Adults with CHD have reduced work participation rates compared to adults without CHD. We aimed to quantify employment rate among adult CHD patients in a population-based registry and to describe factors and barriers associated with work participation.

We retrospectively identified adults with employment information in the North Carolina Congenital Heart Defects Surveillance Network. Employment was defined as any paid work in a given year. Logistic regression was used to examine patients’ employment status during each year.

The registry included 1,208 adult CHD patients with a health care encounter between 2009 and 2013, of whom 1,078 had ≥1 year of data with known employment status. Overall, 401 patients (37%) were employed in their most recent registry year. On multivariable analysis, the odds of employment decreased with older age and were lower for Black as compared to White patients (odds ratio = 0.78; 95% confidence interval: 0.62, 0.98; p = 0.030), and single as compared to married patients (odds ratio = 0.50; 95% confidence interval: 0.39, 0.63; p < 0.001).

In a registry where employment status was routinely captured, only 37% of adult CHD patients aged 18–64 years were employed, with older patients, Black patients, and single patients being less likely to be employed. Further work is needed to consider how enhancing cardiology follow-up for adults with CHD can integrate support for employment.

The Ross procedure involves using the native pulmonary valve for aortic valve replacement then replacing the pulmonary valve with an allograft or xenograft. We aimed to compare our age-matched experience with the bovine jugular vein conduit and the pulmonary homograft for pulmonary valve replacement during the Ross procedure in children.

Between 1998 and 2016, 15 patients <18 years of age underwent a Ross procedure using the bovine jugular vein conduit (Ross-Bovine Jugular Vein Conduit) at our institution. These patients were age-matched with 15 patients who had the Ross operation with a standard pulmonary homograft for right ventricular outflow tract reconstruction (Ross-Pulmonary Homograft). Paper and electronic medical records were retrospectively reviewed.

The median age of the Ross-Bovine Jugular Vein Conduit and Ross-Pulmonary Homograft patients were 4.8 years (interquartile range 1.1–6.6) and 3.3 years (interquartile 1.2–7.6), respectively (p = 0.6). The median follow-up time for the Ross-Bovine Jugular Vein Conduit and Ross-Pulmonary Homograft groups were 1.7 years (interquartile range 0.5–4.9) and 6.8 years (interquartile range 1.9–13.4), respectively (p = 0.03). Overall, 5-year survival, freedom from redo aortic valve replacement, and freedom from pulmonary valve replacement were similar between groups.

The bovine jugular vein conduit and pulmonary homograft have favourable mid-term durability when used for right ventricular outflow tract reconstruction for the Ross operation. The bovine jugular vein conduit may be a suitable replacement for appropriately sized patients undergoing a Ross aortic valve replacement, though longer follow-up is needed.

Adults with CHD are known to greatly benefit from a prompt access to continuous expert care. On the other hand, coronavirus disease 2019 pandemic has determined a dramatic worldwide reconfiguration of the healthcare systems, with rapid redeployment of resources towards this emergency. Italy was the first Western country affected by a large-scale spread of coronavirus disease 2019. The aim of our study is to analyse the impact of the coronavirus disease 2019 outbreak on in-hospital care of patients with CHD in an Italian tertiary centre.

We retrospectively reviewed data on CHD hospital admissions in our centre since 1 March, 2020, when the adoption of a strict infection containment policy throughout the country resulted in limited access of patients to routine hospital care and resources reallocation to the care of infected patients. Comparison with data from the previous year was performed in order to identify any relevant differences attributable to the outbreak. Despite cancellation of all elective procedures, the overall number of urgent hospital admission remained stable throughout the period of study. Patients admitted during the pandemic had greater disease complexity (p = 0.001) with longer length of in-hospital stay (p = 0.01). No adverse events or positive swabs were reported among CHD patients who were admitted to hospital or medical personnel caring for these patients.

Data from our early experience suggest that coronavirus disease 2019 pandemic did not impact significantly on the provision of urgent care to adult patients with CHD.

Pre-mature birth impacts left ventricular development, predisposing this population to long-term cardiovascular risk. The aims of this study were to investigate maturational changes in rotational properties from the neonatal period through 1 year of age and to discern the impact of cardiopulmonary complications of pre-maturity on these measures.

Pre-term infants (<29 weeks at birth, n = 117) were prospectively enrolled and followed to 1-year corrected age. Left ventricular basal and apical rotation, twist, and torsion were measured by two-dimensional speckle-tracking echocardiography and analysed at 32 and 36 weeks post-menstrual age and 1-year corrected age. A mixed random effects model with repeated measures analysis was used to compare rotational mechanics over time. Torsion was compared in infants with and without complications of cardiopulmonary diseases of pre-maturity, specifically bronchopulmonary dysplasia, pulmonary hypertension, and patent ductus arteriosus.

Torsion decreased from 32 weeks post-menstrual age to 1-year corrected age in all pre-term infants (p < 0.001). The decline from 32 to 36 weeks post-menstrual age was more pronounced in infants with cardiopulmonary complications, but was similar to healthy pre-term infants from 36 weeks post-menstrual age to 1-year corrected age. The decline was due to directional and magnitude changes in apical rotation over time (p < 0.05).

This study tracks maturational patterns of rotational mechanics in pre-term infants and reveals torsion declines from the neonatal period through 1 year. Cardiopulmonary diseases of pre-maturity may negatively impact rotational mechanics during the neonatal period, but the myocardium recovers by 1-year corrected age.

This is a cross-sectional study aiming to understand the early characteristics and background of bone health impairment in clinically well children with Fontan circulation.

We enrolled 10 clinically well children with Fontan palliation (operated >5 years before study entrance, Tanner stage ≤3, age 12.1 ± 1.77 years, 7 males) and 11 healthy controls (age 12.0 ± 1.45 years, 9 males) at two children’s hospitals. All patients underwent peripheral quantitative CT. For the Fontan group, we obtained clinical characteristics, NYHA class, cardiac index by MRI, dual x-ray absorptiometry, and biochemical studies. Linear regression was used to compare radius and tibia peripheral quantitative CT measures between Fontan patients and controls.

All Fontan patients were clinically well (NYHA class 1 or 2, cardiac index 4.85 ± 1.51 L/min/m2) and without significant comorbidities. Adjusted trabecular bone mineral density, cortical thickness, and bone strength index at the radius were significantly decreased in Fontan patients compared to controls with mean differences −30.13 mg/cm3 (p = 0.041), −0.31 mm (p = 0.043), and −6.65 mg2/mm4 (p = 0.036), respectively. No differences were found for tibial measures. In Fontan patients, the mean height-adjusted lumbar bone mineral density and total body less head z scores were −0.46 ± 1.1 and −0.63 ± 1.1, respectively, which are below the average, but within normal range for age and sex.

In a clinically well Fontan cohort, we found significant bone deficits by peripheral quantitative CT in the radius but not the tibia, suggesting non-weight-bearing bones may be more vulnerable to the unique haemodynamics of the Fontan circulation.

The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season.

We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1–42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October).

Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06).

Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

The Fontan procedure is the final stage of surgical palliation for the children with functionally single ventricle anatomy. The post-operative medical management of this patient population can be variable and hospital length of stay prolonged. The purpose of this quality improvement project was to determine if the implementation of an evidence-based clinical pathway for post-operative management of the Fontan patient at a large Midwestern academic paediatric medical centre would standardise care and decrease length of stay.

The clinical pathway was developed using key components from three published pathways for the Fontan procedure from other paediatric institutions across the United States. Components of the clinical pathway included (1) supplemental oxygen until pleural drainage tubes are removed, (2) fluid restriction to 80% daily maintenance and a prescribed low-fat diet, (3) aggressive and standardised diuretic therapy while inpatient and (4) central venous access. The pathway was trialed using Plan-Do-Study-Act cycles in 2016, implemented in 2017 and sustained in 2018–2019. A retrospective electronic medical record review was performed to compare key outcomes from pre-pathway (2014–2015, 37 patients) with post-pathway implementation (2017–2018, 30 patients).

Adherence to the pathway was nearly 100% with a statistically significant decrease in length of stay from 12 to 9 days (p = 0.007) and no increase in readmissions.

Standardising care can improve clinical and financial outcomes for the Fontan patient population without negatively impacting quality of care, thus providing a positive benefit to the healthcare institution, industry and patient.

Indexing left ventricular mass to body surface area or height2.7 leads to inaccuracies in diagnosing left ventricular hypertrophy in obese children. Lean body mass predictive equations provide the opportunity to determine the utility of lean body mass in indexing left ventricular mass. Our objectives were to compare the diagnostic accuracy of predicted lean body mass, body surface area, and height in detecting abnormal left ventricle mass in obese children.

Obese non-hypertensive patients aged 4–21 years were recruited prospectively. Dual-energy X-ray absorptiometry was used to measure lean body mass. Height, weight, sex, race, and body mass index z-score were used to calculate predicted lean body mass.

We enrolled 328 patients. Average age was 12.6 ± 3.8 years. Measured lean body mass had the strongest relationship with left ventricular mass (R2 = 0.84, p < 0.01) compared to predicted lean body mass (R2 = 0.82, p < 0.01), body surface area (R2 = 0.80, p < 0.01), and height2.7 (R2 = 0.65, p < 0.01). Of the clinically derived variables, predicted lean body mass was the only measure to have an independent association with left ventricular mass (β = 0.90, p < 0.01). Predicted lean body mass was the most accurate scaling variable in detecting left ventricular hypertrophy (positive predictive value = 88%, negative predictive value = 99%).

Lean body mass is the strongest predictor of left ventricular mass in obese children. Predicted lean body mass is the most accurate anthropometric scaling variable for left ventricular mass in left ventricular hypertrophy detection. Predicted lean body mass should be considered for clinical use as the body size correcting variable for left ventricular mass in obese children.

Murmurs are abnormal audible heart sounds produced by turbulent blood flow. Therefore, murmurs in a child may be a source of anxiety for family members. Families often use online materials to explore possible reasons for these murmurs, given the accessibility of information on the Internet. In this study, we evaluated the quality, understandability, readability, and popularity of online materials about heart murmur.

An Internet search was performed for “heart murmur” using the Google search engine. The global quality score (on a scale of 1 to 5, corresponding to poor to excellent quality) and Health on the Net code were used to measure the quality of information presented. The understandability of the web pages identified was measured using the Patient Education Materials Assessment Tool (score range from 0 to 100%, scores below 70% reflect poor performance). The readability of each web pages was assessed using four validated indices: the Flesch Reading Ease Score, the Flesch–Kincaid Grade Level, the Gunning Frequency of Gobbledygook, and the Simple Measure of Gobbledygook. The ALEXA traffic tool was used to reference domains’ popularity and visibility.

We identified 230 English-language patient educational materials that discussed heart murmur. After exclusion, a total of 86 web pages were evaluated for this study. The average global quality score was 4.34 (SD = 0.71; range from 3 to 5) indicating that the quality of information of most websites was good. Only 14 (16.3%) websites had Health on the Net certification. The mean understandability score for all Internet-based patient educational materials was 74.6% (SD = 12.8%; range from 31.2 to 93.7%). A score suggesting these Internet-based patient educational materials were “easy to understand”. The mean readability levels of all patient educational materials were higher than the recommended sixth-grade reading level, according to all indices applied. This means that the level of readability is difficult. The average grade level for all web pages was 10.4 ± 1.65 (range from 7.53 to 14.13). The Flesch–Kincaid Grade level was 10 ± 1.81, the Gunning Frequency of Gobbledygook level was 12.1 ± 1.85, and the Simple Measure of Gobbledygook level was 9.1 ± 1.38. The average Flesch Reading Ease Score was 55 ± 9.1 (range from 32.4 to 72.9).

We demonstrated that web pages describing heart murmurs were understandable and high quality. However, the readability level of the websites was above the recommended sixth-grade reading level. Readability of written materials from online sources need to be improved. However, care must be taken to ensure that the information of web pages is of a high quality and understandable.

Fascicular tachycardia is a common form of sustained idiopathic left ventricular tachycardia. This study aimed to achieve successful results with catheter ablation procedures performed through three-dimensional electroanatomic mapping using near-zero fluoroscopy in fascicular tachycardia patients.

In this study, we included 33 consecutive children with fascicular tachycardia, for whom we performed a transcatheter radiofrequency ablation procedure using the EnSite® system. Activation mapping was performed in all patients during tachycardia, and the earliest presystolic purkinje potentials were the target site for radiofrequency lesions.

Twenty-five patients were male, and eight were female. The mean weight of the patients was 39.6 ± 10.4 kg, and the mean age was 13.6 ± 2.5 years. The mean procedure time was 121.3 ± 44.3 minutes. The mean follow-up period was 18.4 ± 6.5 months. No fluoroscopy was needed in 30 patients. The mean fluoroscopy time in the remaining patients was 166.6 ± 80 seconds. All of the patients had left posterior fascicular tachycardia except for one who had left anterior fascicular tachycardia. The acute success rate was perfect (100%). No patients developed left bundle branch block or complete atrioventricular block. Recurrence developed in one patient.

We suggest that radiofrequency ablations via an electroanatomic mapping system are quite safe and effective, with high success rates in paediatric patients with fascicular tachycardia. This method has the advantage of avoiding ionising radiation exposure for both the patient and operator, thus reducing the lifetime risk of malignancy in the paediatric population.

Children with complex CHD are at risk for psychopathology such as severe attention-deficit/hyperactivity disorder symptoms after congenital heart surgery.

The aim of this study was to investigate if children with Ventricular Septal Defect, Transposition of Great Arteries, or Tetralogy of Fallot have an increased occurrence of attention-deficit/hyperactivity disorder symptoms compared with the background population and to investigate differences between the three CHDs in terms of occurrence and appearance of attention-deficit/hyperactivity disorder symptoms.

A national register-based survey was conducted, including children aged 10–16 years with surgically corrected CHDs without genetic abnormalities and syndromes. The Attention-Deficit/Hyperactivity Disorder-Rating Scale questionnaires were filled in by parents and school teachers.

In total, 159 out of 283 questionnaires were completed among children with CHDs and compared with age- and sex-matched controls. Children with CHDs had significantly increased inattention scores (p = 0.009) and total attention-deficit/hyperactivity disorder scores (p = 0.008) compared with controls. Post hoc analyses revealed that children with Tetralogy of Fallot had significantly higher inattention scores compared with children both with Ventricular Septal Defect (p = 0.043) and controls (p = 0.004).

Attention-deficit/hyperactivity disorder symptoms and inattention symptoms were significantly more frequent among children aged 10–16 years with CHDs, in particular in children with corrected Tetralogy of Fallot.

We evaluated the safety and feasibility of high-intensity interval training via a novel telemedicine ergometer (MedBIKE™) in children with Fontan physiology.

The MedBIKE™ is a custom telemedicine ergometer, incorporating a video game platform and live feed of patient video/audio, electrocardiography, pulse oximetry, and power output, for remote medical supervision and modulation of work. There were three study phases: (I) exercise workload comparison between the MedBIKE™ and a standard cardiopulmonary exercise ergometer in 10 healthy adults. (II) In-hospital safety, feasibility, and user experience (via questionnaire) assessment of a MedBIKE™ high-intensity interval training protocol in children with Fontan physiology. (III) Eight-week home-based high-intensity interval trial programme in two participants with Fontan physiology.

There was good agreement in oxygen consumption during graded exercise at matched work rates between the cardiopulmonary exercise ergometer and MedBIKE™ (1.1 ± 0.5 L/minute versus 1.1 ± 0.5 L/minute, p = 0.44). Ten youth with Fontan physiology (11.5 ± 1.8 years old) completed a MedBIKE™ high-intensity interval training session with no adverse events. The participants found the MedBIKE™ to be enjoyable and easy to navigate. In two participants, the 8-week home-based protocol was tolerated well with completion of 23/24 (96%) and 24/24 (100%) of sessions, respectively, and no adverse events across the 47 sessions in total.

The MedBIKE™ resulted in similar physiological responses as compared to a cardiopulmonary exercise test ergometer and the high-intensity interval training protocol was safe, feasible, and enjoyable in youth with Fontan physiology. A randomised-controlled trial of a home-based high-intensity interval training exercise intervention using the MedBIKE™ will next be undertaken.