Education of paediatric advanced practice providers takes a generalist approach which lacks in-depth exposure to subspecialties like paediatric cardiac intensive care. This translates into a knowledge gap related to congenital cardiac physiology and management for APPs transitioning to the paediatric cardiac ICU.

A specialised interprofessional peer-reviewed curriculum was created and distributed through the Pediatric Cardiac Intensive Care Society. This curriculum includes a textbook which is complemented by a didactic and simulation review course. Course evaluations were collected following each course, and feedback from participants was incorporated into subsequent courses. Pediatric Cardiac Intensive Care Society partnered with the Pediatric Nursing Certification Board to develop a 200-question post-assessment (exam) bank.

From December 2017 to January 2022, 12 review courses were taught at various host sites (n = 314 participants). Feedback revealed that courses improved preparedness for practice, contributed to advanced practice provider empowerment, and emphasised the importance of professional networking. 97% of attendees agreed/strongly agreed that the course improved clinical knowledge, 97% agreed/strongly agreed that the course improved ability to care for patients, and 88% agreed/strongly agreed that the course improved confidence to practice. 49% of participants rated the course as extremely effective, 42% very effective, 6% moderately effective, and 3% as only slightly effective.

A standardised subspecialty curriculum dedicated to advanced practice provider practice in cardiac intensive care was needed to improve knowledge, advance practice, and empower APPs managing critically ill patients in the cardiac ICU. The developed curriculum provides standardised learning, increasing advanced practice provider knowledge acquisition, and confidence to practice.

We aimed to discuss our unit’s experience performing left ventriculotomies on children.

Between 2000 and 2022, we identified paediatric patients who required left ventriculotomy. Relevant information was gathered retrospectively.

There were eight patients who underwent surgical procedure including left ventriculotomy. The range of weight and age was between 4.5 and 50 kg and 5 months to 17 years, respectively. Left ventriculotomy was primarily performed for the excision of cardiac masses in all but one who had pseudoaneurysm repair. There were no deaths that occurred early or late. Pre-operative and post-operative ejection fractions and fractional shortening values were comparable. There was no arrhythmia detected post-operatively.

We conclude that an apical left ventriculotomy does not compromise the function of the left ventricle, even in young infants. In selected patients, it may be used safely for surgical access to the left ventricle.

Surgical site infection is an important concern due to its association with morbidity and mortality after paediatric cardiac surgery. The aim of this study was to present our approach and experience in the utilisation of a modified care bundle in a recently established paediatric cardiac surgical unit in the low-income region of Turkey.

Between 2019 and 2021, we identified children who underwent cardiac surgical procedures and retrospectively collected relevant demographic data, disease characteristics, operational data, Risk Adjustment For Congenital Heart Surgery (RACHS-1) scores, and post-operative factors such as morbidities, mortality, critical care, and in-hospital stay lengths. Surgical site infections and late infections were scanned.

Ninety-six patients (49 males, 47 females) underwent a total of 127 surgical procedures during the study period. Overall adherence to the protocol was 94%, 100%, and 96% in the pre-operative, intra-operative, and post-operative periods, respectively. There was no reported surgical site infection, and no late infection was encountered throughout the follow-up period.

We conclude that a low rate of surgical site infection, or even a rate of nil, is attainable through the utilisation of locally standardised guidelines for its prevention.

We aimed to evaluate the efficacy of combined (ibuprofen+paracetamol) medical therapy in cases of persistent haemodynamically significant patent ductus arteriosus that are resistant to standard medical monotherapy (ibuprofen and/or paracetamol) in this retrospective multi-centre study.

The combined therapy included the administration of 15mg/kg/dose of paracetamol every 6 h for 3 days and ibuprofen at an initial dose of 10mg/kg/dose followed by 5 mg/kg/dose every 24 h. After 2 days following the administration of the last dose, the researchers evaluated the efficacy of combined treatment by conducting an echocardiographic examination.

Of all 42 patients who received combined therapy, 37 (88.1%) patients exhibited closure of the haemodynamically significant patent ductus arteriosus without requiring surgical ligation. Patients who did not respond to combined therapy had a higher mean birth weight and gestational age compared to those who responded (p < 0.05).

The researchers believe the success of ibuprofen and paracetamol in haemodynamically significant patent ductus arteriosus treatment may be due to their synergistic efficacy and inhibition of the prostaglandin synthesis pathway through different enzymes. The results of our retrospective trial suggest that combination therapy with paracetamol and ibuprofen can be attempted when monotherapy is unsuccessful in treating haemodynamically significant patent ductus arteriosus, especially in centres without a surgical department.

Parents of infants with complex CHDs often describe their infants as especially fussy, irritable, and difficult to sooth, which together with the illness caretaking demands add to their stress. Little is known about how the behavioural style or temperament in the early months after discharge relates to parental quality of life. This study aimed to explore the associations between early infant temperament characteristics and parental quality of life in parents of infants with complex CHD.

This descriptive, cross-sectional study, utilised data collected in a previously described multisite randomised clinical trial in the United States. Multivariable linear regression models were used to examine the associations of interest.

Results demonstrated negative significant associations between most infant temperament subscales and parental quality of life. Higher scores on the Activity (β = −3.03, p = 0.021), Approach (β = −1.05, p = 0.021), Adaptability (β = −3.47, p = 0.004), Intensity (β = −2.78, p = 0.008), Mood (β = −4.65, p < 0.001), and Distractibility (β = −3.36, p = 0.007 were all significantly associated with lower parental quality of life scores, adjusting for parental dyadic adjustment, insurance type, number of medications, and number of unscheduled cardiologist visits.

Parental perceptions of infant’s difficult behavioural style or temperament characteristics appear to be associated with poorer quality of life in parents of infants with complex CHD post-cardiac surgery. Findings can be used in the screening process of families at potential risk of increased stress and poor illness adaptation and in the design of interventions to target parental mental health in this vulnerable patient population.

Health-related quality of life in children who have undergone the Ross procedure has not been well characterised. The aim of this study was to characterise health-related quality of life in this cohort and compare to children with other CHD.

In this cross sectional, single-centre study, health-related quality of life was assessed in patients who underwent a non-neonatal Ross procedure using the Pediatric Quality of Life Inventory. Ross cohort scores were compared with healthy norms, patients with CHD requiring no surgical intervention or had curative surgery (Severity 2, S2) and patients who were surgically repaired with ≥1 surgical procedure and with significant residual lesion or need for additional surgery (Severity 3, S3). Associations between Pediatric Quality of Life Inventory score and patient factors were also examined.

68 patients completed surveys. Nearly one-sixth of patients had overall scores below the cut-off for at-risk status for impaired health-related quality of life. There was no difference in overall health-related quality of life score between the Ross cohort and healthy children (p = 0.56) and S2 cohort (p = 0.97). Health-related quality of life was significantly higher in the Ross cohort compared to S3 cohort (p = 0.02). This difference was driven by a higher psychosocial health-related quality of life in the Ross cohort as compared to S3 cohort (p = 0.007). Anxiety scores were significantly worse in the Ross cohort compared to both S2 (p = 0.001) and S3 (p = 0.0017), respectively.

Children who have undergone a Ross procedure report health-related quality of life equivalent to CHD not requiring therapy and superior to CHD with residual lesions. Despite these reassuring results, providers should be aware of potential anxiety among Ross patients.

We investigated the efficacy and complication profile of intranasal dexmedetomidine for transthoracic echocardiography sedation in patients with single ventricle physiology and shunt-dependent pulmonary blood flow during the high-risk interstage period.

A single-centre, retrospective review identified interstage infants who received dexmedetomidine for echocardiography sedation. Baseline and procedural vitals were reported. Significant adverse events related to sedation were defined as an escalation in care or need for any additional/increased inotropic support to maintain pre-procedural haemodynamics. Minor adverse events were defined as changes from baseline haemodynamics that resolved without intervention. To assess whether sedation was adequate, echocardiogram reports were reviewed for completeness.

From September to December 2020, five interstage patients (age 29–69 days) were sedated with 3 mcg/kg intranasal dexmedetomidine. The median sedation onset time and duration time was 24 minutes (range 12–43 minutes) and 60 minutes (range 33–60 minutes), respectively. Sedation was deemed adequate in all patients as complete echocardiograms were accomplished without a rescue dose. When compared to baseline, three (60%) patients had a >10% reduction in heart rate, one (20%) patient had a >10% reduction in oxygen saturations, and one (20%) patient had a >30% decrease in blood pressure. Amongst all patients, no significant complications occurred and haemodynamic changes from baseline did not result in need for intervention or interruption of study.

Intranasal dexmedetomidine may be a reasonable option for echocardiography sedation in infants with shunt-dependent single ventricle heart disease, and further investigation is warranted to ensure efficacy and safety in an outpatient setting.

To investigate UK variability in prenatal and postnatal management strategy of right aortic arch and double aortic arch (RAA/DAA).

Online surveys were sent to senior physicians (consultants) of the National Fetal Cardiology Working Group regarding prenatal diagnosis, counselling, and perinatal management of antenatally diagnosed RAA/DAA and to the British Congenital Cardiovascular Association regarding postnatal management strategies.

There were 28 prenatal and 90 postnatal surveys completed. Prenatally, there was consensus for potential associated chromosomal/genetic anomalies, but there was variation in the risk quoted. Confidence in defining aortic arch morphology was reported by 43% (12/28) of fetal cardiologists. There was variation in what was felt to be possible symptoms/signs of a compressive vascular ring, postnatal investigation, postnatal management, follow-up duration of asymptomatic patients, and indications for surgical intervention.

This study has highlighted important areas for future research: improving accuracy of prenatal diagnosis, clarification of potential symptoms, optimal investigation strategies, and indications for surgery.

Pulmonary vasodilator therapy in Fontan patients can improve exercise tolerance. We aimed to assess the potential for non-invasive testing of acute vasodilator response using four-dimensional (D) flow MRI during oxygen inhalation.

Six patients with well-functioning Fontan circulations were prospectively recruited and underwent cardiac MRI. Ventricular anatomical imaging and 4D Flow MRI were acquired at baseline and during inhalation of oxygen. Data were compared with six age-matched healthy volunteers with 4D Flow MRI scans acquired at baseline.

All six patients tolerated the MRI scan well. The dominant ventricle had a left ventricular morphology in all cases. On 4D Flow MRI assessment, two patients (Patients 2 and 6) showed improved cardiac filling with improved preload during oxygen administration, increased mitral inflow, increased maximum E-wave kinetic energy, and decreased systolic peak kinetic energy. Patient 1 showed improved preload only. Patient 5 showed no change, and patient 3 had equivocal results. Patient 4, however, showed a decrease in preload and cardiac filling/function with oxygen.

Using oxygen as a pulmonary vasodilator to assess increased pulmonary venous return as a marker for positive acute vasodilator response would provide pre-treatment assessment in a more physiological state – the awake patient. This proof-of-concept study showed that it is well tolerated and has shown changes in some stable patients with a Fontan circulation.

Many parents of infants with CHD find it difficult to recognise symptoms of deterioration in their children. Therefore, a personalised decision support application for parents has been developed. This application aims to increase parents’ awareness of their infant’s normal condition, help them assess signs of deterioration, decide who and when to contact health services, and what to report. The aim of this paper is to describe the concept and report results from a usability study.

An interprofessional group developed a mobile application called the Heart OBServation app in close collaboration with parents using an iterative process. We performed a usability study consisting of semi-structured interviews of 10 families at discharge and after one month and arranged two focus group interviews with nurses caring for these families. A thematic framework analysis of the interviews explored the usability of features in the application. Usability was assessed twice using the System Usability Scale, and a user log was registered throughout the study.

The overall system usability score, 82.3 after discharge and 81.7 after one month, indicates good system usability. The features of Heart OBServation were perceived as useful to provide tailored information, increase awareness of the child’s normal condition, and to guide parents in what to look for. To empower parents, an interactive discharge checklist was added.

The Heart OBServation demonstrated good usability and was well received by parents and nurses. Feasibility and benefits of this application in clinical practice will be investigated in further studies

Current guidelines discourage shunt closure in patients with pulmonary vascular resistance index >8 Wood units x m2. The study examined the long-term outcome of patients over 15 years old, with pulmonary vascular resistance index >8 Wood units x m2 and patent ductus arteriosus.

This was a multi-institutional, retrospective study involving all consecutive patients (>15 years old) with patent ductus arteriosus and severe pulmonary hypertension. Patients who had patent ductus arteriosus closure were divided into the Good (no death or hospital admissions due to worsening pulmonary hypertension) and the Poor Outcome groups and these groups were compared.

Thirty-seven patients [male: 9 (24.3%); mean age: 30.49 ± 9.56 years; median follow-up: 3 (IQR: 1.5,10) years] were included from four centers. Twenty-two patients who underwent patent ductus arteriosus closure, 15 (71.4%) had good outcomes while 7 (28.6%) had poor outcomes. Pulmonary vascular resistance index and pulmonary to systemic resistance ratio (Rp:Rs) were lower in the Good Outcome Group (14.35 ± 1.66 Wood units x m2 vs. 20.07 ± 2.44; p = 0.033 and 0.44 ± 0.16 vs. 1.08 ± 1.21; p = 0.042). Haemoglobin concentrations (<14.3 g/dL) were associated with good long-term outcomes in the Closed Group.

Patients with patent ductus arteriosus with severe pulmonary hypertension have a dismal outcome with or without closure. High haemoglobin levels at the time of occlusion predict a worse outcome for patients with patent ductus arteriosus and pulmonary hypertension.

The purpose of this study was to report outcomes of double outlet right ventricle biventricular repair at a single institution in developing countries and to investigate post-operatively determined risk factors for mortality and late intervention.

Patients with double outlet right ventricle who underwent biventricular repair in our centre from January 2009 to December 2021 were included.

A total of 96 patients (male/female = 61/35) with biventricular repair were included. According to its specific anatomical type, the appropriate individual surgical plan was selected. Multivariate analysis indicated that prolonged cardiopulmonary bypass time(OR = 1.044; p = 0.012) and pre-operative moderate or above pulmonary hypertension(OR = 24.558; p = 0.023) were independent risk factors for early mortality. Univariate analysis showed that different anatomical types and different surgical methods had similar late intervention and late mortality. Concomitant coarctation of the aorta(OR = 40; p = 0.020) and concomitant ventricular septal defect enlargement(OR = 26.667; p = 0.005) were independent risk factors for late intervention by multivariate analysis.

Selection of appropriate surgical techniques based on different anatomical types often results in similar late outcomes. For patients with concomitant ventricular septal defect enlargement during the operation, it is necessary to fully expand and avoid damage to the conduction bundle. We should timely intervention in patients with coarctation of the aorta and pay attention to the occurrence of left ventricular outflow tract obstruction during follow-up.

Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia.

A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro–Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described.

Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3 years (interquartile range 4.3–6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12 mmHg (interquartile range 10–15), the Nakata index 272 mm2/m2 (interquartile range 204–327), and the McGoon index (interquartile range 1.86–2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19 days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%.

The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.

To better understand parents’ accounts of their prenatal and postnatal experience after prenatal diagnosis of CHD – particularly emotional processing and coping mechanisms – to identify strategies to improve support.

This single-centre, longitudinal qualitative study included pregnant mothers and their support persons seen in Fetal Cardiology Clinic at Vanderbilt Children’s Hospital from May through August 2019 for probable complex CHD. Twenty-seven individuals from 17 families participated in 62 phone interviews during pregnancy and postpartum: 27 conducted after the initial prenatal cardiology consultation, 15 after a follow-up prenatal visit, and 20 after birth. Applied thematic analysis approach was used to code and analyse transcribed interviews. Coding and codebook revisions occurred iteratively; intercoder reliability was >80%.

Patients included mothers (16 [59%]), fathers (8 [30%]), and other support persons (3 [11%]). Initial fetal diagnoses included a range of moderate to severe CHD. Prenatally, parents sought to maintain hope while understanding the diagnosis; planning for the future rather than focusing on day-to-day was more common if prognoses were better. Postnatally, with confirmation of prenatal diagnoses, parents’ sense of control expanded, and they desired more active engagement in clinical decision making.

To enhance effective communication and support, understanding how parents conceptualise hope in relation to diagnosis and how that may evolve over time is critical. Expectant parents whose child has a significant risk of mortality may demonstrate hope by focusing on positivity. As prognostic uncertainty diminishes postpartum, the parental role on the team may shift, requiring clinicians to provide different support.

In this study, it was aimed to examine the serum endocan levels in patients with rheumatic aortic regurgitation and to investigate whether it has a value in differentiating it from aortic regurgitation due to bicuspid aortic valve.

Blood samples were collected from patients with rheumatic aortic regurgitation (Group 1), incidentally diagnosed patients with borderline or definite rheumatic aortic regurgitation (Group 2), children with bicuspid aortic valve accompanied by aortic regurgitation (Group 3) and healthy children (Group 4) of similar age.

There were 12 children in Group 1, 13 in Group 2, 25 in Group 3, and 25 in Group 4. Groups were similar in terms of age (p = 0.291). There was no statistically significant difference between median serum endocan levels of Group 1 and Group 2 (p = 0.624), and Group 3 and Group 4 (p = 0.443). Despite that, the median serum endocan levels of Group 1 and Group 2 were significantly higher than that of both Group 3 and Group 4 (p = 0.000 for all).

Our results indicate that serum endocan level can be used to differentiate rheumatic aortic regurgitation from non-rheumatic aortic regurgitation. It is thought that the prognostic role of this marker should be confirmed in long-term, prospective studies with larger samples.