Pulmonary atresia with ventricular septal defect (PA/VSD) is a complex cyanotic CHD that requires an early diagnosis for optimal management and outcomes. The purpose of this study is to evaluate the effectiveness of an inter-hospital management protocol utilising the prenatal CHD diagnosis for achieving favourable postnatal outcomes in PA/VSD patients in Vietnam.

We described the protocol implemented between two tertiary medical settings in Vietnam for the prenatal diagnosis and postnatal management of PA/VSD infants. All PA/VSD patients with prenatal diagnosis between January 2016 and December 2022 were retrospectively reviewed. The primary outcome was postnatal survival, and the secondary outcome was the presence of major morbidities such as bleeding or the need for Extra Corporeal Membrane Oxygenation (ECMO) after total repair.

During the study period, 35 PA/VSD patients were identified including 29 infants who underwent surgical correction utilising a valved conduit and 6 infants who are still waiting for the next evaluation after the palliative surgery. No death prior to the surgery occurred. For 29 patients, one hospital death happened, two patients required ECMO initially in their postoperative course but both survived, one late mortality due to pneumonia, and three reoperations were due to conduit failure. In the mean follow-up time of 2.92 (0.51–7.92) years, all survivors had completed follow-up.

Our protocol including a multidisciplinary management and a close follow-up has shown promising short-term results in achieving favourable postnatal outcomes for PA/VSD patients.

CHD is a major risk factor for acute ischaemic stroke in paediatric patients due to endothelial changes from surgically manipulated vessels, prosthetic material, flow stasis in variable circulations, and hypercoagulability from chronic cyanosis. Stroke recognition in critically or chronically ill patients is challenging, yet rapid identification allows for mechanical thrombectomy to restore cerebral blood flow, particularly in those ineligible for thrombolysis or beyond its therapeutic window. We present a case series highlighting the importance of prompt stroke diagnosis and the role of mechanical thrombectomy in paediatric CHD patients, including children as young as four.

We conducted a single-centre retrospective chart review of paediatric CHD patients who experienced thromboembolic stroke and underwent mechanical thrombectomy from July 2018 to March 2024. Data collected included age, stroke territory, maximum Paediatric NIH Stroke Scale (PedNIHSS) score, pre-thrombectomy neurological deficits, and post-thrombectomy outcomes using thrombolysis in cerebral infarction (TICI) scores.

Four CHD patients underwent mechanical thrombectomy for thromboembolic stroke (Table 1). They exhibited diverse cardiac anatomies, including two-ventricle and single-ventricle physiology, with a wide age range at presentation.

Stroke presentation in CHD patients is variable, necessitating a high index of suspicion. Mechanical thrombectomy is safe and effective in patients as young as four, with no haemorrhagic complications in this series. Further research is needed to develop tailored stroke management guidelines for paediatric CHD patients, particularly younger children and those ineligible for thrombolysis.

Children with CHD are at risk of neurodevelopmental impairment. Modifiable risk factors associated with hospitalisation that could impact neurodevelopment include being left alone for long periods of time with minimal interaction or opportunity to engage in developmentally appropriate play. Volunteers are an underutilised resource to help the medical team and families support neurodevelopment in cardiac care. Our Cardiac Inpatient Neurodevelopmental Care Optimization or CINCO team aimed to develop a volunteer programme specific to paediatric cardiac inpatient units.

CINCO volunteers were recruited from the hospital volunteer pool and, in 2022, partnered with the University of Colorado to recruit health profession-interested students from under-represented backgrounds. All underwent hospital volunteer orientation and CINCO-specific training with cardiac child life, including education and shadowing. Volunteers completed an activity log and provided qualitative feedback.

Between September 2021 and October 2024, 43 volunteers were onboarded and worked a total of 754 shifts. There were 2310 patient interactions, with an average of 3 patients seen per shift. Volunteers held patients 1231 times, played with patients 1230 times, and read to patients 780 times.

A dedicated cardiac volunteer programme is a feasible, low-cost, and low-risk way to enhance neurodevelopmental care for inpatient children with CHD. When parents or caregivers are not present, volunteers participate as therapy extenders and may offset the care burden for nurses. Furthermore, allowing parents breaks may support their mental health, and increasing neurodevelopmental stimulation through volunteer interactions may mitigate disadvantageous aspects of a hospitalisation for neurodevelopment.

To determine the feasibility of developing and implementing a multidisciplinary bootcamp for early-career Paediatric Cardiac Critical Care practitioners.

A one-day pre-conference bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022.

Physicians, fellows, nurses, advanced practice registered nurses, and respiratory therapists who work primarily in paediatric cardiac critical care units.

A modified Delphi needs assessment with interprofessional content experts for the development of a mixed didactic and simulation-based bootcamp at the Pediatric Cardiac Intensive Care Society Annual Meeting in December 2022, with pre- and post-testing to evaluate knowledge gain and additional surveys to assess perceived value.

Eighty-three course participants came from a variety of professions and represented institutions nationally and internationally. Most participants (77%) had two or more years of professional experience, aligning with the bootcamp’s focus on advanced learners. The bootcamp received strong participant evaluations: 84.1% (37/44) strongly agreed that the bootcamp improved their clinical knowledge. Ninety-seven percent (43/44) reported increased confidence in the ability to care for paediatric cardiac critical care patients. The bootcamp demonstrated a significant improvement in participant knowledge, with pre-test scores averaging 54.9% (95% CI: 49.9–59.9) compared to post-test scores of 64.5% (95% CI: 59.7–69.2), achieving statistical significance (p < 0.05).

Our pilot bootcamp has shown an improvement in immediate knowledge retention with valuable insights gathered to enhance future bootcamps. The results of this advanced interprofessional bootcamp evaluation will inform future iterations for providers in paediatric cardiac critical care.

Data on aortic valve outcomes following surgical repair of doubly committed subarterial ventricular septal defect remain limited.

This retrospective study included doubly committed subarterial ventricular septal defect patients who underwent surgical repair at our centre from 2013 to 2023. The primary outcome was the incidence of new-onset aortic regurgitation during follow-up.

A total of 320 patients were included, with a median age of 2.0 (0.9–7.2) years. Among them, 289 patients underwent surgical repair alone (repair group), and 31 received additional aortic valve surgery (repair + aortic valve surgery group). Preoperatively, 58 (18.1%) patients exhibited aortic regurgitation ≥ mild (10.7% in the repair group vs 87.1% in the repair + aortic valve surgery group, P < 0.001). The overall median follow-up was 40.5 (16.0–72.0) months. At the last follow-up, 23 (7.4%) patients had aortic regurgitation ≥ mild (3.8 vs 52.2%, P < 0.001), and 6 (1.9%) had aortic regurgitation > mild (0.3 vs 21.7%, P < 0.001). Sixteen (5.1%) patients developed new-onset aortic regurgitation during follow-up (1.7 vs 47.8%, log-rank P < 0.001), and 6 (1.9%) of them developed new-onset aortic regurgitation > mild (0.3 vs 21.7%, log-rank P < 0.001). Age, ventricular septal defect size, preoperative aortic regurgitation > mild, and maximum aortic valve flow velocity (AVmax) were related to concurrent aortic valve surgery and new-onset aortic regurgitation.

Based on our retrospective data, the mid-term aortic valve outcomes after doubly committed subarterial ventricular septal defect repair were relatively satisfactory, with a low incidence of new-onset aortic regurgitation during follow-up. However, aortic valve outcomes for patients who received concurrent aortic valve surgery were less satisfactory.

Neonatal aortic thrombosis, though rare, is a critical condition with high morbidity and mortality. Spontaneous thrombosis is uncommon, complicating diagnosis due to its similarity to aortic coarctation. Standard treatments include heparinisation and thrombolysis, but severe cases may require interventional or surgical thrombectomy.

This case series examines interventional therapy in neonates with spontaneous aortic thrombosis, focusing on reduced systolic function or organ failure, and evaluates off-label thrombectomy devices.

Three neonates with spontaneous abdominal aortic thrombosis treated at Hannover Medical School between 2017 and 2023 were reviewed. Clinical presentation, diagnostics, treatments, and outcomes were analysed. Treatment involved therapeutic anticoagulation, systemic thrombolysis and interventional thrombectomy using off-label devices. A literature review on interventional thrombectomy in neonates was conducted.

The first neonate, with severe multi-organ failure, died after thrombectomy. The second recovered fully with systemic thrombolysis. The third, critically ill, recovered following successful thrombectomy and thrombolysis. Off-label use of the Amplatzer Vascular Plug II proved more effective than the Amplatzer Piccolo PDA Occluder for managing organised thrombus. A literature review identified seven cases of neonatal aortic thrombectomy using various devices, highlighting interventional thrombectomy as a promising treatment for neonates with cardiac decompensation or imminent organ failure.

We propose a diagnostic approach for newborns with diminished or absent femoral pulses without structural heart or vascular disease. Interventional thrombectomy is a viable option for neonates with spontaneous aortic thrombosis in life-threatening situations involving decreased systolic function or organ failure. Further studies are needed to evaluate safety and long-term outcomes of catheter-based interventions.

Although global knowledge on paediatric cardiomyopathies has advanced, prospective cohort studies from Brazil, particularly those integrating clinical and genetic data, remain limited.

To describe the clinical and genetic characteristics of paediatric cardiomyopathy patients and identify mortality predictors in a metropolitan region of Brazil.

Prospective observational study of paediatric patients with cardiomyopathies. Clinical data, genetic findings, and survival were analysed using Kaplan–Meier curves.

A total of 45 cases, male predominance (55.6%), and mean age at diagnosis of 6.5 years. Dilated and hypertrophic cardiomyopathy were the most common (33.3%). The main reason for diagnosis was the investigation of cardiovascular symptoms (60.9%). Genetic investigation occurred in 66.6%, a positivity rate of 60%. Multi-organ/system involvement was significantly associated with a positive genetic result (77.7%, p = 0.017). Mortality was 11.1%; survival was significantly lower in the following conditions: ejection fraction < 30% (p < 0.0001), functional class III/IV (p < 0.0001), heart failure (p = 0.0091), use of three or more cardiovascular medications (p < 0.001), N-Terminal Pro-B-Type natriuretic peptide >1000pg/mL (p = 0.004), and heart transplant indication (p < 0.001).

These findings provide novel data in Brazil, highlight a high rate of positive genetic test, particularly among patients with systemic involvement and identify key clinical predictors of mortality to guide risk stratification and care.

Right ventricular outflow tract stenting is a palliative treatment option in symptomatic infants with tetralogy of Fallot or with pulmonary atresia with ventricular septal defect. Predominantly bare metal stents are used for this procedure. The authors sought to assess the efficacy and safety of using the covered coronary stent grafts for the right ventricular outflow tract stenting.

Between November 2017 and July 2021, the covered coronary stent graft was used to widen the right ventricular outflow tract in 20 symptomatic patients (pulmonary atresia with ventricular septal defect n = 5, tetralogy of Fallot n = 15).

All stent grafts were implanted successfully. The median time of palliation was 156 (43–1578) days. Eleven patients required stent redilation. Fifteen patients required additional stent implantation to relieve a proximal obstruction in the right ventricular outflow tract. There were three complications observed: right ventricular outflow tract perforation (n = 1), stent embolisation (n = 1), and main pulmonary aneurysm (n = 1). Oxygen saturation improved immediately after the procedure. During the follow-up time, all stents were patent, and we observed a significant increase in the diameters of the pulmonary arteries. Sixteen patients had corrective surgery performed with complete and easy removal of the implanted stents.

Stenting of the right ventricular outflow tract with stent grafts was safe and effective and provided a durable method of palliation. Utilisation of the covered coronary stent graft facilitated surgical removal of the implanted stent during the surgical correction.