Physiologic changes in the peripartum period put women with CHD at increased risk for morbidity. This study examines factors associated with peripartum complications and length of stay compared to patients without CHD.

This single-institution retrospective case-control study included women with CHD (2000–2017) and a control population without CHD. A review of clinical and echocardiographic data was used to assign baseline characteristics, disease severity, and adverse outcomes. Primary outcomes were composite variables of cardiac and obstetric adverse events, along with peripartum length of stay. The relationship between maternal CHD, baseline characteristics, and peripartum adverse events was evaluated by multivariable regression.

The cohort and control groups included 162 deliveries among 113 women and 321 deliveries among 321 women, respectively. Cardiac complications, including arrhythmia, heart failure, pulmonary oedema, and thromboembolic events, occurred in 8.6% of the cohort (RR 2.52, 95% CI 1.17–5.42), with the most common event being arrhythmia. Obstetric events, such as caesarean delivery, assisted vaginal delivery, preterm birth, and pre-eclampsia, occurred in 67.9% versus 56.1% in the control group (RR 1.21, 95% CI 1.05–1.40). In multivariable models, increasing age was associated with increased composite cardiac events. Length of stay was longer in the cohort group (p < 0.001) and significantly associated with modified World Health Organization classification (p = 0.016).

Women with CHD experience increased cardiac and obstetric morbidity compared to controls during peripartum admission. Those with CHD have longer hospital stays around delivery, which is associated with disease severity.

Heart failure is the most common complication of congenital heart disease (CHD), characterized by high morbidity and mortality. Early recognition and intervention are crucial for improving outcomes in pediatric patients with CHD and heart failure. This study aimed to analyze the clinical value of N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I in pediatric patients with CHD and heart failure.

Ninety-eight pediatric patients with CHD complicated by heart failure were included in the Case Group, and 61 pediatric patients with CHD were included in the Control Group. The Case Group was categorized into subgroups based on the cardiac function of patients: grade I (n = 35), grade II (n = 40), and grade III (n = 23). Left ventricular ejection fraction was collected from pediatric patients in the case group. The correlation of the cardiac functional indicators with N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I levels was assessed using Pearson correlation analysis.

The serum levels of N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I in pediatric patients at grade III of cardiac function were significantly elevated compared to those at grade II, and these levels in patients at grade II were higher than those at grade I (P < 0.05). The left ventricular ejection fraction of pediatric patients in the Case Group were markedly negatively correlated with N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I (r = − 0.6807, r = −0.3013, r = −0.5412, P < 0.0001).

N-terminal pro-brain natriuretic peptide, adrenomedullin, and cardiac troponin I have a certain diagnostic value in determining concurrent heart failure in pediatric patients with CHD.

Congenitally corrected transposition of the great arteries is a rare congenital cardiac condition with varied presentations, complicating treatment decisions. This study evaluates the impact of medical management, physiologic repair, and anatomic repair on health-related quality of life.

A cross-sectional follow-up was conducted on 50 congenitally corrected transposition of the great arteries patients from a cohort of 240 at Cleveland Clinic (1995-2020). Health-related quality of life was assessed using MacNew and PROMIS-10 questionnaires. Echocardiographic data on systemic atrioventricular valvular regurgitation and systemic ventricular dysfunction were analysed. A time-varying coefficient model evaluated these factors’ impact on health-related quality of life.

Anatomic repair had significantly higher PROMIS-10 Physical T-scores compared to physiologic repair (median 50.9 vs 41.6, p = 0.04). MacNew Social scores were significantly higher for medical management compared to physiologic repair (median 6.8 vs 6.0, p = 0.02). Echocardiographic analysis revealed that systemic ventricular dysfunction had a stronger immediate impact on health-related quality of life. Systemic atrioventricular valve regurgitation showed a delayed negative effect, significant at 6-10 years after echo, although this effect gradually decreased over time. Anatomic repair patients had better systemic ventricular function (84.6% normal), and less systemic atrioventricular valve regurgitation (69.2% had none) compared to medical management and physiologic repair groups.

Anatomic repair improves health-related quality of life in congenitally corrected transposition of the great arteries patients, with physiologic repair showing some gains over time. Health-related quality of life data should provide important guidance regarding treatment decisions, especially in well-balanced congenitally corrected transposition of the great arteries patients.

Reports of paediatric cardiac neurodevelopmental programmes are predominately limited to high-volume surgical centres. Regional characterisation of neurodevelopmental care practices across multiple centres both with and without cardiac surgery programmes has not yet been described.

Assess cardiac neurodevelopmental infrastructure and approach across New England to describe regional support for children with CHD.

A 16-item survey assessing inpatient and outpatient neurodevelopmental care practices was sent electronically via REDCap to all member institutions of the New England Congenital Cardiology Association Health Disparities Working Group. Centres were characterised by surgical capability, annual volume, and academic affiliation. Descriptive statistics and Fisher’s exact test were used for comparisons.

The survey response rate was 90% (9/10). Participating centres included non-surgical academic centres (55%), small surgical centres (22%) and medium-large surgical centres (22%). Surgical centres typically refer their patients to internal neurodevelopmental services (100%) and rely on automatic referral processes (100%). Non-surgical centres predominately refer to the institution where cardiac surgery was performed (80%) compared to engaging local teams (20%) and depend on family or physician-initiated referral (100%) to schedule neurodevelopmental services. While none of the non-surgical centres surveyed have cardiac-specific neurodevelopmental programmes, all have other developmental programmes that accommodate children with CHD.

Varying neurodevelopmental infrastructure and resources are reported across New England. Academic, non-surgical centres may have infrastructure for neurodevelopmental follow-up despite not having cardiac neurodevelopmental programmes. Collaboration between surgical and non-surgical cardiac centres in New England may be leveraged to promote neurodevelopmental care closer to home.

Integrating psychosocial health services into paediatric surgical specialty care is essential for addressing behavioural and psychological aspects of illness and reducing healthcare disparities. This is crucial for patients facing CHD, who are at higher risk for depression, anxiety, and attention-deficit/hyperactivity disorder, which is significantly influenced by their caregivers’ mental well-being.

The Pediatric Psychosocial Preventative Health Model framework was utilised by a psychosocial team to assess biopsychosocial needs in CHD patients during their first cardiac surgery evaluations. Patient and family needs were categorised into universal, targeted, and clinical tiers, allowing for responsive interdisciplinary services. Screening tools such as the Psychosocial Assessment Tool, Pediatric Quality of Life Inventory, and Depression, Anxiety, and Stress Scales were used during initial consultations to guide appropriate interventions and referrals.

Universal-tier patients received comprehensive support focused on preventive measures, resource access, and education to promote resilience. Targeted-tier care involved intensive, collaborative efforts, providing specialized psychological evaluations, and one-on-one time with experts. Clinical-tier families required specialised, intensive interventions such as advanced cognitive behavioural therapy and medication management. The Pediatric Psychosocial Preventative Health Model framework and psychosocial team workflow allow for individualised management strategies, ensuring that each family received timely and appropriate interventions based on their unique needs.

Integrating psychosocial services into initial surgical evaluations is critical for addressing CHD patients’ psychological and social needs, promoting an interdisciplinary approach that enhances overall family functioning and well-being.

To highlight veno-venous collaterals and portosystemic shunts as possible causes of cyanosis requiring evaluation or treatment below the diaphragm in patients with single-ventricle physiology during staged palliation.

Cyanosis can complicate staged single-ventricle palliation and is often evaluated with imaging isolated to the thorax. As a result, lesions below the diaphragm, such as veno-venous collaterals and portosystemic shunts, may be missed. Identification is important since these lesions can be treated via transcatheter techniques.

This study is a retrospective, single-centre case series of six patients.

Two patients following stage 2 palliation had multiple small veno-venous collaterals connecting to a reconstituted azygos with retrograde flow to below the diaphragm. Accessing veno-venous collaterals closer to their entry points below the diaphragm was more optimal for occlusion. Two patients following stage 3 palliation had veno-venous collaterals originating below the diaphragm and connecting to the pulmonary veins. Both patients required infrarenal inferior vena cava contrast injection to identify the veno-venous collateral. The final two patients had heterotaxy with either stage 2 or 3 palliation and were found to have bilateral pulmonary arteriovenous malformations secondary to a portosystemic shunt diverting hepatic factor away from the lungs. All patients had successful device closure of their veno-venous collaterals or portosystemic shunts.

The six patients discussed demonstrate the importance of evaluating below the diaphragm in single-ventricle physiology patients presenting with cyanosis.

Pulmonary regurgitation leading to right ventricular enlargement may occur after repaired tetralogy of Fallot (rTOF) or balloon dilation for pulmonary valve stenosis. Cardiac magnetic resonance imaging (CMR) guidelines to identify the timing of valve replacement in rTOF are not necessarily applicable to isolated pulmonary regurgitation. This study aims to compare deformation parameters of isolated pulmonary regurgitation and rTOF at comparable right ventricular volume loads.

Adopting a quantitative retrospective analytic framework, CMR was performed in 44 patients (0–30 years), 22 in each of the isolated pulmonary regurgitation and rTOF study arms, matched for age (±12 months), and Right ventricular end-diastolic volume z-score (±1). Right ventricular longitudinal strain/strain rate and circumferential strain/strain rate were measured. Comparisons between groups were analysed using two-tailed T-tests and one-way ANOVA.

Both groups showed predominance of longitudinal over circumferential strain. Circumferential strain was significantly greater in rTOF compared to isolated pulmonary regurgitation (–26.5% versus –22.3%, p < 0.05). Longitudinal strain did not differ between groups. The longitudinal:circumferential strain ratio was significantly lower in rTOF compared to isolated pulmonary regurgitation (1.24 versus 1.53, p = 0.05). Circumferential and longitudinal strain rates did not differ between groups.

The right ventricles in rTOF demonstrate greater reliance on circumferential strain in response to increased volumes. The decrease in longitudinal:circumferential strain ratio suggests rTOF right ventricles display a greater adaptive response to the volume load than isolated pulmonary regurgitation, highlighting the importance of the relative contributions of both circumferential and longitudinal strain in order to understand the mechanisms of right ventricular dysfunction in pulmonary regurgitation.

Low neuromuscular fitness is documented in adolescents with CHD and may be associated with clinical and morphological factors, indicating the need to assess strength in this population.

To evaluate neuromuscular fitness with a multifactorial approach and its associations with other clinical and morphological factors in adolescents with CHD.

This is an observational, cross-sectional study with adolescents with CHD, aged between 10 and 18 years. Neuromuscular fitness was calculated by the sum of the z-scores of four strength tests. Clinical factors of CHD were assessed by medical records and questionnaire. The morphological factors assessed were waist-to-height ratio, sum of skinfolds, and upper arm muscle area. Descriptive statistics, analysis of covariance, and linear regressions were performed.

Sixty adolescents with CHD participated, aged 12,7 ± 2,1 years, 55% girls. Maximum isometric strength was inadequate in 33%, jump height (power) in 33%, abdominal muscle strength resistance in 78%, and upper limb muscle strength resistance in 27%. Neuromuscular fitness was inadequate in 89% (n = 53) of adolescents with CHD. In the unadjusted regression, neuromuscular fitness was associated with arm muscle area (β = 0,12; p = 0,02; R2adj = 0,08) and in the unadjusted and adjusted regression it was lower in cyanotic (vs. acyanotic) CHDs (β = −1,76; p = 0,03 R2adj = 0,24).

The findings reveal deficits in different presentations of musculoskeletal strength in a large proportion of adolescents with CHD, reinforcing the need to measure fitness from a broader perspective. Low muscle mass and the presence of cyanotic CHD may imply in reduced neuromuscular fitness in adolescents with CHD.

B lines in lung ultrasound are useful to diagnose heart failure in adults. This study was to done to correlate lung ultrasound score with heart failure severity in CHD.

Fifty infants with left to right shunt underwent Ross scoring, measurement of NT pro BNP and lung ultrasound. Lung was divided into 14 zones (7 on each side); each zone was scored as: No B lines = 0, less than 3 B lines = 1, 3-7 B lines = 2, > 7 /or confluent B lines = 3. Total score was calculated by adding all scores. A modified ultrasound score was calculated by excluding scores of 1. Relationship between ultrasound scores and heart failure was assessed. Receiver operator curve (ROC) was generated to find a cut-off of ultrasound score to detect moderate or severe heart failure.

No correlation was found between either total or modified lung ultrasound score with the modified Ross score or NT pro BNP. A cut-off of total lung ultrasound score of 11 gave an accuracy of 68% with area under the curve (AUC) of 0.64 to detect moderate or severe heart failure.

Utility of lung ultrasound in detecting severity of heart failure in infants with left to right shunts is limited at present due to its low accuracy and lack of standardised method of scoring.

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.

The impact of CHD on safe driving for adolescents is currently unknown. A prospective, qualitative descriptive study was conducted among adolescents with CHD to describe perceived barriers, facilitators, and impacts of CHD on safe, independent driving among adolescents.

Twenty-eight adolescents aged 15–19 years with CHD participated in virtual, semi-structured interviews in 2023. Adolescent interview data were analysed with conventional content analysis refined by Theoretical Domains Framework in NVivo software.

Mean participant age was 16.4 ± 0.23 years (57% male). Single ventricle physiology (25%) and septal defects (32%) were prevalent diagnoses among the study population. Most participants (92%) did not have driving restrictions.

Driving as a normal rite of passage for adolescents with CHD; and confident—but curious—about the impacts of CHD on driving. Adolescents felt confident that driving is not impacted by CHD. They were curious about the likelihood of cardiovascular emergencies and related symptoms while driving. Perceived barriers and facilitators to safe, independent driving were like what has been described in published literature among adolescents without CHD.

These findings celebrate the normalcy of driving during adolescence and reveal curiosities about the impacts of stress, anxiety, fatigue, and risks of heart attack and stroke on driving. Adolescents may look to CHD healthcare providers to help them learn about driving. These findings may inform the development of tools to facilitate meaningful conversations with adolescents regarding driving safety as part of the transition to adult CHD care.

Haemolysis is developing prominence in the setting of supporting increasingly complex children with heart failure with a ventricular assist device. The goal of this study is to better characterise haemolysis and its implications in children supported with pulsatile ventricular assist devices.

This is a single-centre retrospective review of 44 children who were supported by Berlin Heart EXCOR between January 2006 and June 2020. Patients were divided into major haemolysers and non-major haemolysers. Major haemolysers were defined as patients with lactate dehydrogenase > 500U/L (2.5x the upper limits of normal) with either total bilirubin > 2mg/dL (with predominantly indirect hyperbilirubinemia) or anaemia out of proportion to the clinical scenario more than three days following implantation of the ventricular assist device(s). Patient demographics, ventricular assist device factors, and outcomes, including end-organ function and mortality, were compared between major haemolysers and non-major haemolysers.

Forty-four patients supported by the Berlin EXCOR were included in the analytic cohort of the study: 27 major haemolysers and 17 non-major haemolysers. Major haemolysis was more common in those supported with single-ventricle ventricular assist device (i.e., VAD in the context of functionally univentricular anatomy) compared to those with biventricular hearts, p = 0.01. There were no patients with an isolated left ventricular assist device or isolated right ventricular assist device in our analytic cohort of 44 patients. Of the 19 patients with single-ventricle ventricular assist device, 84% (16/19) were major haemolysers. Of the 25 patients with a biventricular assist device, 44% (11/25) were major haemolysers. Major haemolysers and non-major haemolysers had a body surface area of 0.28 and 0.40, respectively (p = 0.01). Overall, survival to discharge from the hospital was 66% (n = 29/44). Survival to discharge from the hospital was 52% (14/27) in major haemolysers versus 88% (15/17) in non-major haemolysers, p = 0.02. Only 3 of the 27 with major haemolysis had severe haemolysis, that is, lactate dehydrogenase > 2000 and bilirubin above 10. Non-major haemolysers had a better improvement in creatinine clearance during ventricular assist device support, p < 0.0001. (During the same era of this study, 22 patients who were supported with Berlin Heart were excluded from the analytic cohort because they did not have any recorded measurement of lactate dehydrogenase. Seventeen of these 22 patients had no clinical evidence of haemolysis. Survival to discharge from the hospital in this excluded cohort was 86% [19/22].)

Major haemolysis in patients with pulsatile ventricular assist device is more likely with single-ventricle ventricular assist device support and smaller body surface area.

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.

Medical advances in the management of CHD have shifted the focus from childhood mortality to life-long morbidity; therefore, clinical research in paediatric cardiology tends to replace mortality outcomes with functional and patient-reported outcomes. Despite these advances, the stratification of disease severity using a simple and reproducible CHD classification has not been established. The aim was to determine which classification best predicts functional status in children with CHD, in terms of cardiopulmonary fitness.

This retrospective cohort study was assessed from a cohort study of 296 children (mean age 11.3 ± 3.1 years, 129 female), who underwent a baseline and final cardiopulmonary exercise tests with a mean follow-up of 4.1 ± 1.6 years.

Seven CHD classifications were identified, assessing anatomical, physiological, therapeutic, or functional parameters in foetal, paediatric, or adult CHD populations. The four-stage paediatric CHD disease severity classification established by Uzark et al. best predicted functional status at the final cardiopulmonary exercise test assessment, with an area under the receiver operating characteristics curves of 0.62 (0.55–0.69) for impaired cardiopulmonary fitness (maximum oxygen uptake (VO2max) or ventilatory anaerobic threshold < −1.64 Z-score). The best inter-judge classification agreement was also observed for Uzark classification, with a Kappa coefficient of 0.88 (0.78–0.98).

The CHD classification by Uzark is reliable in terms of functional status prediction and reproducibility in children with CHD. This simple classification may contribute to identifying children with CHD most at risk of cardiopulmonary fitness impairment and initiating early preventive cardiovascular interventions in paediatric cardiology.

Pulmonary oedema is a common complication after paediatric cardiac surgery, and it is linked to increased morbidity. Lung ultrasound has been recognised as an alternative to chest radiography, offering rapid and accurate diagnosis without exposure to ionising radiation.

The study aimed to investigate the association between the severity of pulmonary oedema identified through a B-lines score—a lung ultrasound score used to assess the severity of pulmonary oedema—and ventilatory outcomes in the postoperative period of cardiac surgery. It was a prospective single-centre cohort study conducted at a quaternary paediatric hospital. Patients up to 18 years old who underwent cardiac surgery for CHD were included. The primary outcome was extubation failure within 48 hours after surgery, and the secondary outcome was mechanical ventilation time.

Among the 131 included patients, 116 were extubated with an extubation failure rate of 18.3%. Patients with extubation failure were younger and had a longer duration of mechanical ventilation. A robust association between higher B-lines score on lung ultrasound within the first 24 hours after surgery and extubation failure was observed (p < 0.001). The correlation between B-lines score and mechanical ventilation time was positive, with stronger correlation preoperatively.

Severe pulmonary oedema detected through lung ultrasound in the first postoperative day of paediatric cardiac surgery show better accuracy to predict patients at greater risk of extubation failure and prolonged mechanical ventilation.

Membranous subaortic stenosis is a CHD with high recurrence-rate despite surgical treatment. This study investigated the outcome of operated patients and possible predictors for recurrence.

Retrospective review of all patients (n = 38) ≤ 18 years of age operated for membranous subaortic stenosis between 1994–2019 at Sahlgrenska University Hospital. The primary outcomes were recurrence, reintervention, and mortality. Predictors of recurrence and reintervention were secondary outcomes.

Median age (range) at diagnosis, initial intervention, and last follow-up were 2.3 (0.003–17.2), 5.3 (0.03–17.5) and 17.5 (3.6–20.4) years, respectively. Median follow-up time was 9.9 (0.01–19.5) years. 61% were males, and 53% had other associated CHD. 19 patients (56%) developed recurrence and 7 (21%) underwent reintervention. One patient died peri-operatively. Age <5 years at first intervention increased the likelihood of reintervention. Postoperative peak/mean gradients were higher in patients with disease recurrence.

The median echocardiographic peak-/mean gradients at initial diagnosis, pre-, postoperative, and at last follow-up were 61/36, 83/50, 16/8, and 19/17 mmHg respectively (p < 0.0001 pre/post). Pre-/postoperative peak gradients were linearly correlated, decreasing by 80% pre-/postoperatively (p < 0.01). Presence of symptoms and the preoperative peak gradient were positively associated (p < 0.001) with a peak gradient threshold value of > 90 mmHg. The distance between the subaortic stenosis membrane and the aortic valve was inversely correlated to the preoperative peak-gradient (p < 0.01).

Reintervention following surgical intervention of membranous subaortic stenosis is common. A positive correlation exists between high pre- and postoperative peak-gradient. A low postoperative peak gradient may be important in avoiding recurrence.