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.

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.

Single ventricle patients undergoing bidirectional Glenn palliation subjectively experience increased post-operative agitation and discomfort. This is presumed to be secondary to increased intracranial pressure due to physiologic changes. This state of discomfort has been dubbed the “Glenn headache.” The purpose of this study was to determine whether sedation and analgesia requirements were higher in post-operative bidirectional Glenn patients than those of similar age who undergo tetralogy of Fallot or ventricular septal defect repairs.

Retrospective chart review was performed. Medication use, demographic, and haemodynamic data were collected. Comparisons between all groups were performed using the chi-squared test, Fisher’s exact test, Kruskal–Wallis test, and Dunn test.

A total of 96 patients met inclusion criteria. Thirty-nine patients (40.3%) underwent bidirectional Glenn, 36 patients (37.5%) tetralogy of Fallot repair, and 21 patients (21.9%) ventricular septal defect closure. When comparing as needed (PRN) morphine, lorazepam, and oxycodone boluses between the three groups, patients who underwent bidirectional Glenn received significantly more doses in comparison with the other two groups (all p < 0.001). The total dose of morphine (p = 0.001) and dexmedetomidine infusions (p < 0.001) were also higher in the bidirectional Glenn group than in ventricular septal defect patients, but equivalent to tetralogy of Fallot patients. There was no significant difference in post-operative intubation status or total hours of mechanical ventilation when comparing bidirectional Glenn patients to those who underwent both tetralogy of Fallot and ventricular septal defect repairs.

Study demonstrates that patients who undergo bidirectional Glenn have higher analgesic and sedation requirements than similar aged patients who undergo tetralogy of Fallot or ventricular septal defect repairs.

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.

With more than 1 million children in the United States living with a heart defect or condition, it is important to identify interventions that may minimise the long-term impacts of repeated medical surveillance and care. Thus, the purpose of this quasi-experimental study was to examine relationships between facility dog intervention and young children’s anxiety during outpatient echocardiogram.

Participants were seventy children aged 18 months to 8 years undergoing echocardiogram in a paediatric cardiology clinic. Child anxiety was scored by a trained nurse observer pre- and post-procedure using the modified Yale Preoperative Anxiety Scale. Facility dog intervention included individualised play, positioning, therapeutic conversation and touch, and emotional support throughout to promote coping and compliance. Parents and staff completed a post-procedural perceptions survey about their experiences.

Paired samples t-tests demonstrated child anxiety levels were significantly lower post-procedure compared to pre-procedure (Z = −3.974, p < .001). This direction held for nearly all participants; however, those with prior echocardiogram history demonstrated significantly higher anxiety levels at the pre-procedural timepoint (z = −2.442, p = .015). Caregivers (97.2%) and staff (87.9%) agreed or strongly agreed that facility dog intervention was helpful in this context.

Facility dog intervention was associated with a significant reduction in young children’s anxiety across procedural timepoints in outpatient echocardiography. The intervention was perceived as helpful by families and staff; no workflow changes or barriers were noted. Thus, facility dog intervention may be a well-received and promising care innovation for this vulnerable chronic population.

This study aimed to evaluate school-age neurodevelopmental outcomes among children with single ventricle heart disease who underwent neonatal Norwood operation with regional cerebral perfusion compared to deep hypothermic circulatory arrest. Additionally, we aimed to identify predictors of school-age development, including early developmental measures.

Patients enrolled in a prospective randomised trial of infants with single ventricle heart disease undergoing the Norwood operation with either regional cerebral perfusion or deep hypothermic circulatory arrest were included. For the same cohort of patients, this study performed neurodevelopmental testing at 5 years and 10 years of age. At 5 years, a comprehensive neuropsychological evaluation was performed. At 10 years, parent report instruments were used to measure participants’ behaviour and executive function.

Forty-one patients at 5 years of age and 33 patients at 10 years of age completed neurodevelopmental evaluation. There were no significant differences in neurodevelopmental scores between the regional cerebral perfusion and deep hypothermic circulatory arrest groups at either 5 or 10 years. At 5 years of age, the average full scale intelligence quotient (IQ) was 93.4 ± SD18.8. The Bayley Scale of Infant Development Psychomotor Developmental Index (r = 0.68, p < .0001) and mental developmental index (r = 0.64, p < .0001) at 1 year positively correlated with the full scale IQ at 5 years.

Neurodevelopment is delayed in patients with single ventricle heart disease. Neurodevelopmental outcomes at school age did not differ based on the perfusion strategy for the Norwood operation. Mental and psychomotor developmental indices at 1 year are predictive of early school-age measures.

Congenital aortic valvar disease represents a heterogeneous population with suboptimal surgical repair or replacement outcomes. We assess our approach and short-term outcomes in this population using cardiac CT evaluation for personalised surgical planning and execution.

We assessed patients who underwent aortic valvar surgery from February 2022 to August 2024. Pre-surgical evaluation included cardiac CT with quantitative assessment of the leaflet geometry and measures of leaflet coaptation. A standardised approach towards surgical execution guided by this assessment was established and followed.

Seventy-three patients underwent surgery at a median age of 26.0 years (interquartile range 19–44), 65.8% males. Forty-eight patients (65.8%) underwent some form of aortic valvar repair, with 22 of these 48 patients undergoing a valve-sparing aortic root replacement. The remaining 25 patients (34.2%) underwent some form of aortic valvar replacement. Mean post-surgical follow-up was 4.2 ± 6.1 months. Moderate or greater aortic regurgitation was present in 45 patients (61.6%) pre-operatively versus 2 patients (2.7%) post-operatively (p-value < 0.001). The peak and mean gradient improved from 33.2 ± 31.3 mmHg and 16.9 ± 10.7 mmHg pre-operatively, to 16.9 mmHg±10.7 mmHg and 9.5 ± 6.4 mmHg post-operatively (p-value < 0.001).

The heterogeneity and complexity of the dysfunctional and/or dilated (neo-)aortic root encountered in those presenting for surgery necessitates a methodical, detailed three- and four-dimensional assessment. By applying such an approach, we have aimed to standardise not only the assessment, but also description and surgical execution in this challenging patient population. Excellent short-term results have been achieved, necessitating long-term follow-up to understand the potential benefits towards this personalised approach.

Neonates with ductal-dependent CHD rely on the patency of the ductus arteriosus to maintain circulation. Alprostadil is utilised to maintain ductal patency, although optimal dosing has not been determined. This study aims to describe alprostadil dosing in neonates with ductal-dependent CHD.

This is a single-centre retrospective study including neonatal patients with ductal-dependent CHD who received alprostadil from January 2015 to December 2015 (cohort 1) and January 2021 to December 2021 (cohort 2). The primary objective was to describe alprostadil dosing in the two study periods. Secondary objectives included clinical outcomes and adverse events associated with different alprostadil dosing strategies.

Sixty-five patients met eligibility for inclusion in this study: thirty-eight patients in cohort 1 and twenty-seven patient s in cohort 2. Baseline demographics were similar between cohorts. Initial alprostadil dosing in cohort 1 and cohort 2 was 0.006 mcg/kg/min and 0.025 mcg/kg/min (p = < 0.001), respectively. Patients in cohort 2 were found to have a higher incidence of apneic events, apneic events requiring respiratory support, and the incidence of fever ≥38 °C.

In this single-centre study, we report that higher doses of alprostadil were associated with an increased risk of adverse events, which should be validated by prospective multicentre studies.

Double-outlet right ventricle is a complex congenital cardiac anomaly in which both great arteries arise predominantly from the right ventricle. Accurate anatomical evaluation is critical for surgical planning. While transthoracic echocardiography is commonly used for intracardiac assessment, its limitations in visualising extracardiac structures highlight the need for additional imaging modalities like CT angiography.

To compare the diagnostic accuracy and clinical utility of transthoracic echocardiography and CT angiography in the pre-operative evaluation and surgical planning of infants with double-outlet right ventricle.

This retrospective, single-centre study included 78 infants diagnosed with double-outlet right ventricle. All patients underwent both transthoracic echocardiography and CT angiography before surgical intervention. Imaging findings were compared with intraoperative surgical results to assess sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy for each modality.

CT angiography was significantly more effective than transthoracic echocardiography in identifying extracardiac anomalies such as coronary artery anomalies (p = 0.014), aortic arch hypoplasia (p = 0.024), aortopulmonary collateral vessels (p = 0.039), anomalous pulmonary venous connections (p = 0.001), and persistent left superior vena cava (p = 0.001). In contrast, transthoracic echocardiography was more sensitive in detecting small ventricular septal defects (p = 0.035).

Transthoracic echocardiography remains the first-line modality for intracardiac evaluation in double-outlet right ventricle, but CT angiography provides superior visualisation of extracardiac structures. The combined use of both imaging techniques improves diagnostic accuracy and surgical planning, supporting better outcomes in the management of CHD.

This study aimed to evaluate the predictive accuracy of Paediatric Risk of Mortality-III, Paediatric Index of Mortality-II, and Paediatric Logistic Organ Dysfunction scoring systems for major adverse events following congenital heart surgery.

This prospective observational study included patients under 18 years of age who were admitted to the ICU for at least 24 hours postoperatively following congenital heart surgery. Major adverse events were defined as a composite of 30-day mortality, ICU readmission, reintubation, acute neurologic events, requirement for extracorporeal membrane oxygenation, cardiac arrest requiring cardiopulmonary resuscitation, need for a permanent pacemaker, acute kidney injury, or unplanned reoperation.

A total of 116 patients, with a median age of 17.5 months (interquartile range: 5.4–60.0) were included in the study. Major adverse events occurred in 34 patients (29.3%). Paediatric Risk of Mortality-III (11.5 [8.0–18.8] vs. 7.0 [2.3–11.0]; p = 0.001), Paediatric Index of Mortality-II (3.8 [2.8–6.6] vs. 2.2 [1.7–2.8]; p < 0.001), and Paediatric Logistic Organ Dysfunction (12.0 [10.0–21.0] vs. 1.0 [1.0-10.0]; p < 0.001) scores were significantly higher in patients with major adverse events than in those without. The Paediatric Logistic Organ Dysfunction score (area under the curve 0.83; 95% confidence interval: 0.74–0.92) demonstrated the highest discrimination capacity compared to Paediatric Risk of Mortality-III (area under the curve 0.70; 95% confidence interval: 0.60–0.81) and Paediatric Index of Mortality-II (area under the curve 0.77; 95% confidence interval: 0.66–0.88) with good calibration (Hosmer–Lemeshow p > 0.05 for all). Based on the logistic regression model evaluation metrics, Paediatric Logistic Organ Dysfunction demonstrated better performance in predicting major adverse events compared with Paediatric Risk of Mortality-III and Paediatric Index of Mortality-II.

The Paediatric Logistic Organ Dysfunction score outperformed the Paediatric Index of Mortality-II and Paediatric Risk of Mortality-III scores in predicting major adverse events in paediatric patients admitted to the ICU after congenital heart surgery.

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.

Contemporary management of aortic coarctation in adults is primarily by percutaneous intervention; however, this is not always possible. Intrapericardial ascending-to-descending aortic grafting is an alternative approach in adults with complex aortic coarctation. It can be performed off-pump and allows for a simultaneous treatment of concomitant cardiac lesions. Our aim was to examine the indications and the clinical long-term outcomes of intrapericardial ascending-to-descending aortic grafting in adults with aortic coarctation.

We retrospectively reviewed demographic, clinical, surgical, and follow-up data of patients who underwent intrapericardial ascending-to-descending aortic grafting at a single tertiary centre between September 1994 and November 2016.

Ten patients were identified. Indications were primary intervention (n = 4), re-coarctation (n = 5), and iatrogenic coarctation of aorta after stent grafting (n = 1). There were no peri-operative deaths. During a mean follow-up of 13.6 ± 9.4 years, one patient died 11 years after surgery, unrelated to intrapericardial ascending-to-descending aortic grafting. All grafts remained patent with no anastomotic pseudoaneurysms at latest follow-up.

The mean systolic blood pressure decreased from 154 ± 24 mmHg at baseline to 134 ± 20 mmHg at latest follow-up (p = 0.05), with the mean number of prescribed antihypertensive medications decreasing from 2.8 ± 1.5 preoperatively to 1.6 ± 0.5 (p = 0.11).

Intrapericardial ascending-to-descending aortic grafting is a safe and effective approach for adults with complex coarctation of aorta or re-coarctation not suitable for a percutaneous or conventional surgical approach. Improved blood pressure control and reduced anti-hypertensive use were observed long-term.

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.

Patients diagnosed with hypertension (HT) are at high risk for end-organ damage. With changing living conditions and access to healthcare facilities worldwide, the rate of diagnosis in childhood is increasing. In this study, healthy children were compared with a group of pediatric patients diagnosed with hypertension. Cardiac findings in the hypertensive group were compared at presentation and at six months. We aimed to determine the discriminatory value of epicardial adipose tissue (EAT) measurements as an early imaging marker for cardiac involvement in children with HT compared to healthy children and to determine its prognostic feature for HT treatment response.

Fifty-nine primary hypertension patients and 76 control patients aged 0-18 years were compared. EAT values measured between the healthy group and the patient group and at the beginning of treatment and subsequent follow-ups in the patient group were evaluated with M-mode measurements.

There was no difference between the groups in terms of sex, and age. EAT was found to be significantly higher in the patient group than in the healthy group. There was a statistically significant difference between the EAT measurements evaluated before and after treatment in the patient group.

Hypertension is an important cause of morbidity and mortality. Using EAT measurements as a noninvasive parameter may provide information about early cardiac involvement due to HT. EAT is promising as an imaging marker that can be used in diagnosis and follow-up.