We aimed to determine the early and midterm outcomes of ductal stenting in neonates with ductal-dependent pulmonary blood flow.

Between January, 2014 and July, 2018, 102 patients who underwent 115 cardiac catheterisation procedures for ductal stent implantation in our department were retrospectively reviewed. The age of the neonates ranged from 3 to 30 days (median: 11 days) and their weights ranged from 1.8 to 5.8 kg (mean, 2.8 ± 0.53 kg). Fifty-two patients had functional single ventricle and 50 had biventricular physiology. Thirty-one patients’ weights were <2,500 g (30.3%). The patent ductus arteriosus was vertical in 60 patients (58.8%). The mean ductal length was 12.4 ± 4.1 mm (range, 7.8–23 mm), and the mean narrowest ductal diameter was 2.1 ± 0.7 mm (range, 1.2–3.4 mm).

The technical success rate was 85.2%. Procedure-related mortality occurred in three patients (2.9%). After the procedure, the aortic oxygen saturation increased from a mean of 73.1 ± 6.2% to a mean of 90.4 ± 4.3% (p < 0.001), and the ductus diameter increased from a mean of 2.1 ± 0.7 mm to a mean of 4.2 ± 0.9 mm (p < 0.001). Either transcatheter or surgical reinterventions were required in 35 patients (34.3%) during the follow-up period after a median of 101 days (2–356 days). Thirty-three patients (32.3%) were bridged to surgical repair after a median of 288 days (163–650 days). The median duration of palliation with ductal stents was 210 days (range, 2–525 days).

Ductus arteriosus stenting may be a reasonable and effective alternative to surgery for the initial palliation procedure in neonates with ductus-dependent pulmonary flow.

Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies.

An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines.

From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4–9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases; hypertrophic cardiomyopathy, 12.3% (n = 11); restrictive cardiomyopathy, 8.9% (n = 8); non-compacted cardiomyopathy, 7.8% (n = 7); arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6); and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.

Infective endocarditis remains a severe complication associated with a high morbidity and mortality in patients after heart valve replacement. Exploration of the pathogenesis is of high demand and we, therefore, present a competent model that allows studying bacterial adherence and the role of plasma fibrinogen in this process using a new in-house designed low-volume flow chamber. Three cardiac graft tissues used for pulmonary valve replacement have been tested under shear conditions to investigate the impact of surface composition on the adhesion events.

Tissue pieces of cryopreserved homograft (non-decellularised), decellularised homograft and bovine pericardium patch were investigated for fibrinogen binding. Adherence of Staphylococcus aureus to these graft tissues was studied quantitatively under flow conditions in our newly fabricated chamber based on a parallel plates’ modality. The method of counting colony-forming units was reliable and reproducible to assess the propensity of different graft materials for bacterial attachment under shear.

Bacterial perfusions over all plasma-precoated tissues identified cryopreserved homograft with the lowest affinity for S. aureus compared to decellularised homograft presenting a significantly higher bacterial adhesion (p < 0.05), which was linked to a more avid fibrinogen binding (p < 0.01). Bovine pericardial patch, as a reference tissue in this study, was confirmed to be the most susceptible tissue graft for the bacterial adhesion, which was in line with our previous work.

The two studied homograft tissues showed different levels of bacterial attachment, which might be postulated by the involvement of fibrinogen in the adhesion mechanism(s) shown previously for bovine tissues.

Chest pain, as a common cause of hospital admissions in childhood, necessitates detailed investigations due to a wide range of differential diagnoses. In this study, we aimed to determine the distribution of diseases causing chest pain in children and investigate the clinical characteristics of children with chest pain.

This study included 782 patients aged between 3 and 18 years who presented to a paediatric cardiology outpatient clinic with chest pain between April 2017 and March 2018. Aetiological causes and demographic features of the patients were analysed.

Most prevalent causes of chest pain were musculoskeletal system (33%) and psychogenic (28.4%) causes. Chest pain due to cardiac reasons was seen in eight patients (1%). Diseases of musculoskeletal and gastrointestinal systems and psychogenic disorders were significantly more common in male and female patients, respectively (p < 0.001 for all). In winter, patients’ age and the number of patients with ≥12 years were higher than those in other seasons (p < 0.001). Most of the parents (70.8%) and patients (90.2%) thought that chest pain in their children was caused by cardiac causes.

Most of the diagnoses for chest pain in childhood period are benign and include the musculoskeletal system and psychogenic diseases. Although chest pain due to cardiac diseases is rare, a comprehensive analysis of medical history, detailed physical examination and cardiac imaging with echocardiography is needed to reach more accurate diagnoses.

Infants with CHD requiring positive pressure ventilation via tracheostomy are especially vulnerable to malnutrition following cardiac surgery. Current post-operative feeding recommendations may overestimate the caloric needs.

We retrospectively studied infants requiring tracheostomy after cardiac surgery. Anthropometric and nutritional data were collected, including caloric goals, weight-for-age z score, length-for-age z score, and weight-for-length z score. Changes in anthropometrics over time were compared to ascertain the impact of nutritional interventions. Data were shown as mean ± standard deviation.

Nineteen infants with CHD required tracheostomy at 160 ± 109 days (7–364 days), 13 had reparative surgery, and 6 had palliative surgery for single ventricle. The indications for tracheostomy consisted of airway abnormality/obstruction (n = 13), chronic respiratory failure (n = 7), and/or vocal cord paresis (n = 2). Initial maintenance nutritional target was set at 100–130 cal/kg per day. Fourteen patients (73.7%) became obese (maximum weight-for-length z score: 2.59 ± 0.47) under tracheostomy and gastrostomy feeding, whereas five patients did not (weight-for-length z score: 0.2 ± 0.83). Eight obese patients (weight-for-length z score: 2.44 ± 0.85) showed effective reduction of obesity within 6 months (weight-for-length z score: 0.10 ± 0.20; p < 0.05 compared with pre-adjustment) after appropriate feeding adjustment (40–90 cal/kg per day). Overall mortality was high (31.6%) in this population.

Standard nutritional management resulted in overfeeding and obesity in young children with CHD requiring positive pressure ventilation via tracheostomy. Optimal nutritional management in this high-risk population requires close individualised management by multidisciplinary teams.

Catheter ablation is an important therapeutic option for atrial tachycardias in patients with CHD. As a result of extensive scarring and surgical repair, multiple intra-atrial re-entrant tachycardia circuits develop and serve as a substrate for arrhythmias. The best ablation approach for patients with multiple intra-atrial re-entrant tachycardias has not been investigated. Here, we compared substrate-based ablation using extensive scar modification to conventional ablation.

The present study included patients with surgically corrected CHD that underwent intra-atrial re-entrant tachycardia ablation. Extensive scar modification was defined as substrate ablation based on a dense voltage map, aimed to eliminate all potentials in the scar region. The control group had activation mapping-based ablation. A clinical composite endpoint was assessed. Points were given for type, number, and treatment of intra-atrial re-entrant tachycardia recurrence.

In 40 patients, 63 (extensive scar modification 13) procedures were performed. Acute procedural success was achieved in 78%. Procedural duration was similar in both groups. Forty-nine percent had a recurrence within 1 year. During a 5-year follow-up (2.5–7.5 years), 46% required repeat catheter ablation. Compared to baseline, clinical composite endpoint significantly decreased by 46% after 12 months (p = 0.001). Acute procedural success, procedural parameters, recurrence and repeat ablation were similar between extensive scar modification and activation mapping-based ablation.

Catheter ablation using extensive scar modification for intra-atrial re-entrant tachycardias occurring after surgically corrected CHD illustrated similar short- and long-term outcomes and procedural efficiency compared to catheter ablation using activation mapping-based ablation. The choice of ablation approach for multiple intra-atrial re-entrant tachycardia should remain at the discretion of the operator.

Isolated congenital tricuspid regurgitation other than Ebstein’s anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation.

From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients’ clinical data were analysed retrospectively.

Mean age at operation was 48.5 ± 31.0 (range: 9–106) months and mean weight at operation was 16.1 ± 6.9 (range: 8.6–33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 ± 0.05 to 0.54 ± 0.05. At the latest follow-up (50.4 ± 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I.

For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.

Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.

Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.

In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.

Thrombocytopenia is a risk factor for patent ductus arteriosus. Immature and mature platelets exhibit distinct haemostatic properties; however, whether platelet maturity plays a role in postnatal, ductus arteriosus closure is unknown.

In this observational study, counts of immature and mature platelets (=total platelet count − immature platelet count) were assessed on days 1, 3, and 7 of life in very low birth weight infants (<1500 g birth weight). We performed echocardiographic screening for haemodynamically significant patent ductus arteriosus on day 7.

Counts of mature platelets did not differ on day 1 in infants with (n = 24) and without (n = 45) haemodynamically significant patent ductus arteriosus, while infants with significant patent ductus arteriosus exhibited lower counts of mature platelet on postnatal days 3 and 7. Relative counts of immature platelets (fraction, in %) were higher in infants with patent ductus arteriosus on day 7 but not on days 1 and 3. Receiver operating characteristic curve analysis unraveled associations between both lower mature platelet counts and higher immature platelet fraction (percentage) values on days 3 and 7, with haemodynamically significant ductus arteriosus. Logistic regression analysis revealed that mature platelet counts, but not immature platelet fraction values, were independent predictors of haemodynamically significant patent ductus arteriosus.

During the first week of postnatal life, lower counts of mature platelets and higher immature platelet fraction values are associated with haemodynamically significant patent ductus arteriosus. Lower counts of mature platelet were found to be independent predictors of haemodynamically significant patent ductus arteriosus.

Psoriasis is a chronic inflammatory, multi-system disease that often begins in childhood and characterised by inflammatory skin, nails, scalp, and joint manifestations. The inflammation in psoriasis may promote some effect on the cardiac conduction system.

The aim of this study is to investigate myocardial repolarisation anomaly on the conducting system in the paediatric psoriasis using P wave dispersion, Tpeak–Tend interval, and Tp-e/QT ratio.

Forty-two patients diagnosed with psoriasis and 37 age- and sex-matched healthy children were enrolled in the study. Electrocardiographic parameters in psoriasis and control group were recorded from an electrocardiogram for each patient.

The results indicated that the parameters including Pdis, QTc dis, Tp-e dis interval, and Tp-e max/QTmax ratios, which are known to be key indicators for the prediction of severe atrial or ventricular arrhythmia and sudden cardiac death and also important parameters used as the indicators for the non-invasive evaluation of the transmural heterogeneity were significantly longer in the study group compared to the control group (p < 0.05).

This study includes the evidence linking psoriasis with increased myocardial repolarisation heterogeneity. These findings suggest that this patient population may be at an increased risk for arrhythmias. Our findings may be a basis for further studies.

The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.

Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990–2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.

A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.

The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.

A certain degree of pulmonary stenosis after total correction of tetralogy of Fallot has been considered acceptable. But the long-term outcomes are not well understood. We observed the natural course of immediate pulmonary stenosis and investigated related factors for progression.

Fifty-two patients with acceptable pulmonary stenosis immediately after operation were enrolled. Acceptable pulmonary stenosis was defined as peak pressure gradient between 15 and 45 mmHg by Doppler echocardiography. Latent class linear mixed model was used to differentiate patients with progressed pulmonary stenosis, and the factors related to progression were analysed.

Pulmonary stenosis progressed in 14 patients (27%). Between the progression group and no progression group, there were no significant differences in operative age, sex, and the use of the transannular patch technique. However, immediate gradient was higher in the progression group (32.1 mmHg versus 25.7 mmHg, p = 0.009), and the cut-off value was 26.8 mmHg (sensitivity = 65.3%, specificity = 65.8%). Main stenosis at the sub-valve was observed more frequently in the progression group (85.7% versus 52.6%, p = 0.027). Despite no difference in the preoperative pulmonary valve z value, the last follow-up pulmonary valve z value was significantly lower in the progression group (−1.15 versus 0.35, p = 0.002).

Pulmonary stenosis immediately after tetralogy of Fallot total correction might progress in patients with immediate pulmonary stenosis higher than ≥26.8 mmHg and the main site was sub-valve area.

Children with congenital heart disease (CHD) have complex unique post-operative care needs. Limited data assess parents’ hospital discharge preparedness and education quality following cardiac surgery. The goals were to identify knowledge gaps in discharge preparedness after congenital heart surgery and to assess the acceptability of an educational mobile application to improve discharge preparedness.

Telephonic interviews with parents of children with two-ventricle physiology who underwent cardiac surgery 5–7 days post-discharge and in-person interviews with clinicians were conducted. We collected parent and clinician demographics, parent health literacy information and patient clinical data. We analysed interview transcripts using summative content analysis.

We interviewed 26 parents and 6 clinicians. Twenty-two of the 26 (85%) parents felt ready for discharge; 4 of the 6 (67%) clinicians did not feel most parents were ready for discharge. Fifteen of the 26 parents (58%) reported receiving the majority of discharge teaching on the day of discharge. Eight parents did not feel like all of their questions were answered. Most parents (14/26, 54%) preferred visual educational learning aids and could accurately describe important aspects of care. Most parents (23/26, 88%) and all 6 clinicians felt a mobile application for post-operative care education would be helpful.

Most parents received education on the day of discharge and could describe the information they received prior to discharge, although there were some preparedness gaps identified after discharge. Clinicians and parents varied in their perceptions of the readiness for discharge. Most responses suggest that a mobile application for discharge education may be helpful for transition to home.

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.