While most children with multisystem inflammatory syndrome in children have rapid recovery of cardiac dysfunction, little is known about the long-term outcomes regarding exercise capacity. We aimed to compare the exercise capacity among patients with multisystem inflammatory syndrome in children versus viral/idiopathic myocarditis at 3–6 months after initial diagnosis.

We performed a retrospective cohort study among patients with multisystem inflammatory syndrome in children in June 2020 to May 2021 and patients with viral/idiopathic myocarditis in August 2014 to January 2020. Data from cardiopulmonary exercise test as well as echocardiographic and laboratory data were obtained. Inclusion criteria included diagnosis of multisystem inflammatory syndrome in children or viral/idiopathic myocarditis, exercise test performed within 3–6 months of hospital discharge, and maximal effort on cardiopulmonary exercise test as determined by respiratory exchange ratio >1.10.

Thirty-one patients with multisystem inflammatory syndrome in children and 25 with viral/idiopathic myocarditis were included. The mean percent predicted peak VO2 was 90.84% for multisystem inflammatory syndrome in children patients and 91.08% for those with viral/idiopathic myocarditis (p-value 0.955). There were no statistically significant differences between the groups with regard to percent predicted maximal heart rate, metabolic equivalents, percent predicted peak VO2, percent predicted anerobic threshold, or percent predicted O2 pulse. There was a statistically significant correlation between lowest ejection fraction during hospitalisation and peak VO2 among viral/idiopathic myocarditis patients (r: 0.62, p-value 0.01) but not multisystem inflammatory syndrome in children patients (r: 0.1, p-value 0.6).

Patients with multisystem inflammatory syndrome in children and viral myocarditis appear to, on average, have normal exercise capacity around 3–6 months following hospital discharge. For patients with viral/idiopathic myocarditis, those with worse ejection fraction during hospitalisation had lower peak VO2 on cardiopulmonary exercise test.

Permanent pacing in children with isolated congenital complete atrioventricular block may cause left ventricular dysfunction. To prevent it, alternative pacing sites have been proposed: left ventricular epicardial or selective right ventricular endocardial pacing.

To compare the functional outcome (left ventricular systolic function and synchrony) in paediatric patients with congenital complete atrioventricular block and left ventricular apical epicardial or right ventricular transvenous mid-septal pacing.

Retrospective study. Epicardial leads were implanted by standard surgical technique, transvenous leads by 3D electroanatomic mapping systems. 3D mapping acquired 3D right ventricular local pacing map and defined the narrowest paced QRS site. 3D mapping guided screw-in bipolar leads on that ventricular site. Electrocardiogram (ECG) (QRS duration) and echocardiographic data (synchrony: interventricular mechanical delay, septal to posterior wall motion delay, systolic dyssynchrony index; contractility: global longitudinal strain, ejection fraction) were recorded. Data are reported as median [interquartile ranges]. p < 0.05 was significant.

There were 19 transvenous systems (age 8.8 [6–14] years; right ventricular mid-septum) and 17 epicardial systems (0.04 [0.001–0.6] years; left ventricular apex). Post-implantation QRS significantly widened either in endocardial or in epicardial patients. Most patients reached 4-year follow-up. One-year and 4-year ejection fraction and global longitudinal strain were mostly within normal limits and did not show significant differences between the two groups and between the same endocardial/epicardial group. Synchrony parameters were within normal limits in the two groups.

Left ventricular apical epicardial pacing and 3D mapping-guided right ventricular mid-septal pacing preserved left ventricular contractility and synchrony in children and adolescents with congenital complete atrioventricular block at short-/mid-term follow-up, without relevant significant differences between the two groups.

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.

While the incidence of pregnancy has increased among individuals with adult CHD, little has been described about considerations and experiences of patients with adult CHD regarding pregnancy.

We aimed to explore patients’ motivations, concerns, and decision-making processes regarding pregnancy.

In April 2019–January 2020, we conducted in-depth telephone interviews with patients (n = 25) with simple, moderate, or complex adult CHD, who received prenatal care at the University of Washington during 2010–2019 and experienced a live birth. Transcripts were analysed using thematic analysis.

Participants described motivations for pregnancy as both internal desires (motherhood, marriage fulfillment, biological connection, fetal personhood, self-efficacy) and external drivers (family or community), as well as concerns for the health and survival of themselves and the fetus. Factors that enabled their decision to maintain a pregnancy included having a desire that outweighed their perceived risk, using available data to guide their decision, planning for contingencies and knowing their beliefs about termination, plus having a trusted healthcare team, social support, and resources. Factors that led to insurmountable risk in subsequent pregnancies included desire having been fulfilled by the first pregnancy, compounding risk with age and additional pregnancies, new responsibility to an existing child, and reduced healthcare team and social support.

Understanding individuals’ motivations and concerns, and how they weigh their decisions to become or remain pregnant, can help clinicians better support patients with adult CHD considering pregnancy. Clinician education on patient experiences is warranted.

After patent foramen ovale interventional closure, puncture of the interatrial septum through the occluder is difficult but sometimes needed for further interventional treatment. This paper presents findings from an in vivo experimental study of a reserved atrial septal puncture area patent foramen ovale occluder.

A patent foramen ovale model was established in canines using trans-septal puncture of the fossa ovale and high-pressure balloon dilation. Then, patent foramen ovale closure was performed with a reserved atrial septal puncture area and all canines were raised for 3 months. Then, the occluder was crossed and left atrial angiography was performed on the septal area with the occluder. Finally, DSA angiography, echocardiography, and histology were used to evaluate the performance and feasibility of the reserved atrial septal puncture area.

A patent foramen ovale model was successfully established in 10 canines using the atrial septal puncture method. The average diameter of the patent foramen ovale was 3.77 ±0.19 mm, and the patent foramen ovale was successfully closed in all canines using a reserved atrial septal puncture area. As assessed using transoesophageal echocardiography, the new occluder exhibited an ideal position and was occluded entirely without a residual shunt intraoperatively and postoperatively. A 100% success rate of atrial septum puncture was achieved across the new occluder. The occluders were completely endothelialised 3 months post-implantation.

The reserved atrial septal puncture area was effective in patent foramen ovale closure and exhibited positive sealing performance and biological compatibility. Trans-septal puncture was feasible and effective after reserved atrial septal puncture area patent foramen ovale closure.

Growth failure in infants born with CHD is a persistent problem, even in those provided with adequate nutrition.

To summarise the published data describing the change in urinary metabolites during metabolic maturation in infants with CHD and identify pathways amenable to therapeutic intervention

Scoping review.

Studies using qualitative or quantitative methods to describe urinary metabolites pre- and post-cardiac surgery and the relationship with growth in infants with CHD.

NICE Healthcare Databases website was used as a tool for multiple searches.

347 records were identified, of which 37 were duplicates. Following the removal of duplicate records, 310 record abstracts and titles were screened for inclusion. The full texts of eight articles were reviewed for eligibility, of which only two related to infants with CHD. The studies included in the scoping review described urinary metabolites in 42 infants. A content analysis identified two overarching themes of metabolic variation predictive of neurodevelopmental abnormalities associated with anaerobic metabolism and metabolic signature associated with the impact on gut microbiota, inflammation, energy, and lipid digestion.

The results of this scoping review suggest that there are considerable gaps in our knowledge relating to metabolic maturation of infants with CHD, especially with respect to growth. Surgery is a key early life feature for CHD infants and has an impact on the developing biochemical phenotype with implications for metabolic pathways involved in immunomodulation, energy, gut microbial, and lipid metabolism. These early life fingerprints may predict those individuals at risk for neurodevelopmental abnormalities.

Various electrocardiogram (ECG)-based devices are available for home monitoring, but the reliability in adults with CHD is unknown. Therefore, we determined the accuracy of different ECG-based devices compared to the standard 12-lead ECG in adult CHD.

This is a single-centre, prospective, cross-sectional study in 176 consecutive adults with CHD (54% male, age 40 ± 16.6 years, 24% severe CHD, 84% previous surgery, 3% atrial fibrillation (AF), 24% right bundle branch block). Diagnostic accuracy of the Withings Scanwatch (lead I), Eko DUO (precordial lead), and Kardia 6L (six leads) was determined in comparison to the standard 12-lead ECG on several tasks: 1) AF classification (percentage correct), 2) QRS-morphology classification (percentage correct), and 3) ECG intervals calculation (QTc time ≤ 40 ms difference). Both tested AF algorithms had high accuracy (Withings: 100%, Kardia 6L: 97%) in ECGs that were classified. However, the Withings algorithm classified fewer ECGs as inconclusive (5%) compared to 31% of Kardia (p < 0.001). Physician evaluation of Kardia correctly classified QRS morphology more frequently (90% accuracy) compared to Eko DUO (84% accuracy) (p = 0.03). QTc was underestimated on all ECG-based devices (p < 0.01). QTc duration accuracy was acceptable in only 51% of Withings versus 70% Eko and 74% Kardia (p < 0.001 for both comparisons).

Although all devices demonstrated high accuracy in AF detection, the Withings automatic algorithm had fewest uninterpretable results. Kardia 6L was most accurate in overall evaluation such as QRS morphology and QTc duration. These findings can inform both patients and caregivers for optimal choice of home monitoring.

Complex patients requiring operations on the left ventricular outflow tract, aortic valve, or thoracic aorta after previous repair of aortopathy constitute a challenging group, with limited information guiding decision-making. We aimed to use our institutional experience to highlight management challenges and describe surgical pearls to address them.

Forty-one complex patients with surgery on the left ventricular outflow tract, aortic valve, or aorta at Cleveland Clinic Children’s between 2016 and 2021 following previous repair of aortic pathology were retrospectively reviewed. Patients with known connective tissue disease or single ventricle circulation were excluded.

Median age at index procedure was 23 years (range 0.25–48) with median of 2 prior sternotomies. Previous aortic operations included subvalvular (n = 9), valvular (n = 6), supravalvular (n = 13), and multi-level surgeries (n = 13). Four deaths occurred in median follow-up of 2.5 years. Mean left ventricular outflow tract gradients improved significantly for patients with obstruction (34.9 ± 17.5 mmHg versus 12.6 ± 6.0 mmHg; p < 0.001). Technical pearls include the following: 1) liberal use of anterior aortoventriculoplasty with valve replacement; 2) primarily anterior aortoventriculoplasty following the subpulmonary conus in contrast to more vertical incision for post-arterial switch operation patients; 3) pre-operative imaging of mediastinum and peripheral vasculature for cannulation and sternal re-entry; and 4) proactive use of multi-site peripheral cannulation.

Operation to address the left ventricular outflow tract, aortic valve, or aorta following prior congenital aortic repair can be accomplished with excellent outcomes despite high complexity. These procedures commonly include multiple components, including concomitant valve interventions. Cannulation strategies and anterior aortoventriculoplasty in specific patients require modifications.

To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group.

Forty-eight patients, 8–18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area. Health-related quality of life was assessed by the KIDSCREEN-52 self-report and parent proxy report, and life satisfaction was evaluated with the Satisfaction With Life Scale. Physical activity was measured with an accelerometer for 7 days. Sports participation was self-reported.

No differences in the health-related quality of life domains were found between patients and controls in the self-reports. In the proxy reports, parents of the adolescents rated their child’s autonomy lower than did the parents of the healthy controls. A negative relationship was found between moderate to vigorous physical activity, sports participation, life satisfaction and the psychological well-being domain in adolescent patients. In children there was a positive relationship between moderate physical activity and the physical and psychological well-being domains.

Overall, children and adolescents treated for valvular aortic stenosis reported similar life satisfaction and health-related quality of life as their healthy peers. The negative relationships between intense physical activity and sports participation with health-related quality of life and life satisfaction in adolescent patients might be explained by both physical and psychological factors in these teenagers with complex, lifelong heart disease.

Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life.

In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life.

Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 – 176 days, 3rd = 178 – 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year.

Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

Prolonged pleural effusions are common post Fontan operation and are associated with morbidity. Fontan pleural effusions have elevated proinflammatory cytokines. Little is known about the chest tube drainage after a superior cavopulmonary connection. We examined the chest tube drainage and the inflammatory profiles in post-operative superior cavopulmonary connection patients.

This prospective cohort study enrolled 25 patients undergoing superior cavopulmonary connection and 10 age-similar controls. Data are also compared to 25 previously published Fontan patients and their 15 age-similar controls. Chest tube samples were analysed with a 17-cytokine BioPlex Assay. Descriptive statistics and univariate comparisons were made between groups.

Duration of chest tube drainage was significantly shorter in superior cavopulmonary connection patients (median 4 days, [interquartile range 3–5 days]) versus Fontan patients (10 days, [7–11 days], p < 0.0001). Cytokine concentrations were higher on post-operative day 1 in superior cavopulmonary connection patients versus Fontan patients (all p ≤ 0.01), however levels were comparable to age-similar controls. While proinflammatory IL 8, MIP-1β, and TNF-α concentrations increased in chest tube drainage of Fontan patients from post-operative day 1 to last chest tube day (all p < 0.0001), there was no change in these biomarkers in superior cavopulmonary connection patients, their controls, or Fontan controls.

Our study demonstrates that after superior cavopulmonary connection, proinflammatory cytokines in the chest tube drainage remain similar to biventricular controls of both age groups, unlike the significant rise over time observed in Fontan patients. Inflammation within the chest tube drainage is likely not innate to single ventricle patients.

Management of total anomalous pulmonary venous connections has been extensively studied to further improve outcomes. Our institution previously reported factors associated with mortality, recurrent obstruction, and reintervention. The study purpose was to revisit the cohort of patients and evaluate factors associated with reintervention, and mortality in early and late follow-up.

A retrospective review at our institution identified 81 patients undergoing total anomalous pulmonary venous connection repair from January 2002 to January 2018. Demographic and operative variables were evaluated. Anastomotic reintervention (interventional or surgical) and/or mortality were primary endpoints.

Eighty-one patients met the study criteria. Follow-up ranged from 0 to 6,291 days (17.2 years), a mean of 1263 days (3.5 years). Surgical mortality was 16.1% and reintervention rates were 19.8%. In re-interventions performed, 80% occurred within 1.2 years, while 94% of mortalities were within 4.1 months. Increasing cardiopulmonary bypass times (p = 0.0001) and the presence of obstruction at the time of surgery (p = 0.025) were predictors of mortality, while intracardiac total anomalous pulmonary venous connection type (p = 0.033) was protective. Risk of reintervention was higher with increasing cardiopulmonary bypass times (p = 0.015), single ventricle anatomy (p = 0.02), and a post-repair gradient >2 mmHg on transesophageal echocardiogram (p = 0.009).

Evaluation of a larger cohort with longer follow-up demonstrated the relationship of anatomic complexity and symptoms at presentation to increased mortality risk after total anomalous pulmonary venous connection repair. The presence of a single ventricle or a post-operative confluence gradient >2 mmHg were risk factors for reintervention. These findings support those found in our initial study.

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