To evaluate the motor proficiency, identify risk factors for abnormal motor scores, and examine the relationship between motor proficiency and health-related quality of life in school-aged patients with CHD.

Patients ≥ 4 years old referred to the cardiac neurodevelopmental program between June 2017 and April 2020 were included. Motor skills were evaluated by therapist-administered Bruininks-Oseretsky Test of Motor Proficiency Second-Edition Short Form and parent-reported Adaptive Behavior Assessment System and Patient-Reported Outcomes Measurement Inventory System Physical Functioning questionnaires. Neuropsychological status and health-related quality of life were assessed using a battery of validated questionnaires. Demographic, clinical, and educational variables were collected from electronic medical records. General linear modelling was used for multivariable analysis.

The median motor proficiency score was the 10th percentile, and the cohort (n = 272; mean age: 9.1 years) scored well below normative values on all administered neuropsychological questionnaires. In the final multivariable model, worse motor proficiency score was associated with family income, presence of a genetic syndrome, developmental delay recognised in infancy, abnormal neuroimaging, history of heart transplant, and executive dysfunction, and presence of an individualised education plan (p < 0.03 for all predictors). Worse motor proficiency correlated with reduced health-related quality of life. Parent-reported adaptive behaviour (p < 0.001) and physical functioning (p < 0.001) had a strong association with motor proficiency scores.

This study highlights the need for continued motor screening for school-aged patients with CHD. Clinical factors, neuropsychological screening results, and health-related quality of life were associated with worse motor proficiency.

Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.

The Takeuchi procedure remains an important surgical option for treating anomalous origin of the left coronary artery from the pulmonary artery, particularly in cases where direct coronary reimplantation is not feasible. However, long-term outcome data in paediatric patients are limited.

We retrospectively reviewed 18 paediatric patients who underwent Takeuchi repair between 2007 and 2023. Clinical characteristics, echocardiographic data, and outcomes—including survival, ventricular function, mitral regurgitation, and reintervention—were analysed. Kaplan–Meier analysis was used to assess survival and freedom from reintervention, and paired comparisons were evaluated using the Wilcoxon signed-rank test.

The median age at surgery was 6 months (range: 25 days to 12 years). Preoperative left ventricular ejection fraction was significantly depressed (median 23.5%), and mitral regurgitation was present in all patients. There were two early deaths (11.1%), both in patients with severe heart failure. No late mortality was observed during a maximum follow-up of 10.9 years. All survivors achieved New York Heart Association class I status. Left ventricular ejection fraction improved significantly postoperatively (p < 0.0001), and mitral regurgitation grade also decreased significantly (p < 0.001), with 94.4% showing only mild residual mitral regurgitation. Reintervention occurred in three patients (16.7%) for pulmonary artery stenosis or baffle leak. Freedom from reintervention at 10.9 years was 66.7%.

The Takeuchi procedure offers excellent survival and functional recovery in paediatric anomalous origin of the left coronary artery from the pulmonary artery patients when coronary translocation is not feasible. Although late complications such as pulmonary artery stenosis or baffle leak can occur, outcomes remain favourable with appropriate follow-up.

To assess the prevalence of obesity and investigate any changes in body mass index in children with CHD compared to age-matched healthy controls, in Southwestern Ontario.

The body mass index z-scores of 1259 children (aged 2–18) with CHD were compared with 2037 healthy controls. The body mass index z-scores of children who presented to our paediatric cardiology outpatient clinic from 2018 to 2021 were compared with previously collected data from 2008 to 2010. A longitudinal analysis of patients with data in both cohorts was also completed.

In total, 21.4% of patients with CHD and 26.6% of healthy controls were found to be overweight or obese (p < 0.001). The 2018–2021 cohort of CHD patients and controls had significantly higher body mass index z-scores compared to the 2008–2010 cohort (p < 0.001). Longitudinal analysis showed that body mass index z-scores significantly increased over time for CHD patients with data in both cohorts (2018–2021: M = 0.59, SD = 1.26; 2008–2010: M = −0.04, SD = 1.05; p < 0.001).

The prevalence of obesity in all children, irrespective of CHD, is rising. The coexistence of obesity and CHD may pose additional cardiovascular risks and complications.

The functional roles of ventricular dominance and additional ventricular chamber after Fontan operation are still uncertain. We aim to assess and correlate such anatomical features to late clinical outcomes.

Fontan patients undergoing cardiac MRI and cardiopulmonary exercise test between January 2020 and December 2022 were retrospectively reviewed. Clinical, cardiac MRI, and cardiopulmonary exercise test data from the last follow-up were analysed.

Fifty patients were analysed: left dominance was present in 29 patients (58%, median age 20 years, interquartile range:16–26). At a median follow-up after the Fontan operation was 16 years (interquartile range: 4–42), NYHA classes III and IV was present in 3 patients (6%), 4 (8%) underwent Fontan conversion, 2 (4%) were listed for heart transplantation, and 2 (4%) died. Statistical analysis showed that the additional ventricular chamber was larger (>20 mL/m2) in patients with a right dominant ventricle (p = 0.01), and right dominance was associated with a higher incidence of post-operative low-cardiac output syndrome (p = 0.043). Left ventricular dominance was associated with a better ejection fraction (p = 0.04), less extent of late gadolinium enhancement (p = 0.022), higher metabolic equivalents (p = 0.01), and higher peak oxygen consumption (p = 0.033). A larger additional ventricular chamber was associated with a higher need for post-operative extracorporeal membrane oxygenation support (p = 0.007), but it did not influence functional parameters on cardiac MRI or cardiopulmonary exercise test.

In Fontan patients, left ventricular dominance correlated to better functional outcomes. Conversely, a larger additional ventricular chamber is more frequent in right ventricular dominance and can negatively affect the early post-Fontan course.

To evaluate the outcomes of patients with single ventricle physiology supported with extracorporeal membrane oxygenation as a bridge to first-stage palliation.

This was a retrospective registry-based study. Data from the Extracorporeal Life Support Organization registry were used to identify single ventricle physiology patients supported with extracorporeal membrane oxygenation prior to palliation from 2016 to 2021. Descriptive statistics and multivariate analyses for associations with mortality were conducted.

Primary outcome was death before hospital discharge. Patient characteristics including demographics and associated complications were evaluated as secondary outcomes. Sixty-five patients met inclusion criteria. Survival to discharge was 42%. Twenty-four (37%) patients died while on extracorporeal membrane oxygenation. There was no significant difference in demographics between survivors and non-survivors. Non-survivors had a significantly longer median duration on extracorporeal membrane oxygenation compared to survivors, 99-hrs [IQR (Interquartile Range), 160, 300] vs. 59-hrs [43, 124] (p<0.001). Multivariate analysis demonstrated extracorporeal membrane oxygenation duration (adjusted-OR [Odds Ratio] 1.01, 95% CI [Confidence Interval] 0.98, 0.99; p = 0.03) and requiring renal replacement therapy (42% vs. 19%; p = 0.04) were associated with mortality prior to discharge.

Clinicians managing decompensated patients with single ventricle physiology may consider extracorporeal membrane oxygenation as a bridge to palliation. Survival to discharge was 42%. Evidence of renal injury and longer extracorporeal membrane oxygenation durations were associated with mortality. These data may be used to guide providers and to counsel families. However, more data are needed to refine indications and assess associations related to outcomes and decision-making.

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.

Duchenne muscular dystrophy is a devastating neuromuscular disorder characterized by the loss of dystrophin, inevitably leading to cardiomyopathy. Despite publications on prophylaxis and treatment with cardiac medications to mitigate cardiomyopathy progression, gaps remain in the specifics of medication initiation and optimization.

This document is an expert opinion statement, addressing a critical gap in cardiac care for Duchenne muscular dystrophy. It provides thorough recommendations for the initiation and titration of cardiac medications based on disease progression and patient response. Recommendations are derived from the expertise of the Advance Cardiac Therapies Improving Outcomes Network and are informed by established guidelines from the American Heart Association, American College of Cardiology, and Duchenne Muscular Dystrophy Care Considerations. These expert-derived recommendations aim to navigate the complexities of Duchenne muscular dystrophy-related cardiac care.

Comprehensive recommendations for initiation, titration, and optimization of critical cardiac medications are provided to address Duchenne muscular dystrophy-associated cardiomyopathy.

The management of Duchenne muscular dystrophy requires a multidisciplinary approach. However, the diversity of healthcare providers involved in Duchenne muscular dystrophy can result in variations in cardiac care, complicating treatment standardization and patient outcomes. The aim of this report is to provide a roadmap for managing Duchenne muscular dystrophy-associated cardiomyopathy, by elucidating timing and dosage nuances crucial for optimal therapeutic efficacy, ultimately improving cardiac outcomes, and improving the quality of life for individuals with Duchenne muscular dystrophy.

This document seeks to establish a standardized framework for cardiac care in Duchenne muscular dystrophy, aiming to improve cardiac prognosis.

In paediatric cardiac patients requiring staged palliation, superior cavopulmonary anastomosis is common. Pain control is a crucial aspect of postoperative care as agitation, untreated pain, and hypoventilation can cause increased pulmonary vascular resistance reduction and pulmonary blood flow.

This was a large volume single-centre, retrospective cohort study evaluating the impact of gabapentin on opioid and benzodiazepine exposure in infants undergoing superior cavopulmonary anastomosis between January 2018 and December 2022. The primary endpoint was opioid exposure in morphine milligram equivalents per kilogram in infants receiving gabapentin compared to no gabapentin.

The study analysed 85 infants, 40 of which received perioperative gabapentin. Other than there being more males in the gabapentin group (70% versus 47%; p = 0.03), there was no difference in baseline characteristics. Opioid use, measured in morphine milligram equivalents per kilogram, was similar in the no gabapentin group compared to the gabapentin group during the first 5 POD’s (2.66 (interquartile range1.76, 3.30) versus 2.27 (interquartile range R 1.75, 3.40); p = 0.93. However, there was a lower benzodiazepine exposure, measured in midazolam equivalents per kilogram, in the gabapentin group both on POD 2 (0.05 (interquartile range 0.00, 0.11) versus 0 (interquartile range 0.00, 0.08); p = 0.031) and cumulative (0.15 (interquartile range 0.00, 0.35) versus 0.05 (interquartile range 0.00, 0.15); p = 0.031).

Gabapentin did not significantly reduce opioid exposure; however, its use was associated with modest reduction in benzodiazepine exposure. There were no differences in adverse events. Our findings suggest gabapentin is safe in infants undergoing superior cavopulmonary anastomosis; however, additional studies should be conducted to evaluate optimal gabapentin dosing.

Despite advances in treatment and outcomes for paediatric heart failure, both physical and psychosocial comorbidities remain notable among this patient population. We aimed to qualitatively describe the psychosocial experiences of adolescent and young adults with heart failure and their caregivers’ perceptions, with specific focus on personal challenges, worries, coping skills, and resilience.

Structured, in-depth interviews were performed with 16 adolescent and young adults with heart failure and 14 of their caregivers. Interviews were recorded and transcribed. Content analysis was performed, and themes were generated. Transcripts were coded by independent reviewers.

Ten (63%) adolescent and young adults with heart failure identified as male and six (37.5%) patients self-identified with a racial or ethnic minority group. Adolescent and young adults with heart failure generally perceived their overall illness experience more positively and less burdensome than their caregivers. Some adolescent and young adults noted specific worries related to surgeries, admissions, major complications, death, and prognostic/treatment uncertainty, while caregivers perceived their adolescent and young adult’s greatest worries to be around major complications and death. Adolescent and young adults and their caregivers were able to define and reflect on adolescent and young adult experiences of resilience, with many adolescent and young adults expressing a sense of optimism and gratitude as it relates to their medical journey.

This study is the first of its kind to qualitatively describe the psychosocial experiences of a racially and socioeconomically diverse sample of adolescent and young adults with heart failure, as well as their caregivers’ perceptions of patient experiences. Findings underscore the importance of identifying distress and fostering resilient processes and outcomes in young people with advanced heart disease.

Several organizations including the Environmental Protection Agency, World Health Organization and American Academy of Pediatrics recommend that hospital sound levels not exceed 45 decibels. Yet, several studies across multiple age groups have observed higher than recommended levels in the intensive care setting. Elevated sound levels in hospitals have been associated with disturbances in sleep, patient discomfort, delayed recovery, and delirium.

We measured sound levels in a pediatric cardiac intensive care unit and collected vital signs data, sedation dosing and delirium scores. During a 5-week study period, sound levels for 68 patients in 22 private and 4 semi-private rooms were monitored.

Sound levels were consistently above stated recommendations with an average daytime level of 50.6 decibels (maximum, 76.9 decibels) and an average nighttime level of 49.5 decibels (maximum, 69.6 decibels). An increase in average and maximum sound levels increased the probability of sedation administration the following hour (p-value < 0.001 and 0.01, respectively) and was predictive of an increase in heart rate and blood pressure (p-value < 0.001).

Sound levels in the CICU were consistently higher than recommended. An increase in heart rate, blood pressure and sedation utilization may suggest a stress response to persistent and sudden loud sounds. Given known negative impacts of excessive noise on stress, sleep, and brain development, as well as the similar adverse effects from the related use of sedative medications, reducing excessive and sudden noise may provide an opportunity to improve short- and long-term hemodynamic and neurodevelopmental outcomes in the pediatric cardiac intensive care unit.

Primary pulmonary vein stenosis is a rare congenital heart condition and carries a poor prognosis. Various surgical techniques have been introduced to treat primary pulmonary vein stenosis. However, there is no consensus on the optimal approach. This study aimed to evaluate the outcomes of surgical treatment of primary pulmonary vein stenosis, identify the risk factors for pulmonary vein restenosis and reintervention, and determine the optimal strategy for primary pulmonary vein stenosis repair.

From December 2008 to December 2023, 64 patients with primary pulmonary vein stenosis undergoing surgical repair in our institution were included [non-sutureless group, n = 42; sutureless group, n = 22]. The Cox proportional hazard model was used to identify risk factors for pulmonary vein restenosis and pulmonary vein restenosis-related reintervention.

There were three in-hospital deaths and one late death. The survival rates at 1, 5, and 15 years were 91.9%, 84.2%, and 69.8%, respectively, in the whole cohort. Compared with the non-sutureless group, the sutureless group did not significantly improve the long-term survival of primary pulmonary vein stenosis patients (P = 0.484). Pulmonary vein restenosis occurred in 15 patients. Multivariable analysis showed that Involvement of the left inferior pulmonary vein (P < 0.001) was the only independent risk factor for pulmonary vein restenosis, while sutureless repair (P = 0.037) was a protective factor. Pulmonary vein restenosis-related reintervention occurred in 12 patients. On multivariate analysis, three or four pulmonary veins involvement (P = 0.001) and preoperative severity score exceeding 5 (P = 0.050) were found to be independent risk factors associated with pulmonary vein restenosis-related reintervention; however, sutureless repair (P = 0.021) was a protective factor.

Management of infants with primary pulmonary vein stenosis is challenging. Surgical repair of primary pulmonary vein stenosis can be safely achieved using different techniques, with similar long-term mortality. Compared with non-sutureless repair, sutureless repair was significantly associated with decreased pulmonary vein restenosis and decreased pulmonary vein restenosis-related reintervention, respectively. Preoperative severity score exceeding 5, 3, or 4 pulmonary veins involvement, and left inferior pulmonary vein involvement are independent risk factors for adverse outcomes.

To describe the experience of a series of paediatric patients with Fontan-associated liver dysfunction.

A retrospective study including 12 patients with Fontan-associated liver disease. Patients were selected from the single-ventricle program at a high-complexity centre in Colombia between 2001 and 2024.

During the study period, 108 patients were in the Fontan stage. Among them, 12 met the criteria for Fontan-associated liver disease (11.1%). The median age at extracardiac Fontan completion was 3.9 years, while the median age at Fontan-associated liver disease diagnosis was 14.5 years. Concomitant protein-losing enteropathy and/or plastic bronchitis were present in 33% of cases. Echocardiographic follow-up showed systolic dysfunction in 41% and diastolic dysfunction in 16% of patients. All patients exhibited some degree of valvular insufficiency, mild in 83.3% and moderate in 16%. Haemodynamic variables at the time of diagnosis did not show significant abnormalities.

Liver disease is a persistent concern in paediatric patients with Fontan physiology, posing diagnostic and therapeutic challenges. Healthcare professionals managing these patients should be aware of its early identification and appropriate treatment.

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.

We aimed to evaluate the clinical and laboratory characteristics and aetiological factors of patients who underwent pericardiocentesis for moderate to large pericardial effusion.

A total of 38 patients who underwent pericardiocentesis due to moderate-severe pericardial effusion and not related to cardiac surgery were included in the study.

The male-to-female ratio was 2.16, and found to be 7.5 in patients over 3 years of age. Mean age and body weight of the patients were 69.4 ± 74.9 months and 22.5 ± 22.4 kg. Dyspnoea (51.7%) was the most common complaint, followed by chest pain (37.9%). Tamponade was present in 23.7% of the patients. The largest diameter of effusion was 24.4 ± 10.4 mm. The amount of fluid drained was 279.24 ± 279 ml. Macroscopic appearance was serous in 12 (34.3%), and haemorrhagic in 18 (51.4%). No complication related to procedure was seen. Aetiology for efusion was infectious in 26%, idiopathic in 18%, iatrogenic in 11%, rheumatological in 11%, malignancy in 8%, cardiomyopathy in 8%, and other factors related in 18%. Of the 38 patients, 16 received nonsteroidal anti-inflammatory drugs (NSAID), and colchicine and corticostreoid were added in nine and two patients, respectively. A total of eight (21%) patients died during follow-up.

In conclusion, percutaneous pericardiocentesis can be applied safely and the underlying aetiology is decisive in the prognosis of the patient. Although pericardial effusion in children is often due to inflammation of the pericardium, it can develop as a finding of many local or systemic diseases that should be kept in mind.

The sutureless repair technique has been favoured due to its purported reduction in post-operative pulmonary venous obstruction rates. This study aims to compare the outcomes of conventional versus sutureless repair techniques in Total Anomalous Pulmonary Venous Drainage.

In this retrospective single-centre analysis (2012–2022), we evaluated children who underwent conventional or sutureless repair for isolated total anomalous pulmonary venous drainage, excluding complex cardiac anomalies and incomplete data. Patients were categorised into conventional (Group C, n = 58) and sutureless (Group S, n = 41) groups. Primary outcomes included mortality, morbidity, and post-operative complications. Statistical analysis included Mann–Whitney U, chi-square, and Fisher’s exact tests where appropriate.

Supracardiac type predominated in both groups (53.4% in Group C and 70.7% in Group S), with higher cardiac type frequency in Group C (24.1% versus 2.4%, p = 0.016). Early complications occurred in 58.5% versus 53.4% of cases in Groups S and C, respectively (p = 0.767). The mortality rate (17.2% versus 14.6%, p = 0.944) and post-operative pulmonary venous obstruction (21.2% versus 19.0%, p = 0.809) were higher in Group C, though not significantly. Mean cardiopulmonary bypass times were comparable between groups (105 versus 89 minutes, p = 0.424).

In this comprehensive analysis of paediatric Total Anomalous Pulmonary Venous Drainage repair, both conventional and sutureless techniques demonstrated comparable safety profiles and clinical outcomes. These findings suggest that surgical approach selection should be individualised based on patient characteristics and surgeon expertise. Further prospective studies with larger cohorts are needed to validate these observations.

Current research suggests that a small pulmonary artery can cause adverse events and reduce exercise capacity after the Fontan procedure. This study aimed to evaluate the impact of pulmonary artery size on early haemodynamic and laboratory variables after total cavopulmonary connection.

We reviewed all patients who underwent staged Fontan between 2012 and 2022. Pulmonary artery index before bidirectional cavopulmonary shunt and before total cavopulmonary connection was calculated according to Nakata and colleagues. We sought to analyse the impact of the pulmonary artery index on early haemodynamic and laboratory variables, including pulmonary artery pressure and mean arterial pressure 12 hours after extubation and lactate levels 6 hours after extubation.

A total of 263 patients were included. Median age and weight at total cavopulmonary connection were 2.2 (interquartile ranges: 1.8–2.7) years and 11.7 (interquartile range: 10.7–13.3) kg, respectively. Before that, all patients underwent bidirectional cavopulmonary shunt at a median age of 4.1 (interquartile range: 3.2–5.8) months. In the multivariable analysis, pre-bidirectional cavopulmonary shunt pulmonary artery index (p = 0.016, odds ratio 0.993), with a cut-off value of 154 mm2/m2 was an independent risk factor for a higher pulmonary artery pressure (> 17 mmHg). No variable was identified as a significant risk factor for lower mean arterial pressure (< 57 mmHg). Regarding lactate levels (> 4.5 mg/dl), pre-bidirectional cavopulmonary shunt right pulmonary artery index (p < 0.001, odds ratio 0.983), with a cut-off value of 70 mm2/m2 was identified as an independent risk factor.

In patients with staged Fontan palliation, a small pulmonary artery size before bidirectional cavopulmonary shunt and total cavopulmonary connection was a determinant factor associated with unfavourable early postoperative haemodynamics after total cavopulmonary connection.

Persistent pulmonary hypertension of the newborn is a serious disease with significant morbidity and mortality. Magnesium sulphate has been proposed as a potential treatment for persistent pulmonary hypertension of the newborn, but its efficacy remains unclear. The article aims to evaluate the effects of magnesium sulphate on persistent pulmonary hypertension of the newborn.

A comprehensive search of PubMed, Web of Science, Embase, and Cochrane Library was conducted to identify relevant studies. The primary outcomes were pulmonary artery pressure and oxygenation index, while secondary outcomes included mean blood pressure, alveolar-arterial oxygen difference, arterial partial pressure of oxygen (PaO2), arterial partial pressure of carbon dioxide (PaCO2), and arterial oxygen saturation. Statistical analysis was performed by Cochrane Review Manager 5.3.

The study analysed twelve studies involving 380 patients. Results indicated that magnesium sulphate treatment significantly reduced pulmonary artery pressure levels (MD −24.96, 95% CI −28.19 to −21.73, P < 0.0001) and mean blood pressure (MD −3.11, 95% CI −3.91 to −2.32, P < 0.0001) compared to pretreatment. Additionally, it led to a notable decrease in oxygenation index (P < 0.00001) and alveolar-arterial oxygen difference (P < 0.0001), while increasing PaO2 (P < 0.0001) and arterial oxygen saturation (P < 0.001). However, there was no significant effect on PaCO2 levels compared to pretreatment.

Magnesium sulphate is a valuable therapy for persistent pulmonary hypertension of the newborn. It markedly reduced pulmonary artery pressure, alveolar-arterial oxygen difference, and oxygenation index, while enhancing PaO2, and arterial oxygen saturation, with no impact on PaCO2 levels. Magnesium sulphate may also reduce mean blood pressure following a 2-hour treatment. Additional studies are necessary to further clarify its efficacy and potential side effects.

This study was registered with PROSPERO (CRD42024578092).

This study explored the prospective use of the Ages and Stages Questionnaires-3 in follow-up after cardiac surgery.

For children undergoing cardiac surgery at 5 United Kingdom centres, the Ages and Stages Questionnaires-3 were administered 6 months and 2 years later, with an outcome based on pre-defined cut-points: Red = 1 or more domain scores >2 standard deviations below the normative mean, Amber = 1 or more domain scores 1–2 standard deviations below the normal range based on the manual, Green = scores within the normal range based on the manual.

From a cohort of 554 children <60 months old at surgery, 306 participated in the postoperative assessment: 117 (38.3%) were scored as Green, 57 (18.6%) as Amber, and 132 (43.1%) as Red. Children aged 6 months at first assessment (neonatal surgery) were likely to score Red (113/124, 85.6%) compared to older age groups (n = 32/182, 17.6%). Considering risk factors of congenital heart complexity, univentricular status, congenital comorbidity, and child age in a logistic regression model for the outcome of Ages and Stages score Red, only younger age was significant (p < 0.001). 87 children had surgery in infancy and were reassessed as toddlers. Of these, 43 (49.2%) improved, 30 (34.5%) stayed the same, and 13 (16.1%) worsened. Improved scores were predominantly in those who had a first assessment at 6 months old.

The Ages and Stages Questionnaires results are most challenging to interpret in young babies of 6 months old who are affected by complex CHD.