of 3.7 (95% confidence interval 1.3 to 10.7, p = 0.01). In the adjusted model including cyanosis, functional class, and protein-losing enteropathy, malnutrition did not reach statistical significance (p = 0.81).

Malnutrition as defined by Controlling Nutritional Status score is common in adults with Fontan circulation and represents a strong prognostic marker. Controlling Nutritional Status scale could be used in Fontan patients as a simple tool to identify a high-risk population.

Dysphagia is common in infants born with critical CHD. Thickened liquids are often used to treat dysphagia, but associated risks limit widespread use among feeding specialists. This survey aims to assess dysphagia treatment patterns and thickened liquid use across paediatric cardiac surgical centres.

A 24-question, cross-sectional survey. Convenience and snowball sampling methods were used to engage 52 paediatric cardiac surgical centres affiliated with the Cardiac Newborn Neuroprotective Network. Descriptive statistics were used to analyse and compare responses.

Twenty-six individual respondents represented 21 unique paediatric cardiac surgical centres. Most responses were from experienced, speech–language pathologists (78%) at medium size centres (88%). Ninety-three percent of responding centres used thickened liquids to treat dysphagia and 81% only after formal instrumental assessment of swallowing. Thickened oral feeding was used for single-ventricle patients by 85% versus 69% for two-ventricle patients. Barriers to recommending thickened oral feedings included the cost of thickening agents, parental non-adherence, and gastrointestinal concerns.

This is the first survey to report multi-institutional dysphagia treatment practice variation at United States congenital cardiac surgical centres. Thickened oral feedings are frequently used across centres in high-risk critical CHD patients but treatment benefit remains unclear. This survey highlights a broad scientific community poised to direct dysphagia research in critical CHD to address practice variation, short- and long-term impact of thickened oral feeding on feeding outcomes, and barriers to use and access of thickening agents.

Children with CHD are at increased risk for neurodevelopmental disabilities and neuropsychological impairments throughout their life span. The purpose of this report is to share our experience building a sustainable, novel, inpatient, interdisciplinary Neurocardiac Critical Care Program to mitigate risks and optimize outcomes during the ICU stay.

A descriptive review was chosen to identify meaningful characteristics, challenges and lessons learned related to the establishment, expansion of and sustainability of Neurocardiac Critical Care Program in a 26-bed pediatric cardiac ICU.

We successfully launched, expanded, and sustained an interdisciplinary Neurocardiac Critical Care Program. Here, we share the foundation, framework, challenges, and lessons learned as we established and sustained the Neurocardiac Critical Care Program. The key elements of our program are (1) consistent engagement by pediatric neurologists in the cardiac ICU, (2) comprehensive education initiatives, (3) evidence-based clinical practice changes, and (4) quality improvement and research projects.

The development of a pediatric Neurocardiac Critical Care Program is feasible and sustainable. This program was informed by recent research related to perioperative and psychosocial risk factors that impact brain development and neurodevelopmental outcomes in this vulnerable population. By aligning our efforts, our multidisciplinary team is helping shift the paradigm in pediatric cardiac critical care to actively manage complex heart disease, while simultaneously and proactively mitigating risks to the developing brain and family unit.

Neurodevelopmental follow-up programmes for children with CHD help identify neurodevelopmental impairments and support the delivery of recommended interventions. The Cardiac Neurodevelopmental Outcome Collaborative, Diversity, Equity, and Inclusion Special Interest Group surveyed members to assess perceived patient barriers to neurodevelopmental follow-up, previous diversity and inclusion education, and confidence in caring for historically marginalised populations.

A link to a Redcap online survey was emailed to Cardiac Neurodevelopmental Outcome Collaborative members on 23 April 2022, with 4 weeks given to complete.

Eighty-four participants from 37 institutions in North America completed the survey. Respondents acknowledged that education on the importance of neurodevelopmental follow-up and frequent accommodations for cancellations or rescheduling clinic visits is essential. Language interpretation and written materials were available in languages other than English, but a limited number provided fully translated evaluation reports. Driving distance and the caregiver’s lack of understanding of the rationale for neurodevelopmental follow-up were the top perceived barriers to programme attendance. At the institutional level, training for cultural competency was typically provided, and most respondents felt comfortable caring for patients from diverse backgrounds. However, many agreed their programmes could do more to make evaluations accessible to historically marginalised/underserved populations.

Multiple barriers exist to cardiac neurodevelopmental follow-up, particularly for patients from under-represented minorities and for those whose primary language is not English. Surveying families will be valuable to understand how we may overcome these barriers. Further education about the importance of neurodevelopmental follow-up programmes continues to be essential.

Acquired chylothorax is an established complication of CHD surgery, affecting 2–9% of patients. CHD places a child at risk for failure to thrive, with subsequent chylothorax imposing additional risk.

We conducted a retrospective chart review to ascertain quantitative markers of nutrition and growth in children affected by chylothorax following CHD surgery between 2018 and 2022 compared to controls.

We utilised electronic medical record system, EPIC, at Children’s Hospital, New Orleans, targeting subjects < 18 years old who underwent CHD surgery between 2018 and 2022 and developed a subsequent chylothorax. Study subjects were identified using the 10th revision of the International Classification of Diseases codes (ICD-10 codes: J94.0, I89.8, and J90.0). Each chylothorax case (n = 20) was matched by procedure type and age to a control with no chylothorax (n = 20). Data were recorded in REDCap and analysed using SPSS.

After removal of outliers, we analysed 19 total matched pairs. There was no statistical difference in growth velocity (p = 0.12), weight change (operation to discharge) (p = 0.95), weight change (admission to discharge) (p = 0.35), Z-score change (operation to discharge) (p = 0.90), Z-score change (admission to discharge) (p = 0.21), serum protein (p = 0.88), or serum albumin (p = 0.82). Among cases, linear regression demonstrated no significant association between maximum chylous output and growth velocity (p = 0.91), weight change (operation to discharge) (p = 0.15), or weight change (admission to discharge) (p = 0.98).

We did not observe statistically significant markers of growth or nutrition in children with chylothorax post-CHD surgery compared to those without chylothorax. Multisite data collection and analysis is required to better ascertain clinical impact and guide clinical practice.

Right anterolateral thoracotomy or left anterolateral thoracotomy applied to partial anomalous pulmonary venous connection correction have been reported to obtain cosmetic and less invasive outcomes as alternative approaches to median sternotomy. However, the application of different approaches is still confusing. We compared the perioperative and mid-term outcomes to seek indications of different approaches and guide surgical treatment of partial anomalous pulmonary venous connection.

From July 2019 to August 2023, 44 patients who underwent surgical correction of partial anomalous pulmonary venous connection were recruited. Of these, 13 (29.6%) patients who underwent median sternotomy were separated into M group, 28 (63.6%) patients who underwent right anterolateral thoracotomy were separated into R group, and three (6.8%) patients who underwent left anterolateral thoracotomy were separated into L group. Clinical data were reviewed and compared between each group.

In M group, direct anastomosis accounted for the most (7, 53.8%), in R group, intra-atrial rerouting accounted for the most (22, 78.6%), and in L group, direct anastomosis accounted for the most (3, 100%). R group had shorter cardiopulmonary bypass time (70.0 ± 32.4 vs113.1 ± 83.3, p = 0.029), shorter ICU stay (1.0 ± 0.2 vs 1.9 ± 1.7, p = 0.01), shorter postoperative stay (6.0 ± 1.2 vs 8.3 ± 5.0, p = 0.021), and fewer chest tube drainage (8.4 ± 4.3 vs 13.9 ± 10.2, p = 0.026) compared with M group. Three left-sided partial anomalous pulmonary venous connection in L group adopted off-pump approach.

Median sternotomy S.V.C. can be applicable to all anatomic types of partial anomalous pulmonary venous connection especially for complex types. Considering the minimally invasive advantages, we prefer applying right anterolateral thoracotomy for pulmonary vein connected to right atrium or proximal end of off-pump. Applying left anterolateral thoracotomy for left-sided partial anomalous pulmonary venous connection without atrial septal defect by off-pump.

Fear of cardiac arrest among parents of infants with heart disease can cause stress and anxiety. Literature is scarce on the effects of cardiopulmonary resuscitation training (CPRt) on anxiety and stress of parents. We analysed the impact of CPRt on anxiety, stress, and comfort levels on parents of infants with heart disease.

Cardiopulmonary resuscitation (CPR) and choking relief manoeuvre (CRM) comfort level, Parental State-Trait Anxiety Inventory (STAI), and Parenting Stress Index (PSI) scores were prospectively collected pre-, immediately post-, and 3 months post-CPRt.

There were 97 participants: 80% (n = 78) mothers/grandmothers and 20% (n = 19) fathers. The mean (SD) age of participants was 28.7 (5.6) years old. There was a significant decrease in STAI across the three time points collected; STAI decreased by 12% from baseline to immediately post-CPRt and 19% from baseline to 3 months post-CPRt (p < .0001). There were no significant changes in PSI across the time points. Baseline to immediately post-teaching, we found that CPRt significantly increased comfort performing CPR, CRM, and comfort in knowing what to do (p=< .001, p=< .001, p=< .001, respectively). Comfort levels persisted elevated when comparing pre- to 3 months post-CPRt (p=< .001, p= .002, p= .001, respectively), maintaining at least a 177% average increase up to 3 months post-CPRt for all aspects.

CPRt can aid in improving anxiety and comfort levels of parents of infants with heart disease around hospital discharge. Parental preparedness and reassurance to know what to do in emergency situations can be enhanced by a simple intervention such as CPRt.

This study aims to examine the surgical outcome of Kabuki syndrome patients after neonatal congenital heart surgery.

This was a single-centre retrospective study of Kabuki syndrome patients undergoing neonatal congenital heart surgery from 2018 to 2023. Primary outcome was survival to discharge after index surgery. Secondary outcomes were morbidities and complications. Survival and hospital length of stay were compared to neonates with non-Kabuki genetic anomalies undergoing congenital heart surgery in the same time period.

A total of seven patients were reviewed. All Kabuki syndrome patients had left-sided lesions including three with hypoplastic left heart syndrome, three with aortic stenosis and/or aortic arch hypoplasia, and one with an isolated coarctation of aorta. Hospital survival was 5/7 (71% compared to 88% for neonates with non-Kabuki genetic anomalies). To date, four remain alive, including one with hypoplastic left heart syndrome. A higher percentage of Kabuki syndrome patients had unplanned interventions (43% vs 15% in non-Kabuki), abnormal brain imaging (29% vs 5%), and bacteremia (29% vs 9%). Median total ventilator days for Kabuki patients were also longer (16 days vs 6 days in non-Kabuki) as was hospital length of stay (66 days vs 41 days).

Despite survival to discharge after index operation, Kabuki syndrome patients with single ventricle physiology remain at high risk of mortality and morbidity after cardiac surgery. However, they may be discharged without ventilator dependency and survive to toddler years.

The interstage period is a critical phase for single ventricle infants due to their fragile cardiovascular state. Infants often experience medical and feeding challenges during this period, resulting in caregiver stress. We completed a quality improvement project at Children’s Healthcare of Atlanta to understand these challenges to inform targeted interventions.

This single-center project included a medical chart review and a cross-sectional caregiver survey. Data were collected on patient and caregiver demographics and clinical variables. Feeding outcomes were assessed using the Pediatric Functional Oral Intake Scale. Caregiver impact was measured using the Feeding/Swallowing Impact Survey.

The project included 15 single ventricle patients with a mean (standard deviation) age of 151.73(25.92) days at the time of the second-stage palliation. Forty percent of patients experienced at least one readmission, primarily due to feeding intolerance (20%) and desaturations (26.7%). Milk protein allergy (26.9%) was the most common medical complication, followed by interstage unplanned reinterventions. Pediatric Functional Oral Intake Scale scores demonstrated that 33% consumed minimal volumes or no oral intake at the time of the bidirectional Glenn, and 93.3% of patients did not receive outpatient feeding services during the interstage. Caregiver stress scores resulted in mean scores (standard deviation) of 2.23(1.54), with the highest impact on daily activities. All caregivers affirmed the need for a dedicated multidisciplinary clinic.

The interstage period for single ventricle patients poses significant medical and feeding challenges, resulting in caregiver stress. Comprehensive, multidisciplinary feeding support during the interstage period may improve patient outcomes and alleviate caregiver burden.

To demonstrate safety and efficacy of using the AmplatzerTM vascular plug II device for perimembranous ventricular septal defect closure with retrograde approach and show the follow-up in all patients.

At present, there is no FDA-approved device for transcatheter closure of perimembranous ventricular septal defects. Small studies and case reports have shown the use of various catheter-based devices in an off-label management; however, there are no large studies to show their efficacy. The second generation of AmplatzerTM vascular plug seems to offer a safe and attractive alternative for this procedure. Besides, a retrograde approach might decrease procedure time and radiation exposure time.

Patients with congenital perimembranous ventricular septal defects who underwent transcatheter closure using Amplatzer vascular plug II devices were included. Primary end point was to determine efficacy and safety of this generation of devices and the incidence of complications at follow-up (complete heart block and aortic/tricuspid/mitral regurgitation). Forty-five patients underwent perimembranous ventricular septal defect closure at a median age of 6 years (9 months–17 years). During the catheterization, there were only minor complications and at follow-up of 48 ± 25.7 months (up to 96 months). Closure rate was high of 93.3% and freedom from atrioventricular block was 100%.

The second generation of the AmplatzerTM vascular plug II seems to offer a safe and attractive alternative for percutaneous closure of the perimembranous ventricular septal defects.

Due to the high postoperative mortality, tools for an adaequate risk stratification are important to identify high-risk patients undergoing the Norwood procedure. As a marker of ventricular wall stress, NT-proBNP might be of particular interest in these children.

This study evaluated whether NT-proBNP’s age-adjusted z-score (“zlog-NT-proBNP”) predicts outcomes after stage I Norwood procedure.

Patients who underwent the Norwood procedure between 1 January 2011 and 31 December 2022, with perioperative NT-proBNP measurements available were enrolled. Since reference intervals of NT-proBNP are highly age-dependent, age-adjusted zlog-NT-proBNP was used. Serial zlog-NT-proBNP values were analysed to predict the occurrence of major adverse cardiovascular events after the Norwood procedure. Major adverse cardiovascular events was defined as death, resuscitation, or mechanical circulatory support. Secondary endpoints were re-operation and re-intervention for shunt.

A total of 139 patients underwent the Norwood procedure and had at least one NT-proBNP measurement available. Preoperative zlog-NT-proBNP measurements (median 3.7, interquartile range 3.1–4.19) showed no association with the occurrence of major adverse cardiovascular events or mortality. Zlog-NT-proBNP early after ICU admission (3.2, interquartile range 2.4–3.8) was predictive of mortality but showed no association with the occurrence of major adverse cardiovascular events. Zlog-NT-proBNP before ICU discharge (3.2, interquartile range 2.8–3.8) was significantly associated with the occurrence of both major adverse cardiovascular events (hazard ratio 1.83, 95% confidence interval 1.25–2.67, P = 0.002) and death (hazard ratio 2.1, 95% CI 1.4–3.2, P < 0.001).

High zlog-NT-proBNP levels after the Norwood surgery were strongly associated with the occurrence of major adverse cardiovascular events and death. Therefore, zlog-NT-proBNP has the potential to identify high-risk patients before life-threatening complications occur.

Poor weight gain in infants with single ventricle cardiac physiology between stage 1 and stage 2 palliative surgeries is associated with worse outcomes. The growth of infants with single ventricle physiology, enrolled in home monitoring programmes in the United Kingdom, has not been widely described.

To explore the growth of infants with single ventricle physiology supported by a home monitoring programme, at a tertiary centre in the South of England.

A retrospective review of two cohorts, comparing weight gain amongst infants with single ventricle physiology, before and following the implementation of a home monitoring programme. Inclusion was dependent on a diagnosis compatible with single ventricle physiology during the interstage.

Enrolment into a home monitoring programme (cohort 2) was associated with 55% more infants being discharged home during the interstage period (p < 0.05). Interstage mortality did not differ between cohorts. There were no differences in interstage growth velocity between cohorts (cohort 1 23.98 ± 11.7 g/day and cohort 2 23.82 ± 8.3 g/day); however, infants in cohort 2 experienced less growth deceleration early in life, and achieved catch-up growth at 12-23 months. Interstage nasogastric feeding, regardless of the cohort, was associated with worse growth outcomes.

A home monitoring programme for infants with single ventricle physiology provides the opportunity for infants to be safely discharged home to their families and cared for at home during the interstage. Infants in the home monitoring programme experienced better growth, achieving weight restoration at 12–23 months.

Glenn procedure carries low morbidity and mortality within stages of single-ventricle palliation. However, some patients with Glenn failure need a stage reversal, while others require unanticipated surgical interventions. Our understanding of perioperative factors and outcomes associated with such unexpected interventions is extremely limited.

Patients who underwent unexpected surgery after the Glenn procedure between January 2010 and December 2019 within the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were identified with a subgroup analysis of those reverting to stage I physiology. Patient’s requiring reversal to stage I palliation were matched 1:5 with controls. Multivariable logistic regression analysis was performed to evaluate risk factors for reintervention.

A total of 16,913 patients underwent Glenn procedure with 1221 (7.2%) requiring unexpected cardiac surgical intervention and 95 (0.56%) patients required takedown to a stage I. Significant clinical and operative risk factors were identified for such unexpected interventions.

The overall mortality after Glenn procedure was 1.2%, while mortality after unexpected reintervention was 6.6% at 30 days and after Glenn takedown was 27.5% at last follow-up. Unexpected surgical intervention and right ventricular dominance were significant risk factors for mortality.

Unexpected reinterventions, including need for takedown after the Glenn procedure, are associated with significantly higher mortality. Further studies should focus on improving our patient selection, understanding the risk factors mechanistically, including impact of the right ventricle as systemic ventricle in order to avoid need for unexpected surgical interventions.

Northwest axis on an electrocardiogram is conventionally thought to be associated with CHD, but there is a paucity of data regarding the types and incidence of CHD associated with this finding. The purpose of this study was to report the types and incidence of CHD found at our institution to determine the efficacy of electrocardiograms as a screening test in infants.

Retrospective, single-centre study of all infants ≤ 60 days of age who underwent a first-time electrocardiogram from 2015 to 2021. Our inclusion criteria included those found to have a northwest axis on electrocardiogram and who also had an echocardiogram performed. Data were described using standard summary statistics.

Two hundred sixty-eight infants met inclusion criteria (median age 4 days, interquartile range 2–24). The most common reason for an electrocardiogram to be obtained was the presence of a heart murmur (n = 102, 38%). One hundred thirty-one infants (49%) were found to have significant CHD. Cardiac defects included ventricular septal defect (n = 36, 28%), severe pulmonary valve stenosis (n = 17, 13%), atrioventricular canal defect (n = 16, 12%), and single ventricle disease (n = 16, 12%).

In this study, nearly half (49%) of these infants with northwest axis on electrocardiogram were found to have significant CHD. Electrocardiograms remain an important screening tool for infants with findings associated with potential cardiac disease and echocardiogram is warranted for infants found to have northwest axis on a first-time electrocardiogram.