The increased survival rate among individuals with CHD has sparked interest in their transition to adult healthcare. Although there is a general agreement on the importance of transition interventions, the empirical evidence supporting them is insufficient. Therefore, this study aimed to conduct a systematic review and meta-analysis of transition interventions for adult healthcare in adolescents and young adults.

A literature search was conducted for studies comparing the quantitative effects of transition interventions with control groups, published up to March 15, 2023, in major databases (CENTRAL, Embase, PubMed, Web of Science, CINAHL, KISS, and KMbase), major clinical trial registries, academic journal sites related to the topic, and grey literature databases. Ten studies involving a total of 1,297 participants were identified. Transition interventions proved effective in enhancing disease-related knowledge (Hedge’s g = 0.89, 95% CI = 0.29−1.48) and self-management (Hedge’s g = 0.67, 95% CI = 0.38−0.95), as well as reducing loss to follow-up (OR = 0.41, 95% CI = 0.22−0.77). The certainty of evidence for the estimated values of each major outcome was low or very low.

This study supports the implementation of transition interventions by demonstrating that they can improve patients’ disease knowledge and self-management, while also promoting treatment continuity. However, since the available data on transition interventions for adolescents and young adults with CHD remain limited, the widespread adoption of structured transition interventions in the future may alter the conclusions of this study.

URL: https://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42023399026.

The main objective measure to assess the health of the Fontan circulation is the pressure measurement of the superior vena cava or pulmonary arteries. We reviewed the literature for benefits of measuring resting pressure in the Fontan circuit and explored whether dynamic measurement by volume loading or exercise has the potential to refine this diagnostic tool.

PubMed was searched for articles showing a relationship between resting post-operative central venous pressure or pulmonary artery pressure and Fontan failure. Relationships between post-operative central venous pressure or pulmonary artery pressure and volume loading changes, such as during exercise or volume loading during cardiac catheterization, were also queried.

A total of 44 articles mentioned relationships between resting central venous pressure or pulmonary artery pressure and Fontan failure. Only 26 included an analysis between the variables and only seven of those articles found pressure to be predictive of Fontan failure. Ten articles examined the relationship between exercise or volume loading and outcomes and demonstrated a large individual variation of pressures under these dynamic conditions.

Based on current literature, there is not a lot of strong evidence to show that elevated resting central venous pressure or pulmonary artery pressure is predictive of Fontan failure. Some individuals experience dramatic increases in central venous pressure or pulmonary artery pressure under increased loading conditions with exercise or bolus fluid infusion, while others experience increases closer to that of a healthy control population. Further studies are needed to examine whether more dynamic and continuous monitoring of systemic venous pressures might better predict outcomes in patients with Fontan circulation.

Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation.

We sought to validate the score in an external, modern cohort.

This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type—Norwood/Blalock–Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant.

In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4–89.5%).

In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care.

This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions). Patients were classified as “too late” (death or declined for transplant due to being too sick) and/or “care escalation” (ventricular assist device implanted, inotrope initiated, and/or listed for transplant), within 30 days. “Late referral” was defined as those referred too late and/or had care escalation.

Between 7/2020 and 7/2022, 77 Fontan patients (52% inpatient) had an initial heart failure consultation. Ten per cent were referred too late (6 were too sick for heart transplantation with one subsequent death, and two others died without heart transplantation evaluation, within 30 days), and 36% had care escalation (21 listed ± 5 ventricular assist device implanted ± 6 inotrope initiated). Overall, 42% were late referrals. Heart failure consultation < 1 year after Fontan surgery was strongly associated with late referral (OR 6.2, 95% CI 1.8–21.5, p=0.004).

Over 40% of Fontan patients seen for an initial heart failure consultation were late referrals, with 10% dying or being declined for transplant within a month of consultation. Earlier referral, particularly for those with heart failure soon after Fontan surgery, should be encouraged.

CHD is a lifelong condition with a significant burden of disease to patients and families. With increased survival, attention has shifted to longer-term outcomes, with a focus on social determinants of health. Among children with CHD, socioeconomic status is associated with disparities in outcomes. Household material hardship is a concrete measure of poverty and may serve as an intervenable measure of socioeconomic status.

A longitudinal survey study was conducted at multiple time points (at acute hospitalisation, then 12–24 months later in the chronic phase) to determine the prevalence of household material hardship among parents of children with advanced heart disease and quality of life during long-term follow-up.

The analytic cohort was 160 children with a median patient age of 1 year (IQR 1,4) with 54% of patients <2 years. During acute hospitalisation, over one-third of families reported household material hardship (37%), with significantly lower household material hardship in the chronic phase at 16% (N = 9 of 52). For parents reporting household material hardship during acute hospitalisation, 50% had resolution of household material hardship by the chronic phase. Household material hardship-exposed children were significantly more likely to be publicly insured (56% versus 20%, p = 0.03) with lower quality of life than those without household material hardship (64% versus 82%, p = 0.013).

The burden of heart disease during the chronic phase of illness is high. Household material hardship may serve as a target to ensure equity in the care and outcomes of CHD patients and their families.

Catecholaminergic polymorphic ventricular tachycardia is an ion channelopathy, caused by mutations in genes coding for calcium-handling proteins. It can coexist with left ventricular non-compaction. We aim to investigate the clinical and genetic characteristics of this co-phenotype.

Medical records of 24 patients diagnosed with catecholaminergic polymorphic ventricular tachycardia in two Chinese hospitals between September, 2005, and January, 2020, were retrospectively reviewed. We evaluated their clinical and genetic characteristics, including basic demographic data, electrocardiogram parameters, medications and survival during follow-up, and their gene mutations. We did structural analysis for a novel variant ryanodine receptor 2-E4005V.

The patients included 19 with catecholaminergic polymorphic ventricular tachycardia mono-phenotype and 5 catecholaminergic polymorphic ventricular tachycardia-left ventricular non-compaction overlap patients. The median age of onset symptoms was 9.0 (8.0,13.5) years. Most patients (91.7%) had cardiac symptoms, and 50% had a family history of syncope. Overlap patients had lower peak heart rate and threshold heart rate for ventricular tachycardia and ventricular premature beat during the exercise stress test (p < 0.05). Sudden cardiac death risk may be higher in overlap patients during follow-up. Gene sequencing revealed 1 novel ryanodine receptor 2 missense mutation E4005V and 1 mutation previously unreported in catecholaminergic polymorphic ventricular tachycardia, but no left ventricular non-compaction-causing mutations were observed. In-silico analysis showed the novel mutation E4005V broke down the interaction between two charged residues.

Catecholaminergic polymorphic ventricular tachycardia overlapping with left ventricular non-compaction may lead to ventricular premature beat/ventricular tachycardia during exercise stress test at lower threshold heart rate than catecholaminergic polymorphic ventricular tachycardia alone; it may also indicate a worse prognosis and requires strict follow-up. ryanodine receptor 2 mutations disrupted interactions between residues and may interfere the function of ryanodine receptor 2.

Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood.

In this prospective longitudinal multi-institutional study, patients 13–30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies.

Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19–31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients.

In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

The left ventricular outflow tract is an important source of ventricular arrhythmias. Up to one-third of all idiopathic ventricular arrhythmias in patients with structurally normal hearts may arise from this region. We would like to share the results of our left ventricular outflow tract ablation using three-dimensional mapping and limited fluoroscopy.

This is a single-centre retrospective cohort study. Forty-six consecutive patients who underwent left ventricular outflow tract ablation procedures between January 2015 and June 2023 were included in the study. The EnSite Precision System (Abbott, St. Paul, MN, USA) was used to facilitate mapping and to reduce or eliminate the need for fluoroscopy.

The study group comprised 29 males and 17 females, with a mean age of 13.4 ± 4.5 years. The most common location for arrhythmias was the left coronary cusp (n : 21). Other locations, in sequence, included the junction of the right and left coronary commissure (n : 10), right coronary cusp (n : 10), left ventricular outflow tract endocardium (n:4), aorto-mitral junction (n : 1), and great cardiac vein (n : 1). Nine of these patients had previously undergone unsuccessful right ventricular outflow tract ablation at another centre. Cryoablation was performed in three patients, irrigated radiofrequency ablation in three patients, and conventional radiofrequency ablation in the remaining patients. The acute success rate was 100%, and no recurrences were observed. The mean follow-up period was 49.6 ± 24.4 months. All patients were asymptomatic and were being followed without antiarrhythmic medication.

Although left ventricular outflow tract ablations pose a risk for coronary artery and heart valve complications, they can be performed successfully and safely with the guidance of three-dimensional mapping.

Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown.

Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated.

Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down.

The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors’ knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Informed consent for surgery is a complex process particularly in paediatrics. Complexity increases with procedures such as CHD surgery. Regulatory agencies outline informed consent contents for surgery. We assessed and described CHD surgical informed consent contents through survey dissemination to paediatric CHD centres across United States of America.

Publicly available email addresses for 125 paediatric cardiac clinicians at 70 CHD surgical centres were obtained. Nine-item de-identified survey assessing adherence to The Joint Commission informed consent standards was created and distributed via RedCap® 14 March, 2023. A follow-up email was sent 29 March, 2023. Survey link was closed 18 April, 2023.

Thirty-seven surveys were completed. Results showed informed consent documents were available in both paper (25, 68%) and electronic (3, 8%) format. When both (9, 24%) formats were available, decision on which format to use was based on centre protocols (1, 11%), clinician personal preference (3, 33%), procedure being performed (1, 11%), or other (4, 45%). Five (13%) centres’ informed consent documents were available only in English, with 32 (87%) centres also having a Spanish version. Review of informed consent documents demonstrated missing The Joint Commission elements including procedure specific risks, benefits, treatment alternatives, and expected outcomes.

Informed consent for CHD surgery is a complex process with multiple factors involved. Majority of paediatric CHD surgical centres in the United States of America used a generic informed consent document which did not uniformly contain The Joint Commission specified information nor reflect time spent in discussion with families. Further research is needed on parental comprehension during the informed consent process.

To explore how the COVID-19 pandemic affected fetal care, social support, and emotional functioning for women carrying a fetus with CHD.

This was a single-centre qualitative study of 31 women who received a prenatal diagnosis of CHD during the pandemic. Patients completed semi-structured interviews about their experiences with fetal care, social support, and perceptions of risk to themselves and their fetus. Consistent themes regarding the impact of the pandemic were identified using an inductive thematic approach. Demographic data were collected via self-report and chart review.

Women generally reported consistent access to fetal care throughout the pandemic, with frequent use of telemedicine in addition to in-person care, but negative impacts resulting from restrictions on family support at appointments. Limited access to social support overall and a loss of pregnancy traditions were described. Many women reported feeling isolated and experiencing worries and fears about COVID-19 but also noted feeling supported by their healthcare team. Partner/family support during appointments and connection to peer-to-peer support were identified as recommendations to mitigate negative impacts.

Women carrying a fetus with CHD during the COVID-19 pandemic experienced unique stressors that may affect mental health. However, many also experienced unexpected supports that may mitigate effects of pandemic-related stressors. Results can inform efforts to promote positive family outcomes during and following the COVID-19 pandemic.

CHD are among the most common congenital defects. Due to the chronic nature of CHD, patients face various risk factors that threaten their mental health. However, a comprehensive understanding of the medical and social predictors of mental health issues in adults with CHD is lacking. This study aims to investigate the prevalence of anxiety and depression in adults with CHD.

This cross-sectional descriptive study focused on adults with CHD in Kerman, Iran. The participants completed demographic information alongside two psychological assessment tools: the Beck Anxiety Inventory (BAI) and the Depression Anxiety Stress Scales (DASS)-21. The data were analyzed using SPSS 26.

The mean age of the participants was 29.94 ± 12.36 years, and 63.8% were female. According to the DASS, 73.4% did not have depression, 61% did not have anxiety, and 76.2% did not have stress. In total, 19% had mild stress, and 4.8% had moderate stress. According to the BAI, 27.6% did not have anxiety. Individual characteristics were not significantly associated with depression. However, gender, age, and type of surgery were significantly associated with anxiety. Cyanosis was significantly associated with stress.

The results show that mental disorders like depression, anxiety, and stress are highly prevalent in adults with CHD. The prevalence depends on individual factors such as age, gender, and disease severity. Therefore, it is recommended that mental disorders in this population be evaluated and treated accurately.

Chylothorax following paediatric cardiac surgery is associated with significant morbidity, particularly those that are refractory to conservative therapy. It is our impression that there is important variability in the medical, surgical, and interventional therapies used to manage refractory chylothorax between congenital heart programmes. We therefore conducted a survey study of current practices for managing refractory chylothorax.

The Chylothorax Work Group, formed with the support of the Pediatric Cardiac Critical Care Consortium, designed this multi-centre survey study with a focus on the timing and indication for utilising known therapies for refractory chylothorax. The survey was sent to one chylothorax expert from each Work Group centre, and results were summarised and reported as the frequency of given responses.

Of the 20 centres invited to participate, 17 (85%) submitted complete responses. Octreotide (13/17, 76%) and sildenafil (8/17, 47%) were the most utilised medications. Presently, 9 (53%) centres perform pleurodesis, 15 (88%) perform surgical thoracic duct ligation, 8 (47%) perform percutaneous lymphatic interventions, 6 (35%) utilise thoracic duct decompression procedures, and 3 (18%) perform pleuroperitoneal shunts. Diagnostic lymphatic imaging is performed prior to surgical thoracic duct ligation in only 7 of the 15 (47%) centres that perform the procedure. Respondents identified barriers to referring and transporting patients to centres with expertise in lymphatic interventions.

There is variability in the treatment of refractory post-operative chylothorax across a large group of academic heart centres. Few surveyed heart centres have replaced surgical thoracic duct ligation or pleurodesis with image-guided selective lymphatic interventions.

This study examined the relationship between changes in physical activity and their impact on exercise capacity and health-related quality of life over a 3-year span in patients with CHD.

We evaluated 99 young patients with CHD, aged 13–18 years at the outset. Physical activity, health-related quality of life, and exercise capacity were assessed via questionnaires and peak oxygen uptake measurements at baseline and after 3 years; changes in measures were estimated between the two time points and categorised into quartiles. Participants were stratified according to achieved (active) or not-achieved (inactive) recommended levels of physical activity (≥150 minutes/week) at both time points.

Despite increases in physical activity, exercise capacity, and health-related quality of life over 3 years, the changes were not statistically significant (all p > 0.05). However, a positive association was found between physical activity changes and exercise capacity (ß = 0.250, p = 0.040) and health-related quality of life improvements (ß = 0.380, p < 0.001). Those with the most pronounced physical activity increase showed notable exercise capacity (p < 0.001) and health-related quality of life increases (p < 0.001) compared with patients with the largest decline in physical activity. The active-inactive category demonstrated a notable decline in exercise capacity compared to the active-active group, while the inactive-active group showed health-related quality of life improvements.

Over 3 years, increased physical activity was consistently linked to increases in exercise capacity and health-related quality of life in patients with CHD, highlighting the potential of physical activity augmentation as an intervention strategy.

In this study, we aimed to evaluate quality of life and parental attitudes in children who underwent an atrial septal defect closure procedure with a transcatheter or surgical method in childhood and whether they continued their lives with similar activities to their healthy peers by comparing the two groups.

Patient forms to define sociodemographic and clinical features, the Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents (KINDL) to measure the quality of life of children, and the Parent-Child Relationship Test (Parental Attitude Research Instrument) to measure parental attitudes were used.

The groups were similar in terms of age and sex. The mean quality of life scale scores were high in all groups, and there was no statistically significant difference between the scores. Parents of the patients who underwent closure received higher scores from the demographic attitudes and recognition of quality subdimension compared to the parents of the healthy group.

The quality of life of children with atrial septal defect closure was found to be similar to their healthy peers. Additionally, the effects of surgical or percutaneous closure of atrial septal defect on quality of life are similar. Children with atrial septal defect closure perceive their health status as well as their healthy peers, and this perception does not cause any difference in the attitudes and behaviours of families.

Cholestasis characterised by conjugated hyperbilirubinemia is a marker of hepatobiliary dysfunction following neonatal cardiac surgery. We aimed to characterise the incidence of conjugated hyperbilirubinemia following neonatal heart surgery and examine the effect of conjugated hyperbilirubinemia on post-operative morbidity and mortality.

This was a retrospective study of all neonates who underwent surgery for congenital heart disease (CHD) at our institution between 1/1/2010 and 12/31/2020. Patient- and surgery-specific data were abstracted from local registry data and review of the medical record. Conjugated hyperbilirubinemia was defined as perioperative maximum conjugated bilirubin level > 1 mg/dL. The primary outcome was in-hospital mortality. Survival analysis was conducted using the Kaplan–Meier survival function.

Conjugated hyperbilirubinemia occurred in 8.5% of patients during the study period. Neonates with conjugated hyperbilirubinemia were more likely to be of younger gestational age, lower birth weight, and non-Caucasian race (all p < 0.001). Patients with conjugated hyperbilirubinemia were more likely to have chromosomal and non-cardiac anomalies and require ECMO pre-operatively. In-hospital mortality among patients with conjugated hyperbilirubinemia was increased compared to those without (odds ratio 5.4). Post-operative complications including mechanical circulatory support, reoperation, prolonged ventilator dependence, and multi-system organ failure were more common with conjugated hyperbilirubinemia (all p < 0.04). Patients with higher levels of conjugated bilirubin had worst intermediate-term survival, with patients in the highest conjugated bilirubin group (>10 mg/dL) having a 1-year survival of only 6%.

Conjugated hyperbilirubinemia is associated with post-operative complications and worse survival following neonatal heart surgery. Cholestasis is more common in patients with chromosomal abnormalities and non-cardiac anomalies, but the underlying mechanisms have not been delineated.