CHD care is resource-intensive. Unwarranted variation in care may increase cost and result in poorer health outcomes. We hypothesise that process variation exists within the pre-operative evaluation and planning process for children undergoing repair of atrial septal defect or ventricular septal defect and that substantial variation occurs in a small number of care points.

From interviews with staff of an integrated congenital heart centre, an initial process map was constructed. A retrospective chart review of patients with isolated surgical atrial septal defect and ventricular septal defect repair from 7/1/2018 through 11/1/2020 informed revisions of the process map. The map was assessed for points of consistency and variability.

Thirty-two surgical atrial septal defect/ventricular septal defect repair patients were identified. Ten (31%) were reviewed by interventional cardiology before surgical review. Of these, 6(60%) had a failed catheter-based closure and 4 (40%) were deemed inappropriate for catheter-based closure. Thirty (94%) were reviewed in case conference, all attended surgical clinic, and none were admitted prior to surgery. The process map from interviews alone identified surgery rescheduling as a point of major variability; however, chart review revealed this was not as prominent a source of variability as pre-operative interventional cardiology review.

Significant variation in the pre-operative evaluation and planning process for surgical atrial septal defect/ventricular septal defect patients was identified. If such process variation is widespread through CHD care, it may contribute to variations in outcome and cost previously documented within CHD surgery. Future research will focus on determining whether the variation is warranted or unwarranted, associated health outcomes and cost variation attributed to these variations in care processes.

Tetralogy of Fallot is the most prevalent cyanotic CHD. With the advent of advanced surgical methods, the majority of tetralogy of Fallot patients reach adulthood. However, many need re-intervention for the residual anomalies including residual right ventricular outflow obstruction, pulmonary regurgitation, residual ventricular septal defects, and progressive aortic dilatation. Aortic dilation could lead to aortic regurgitation or dissection requiring surgical correction. In the current study, we aimed to determine the prevalence and outcomes of aortic root dilatation in adults with repaired tetralogy of Fallot in our tertiary care centre.

In this retrospective study, 730 consecutive patients with history of repaired tetralogy of Fallot were included. Aortic diameter at the level of annulus, the sinus of Valsalva, sinotubular junction, and the ascending aorta as measured by echocardiography were evaluated. Prevalence of outcomes necessitating re-intervention including aortic regurgitation and dissection were recorded.

The mean size of annulus, sinus of Valsalva, sinotubular-junction, and ascending aorta in the latest available echocardiography of patients were 2.4+/-0.4 cm, 3.3+/-0.5 cm, 2.9+/-0.5cm, and 3.2+/-0.5cm, respectively. Prevalence of dilatation of sinus of Valsalva, dilation of Ascending aorta, sinotubular-junction, and aortic annulus was 28.7%, 21%, 8.3%, and 1 %, respectively. Five patients had severe aortic regurgitation (0.6%) and underwent surgical repair. One of these patients presented with acute aortic dissection.

Aortic dilation is common in tetralogy of Fallot but prevalence of redo surgery for aortic dilation, regurgitation, and adverse events including acute dissection is low.

This study aimed to assess the impact of caloric intake and weight-for-age-Z-score after the Norwood procedure on the outcome of bidirectional cavopulmonary shunt.

A total of 153 neonates who underwent the Norwood procedure between 2012 and 2020 were surveyed. Postoperative daily caloric intake and weight-for-age-Z-score up to five months were calculated, and their impact on outcome after bidirectional cavopulmonary shunt was analysed.

Median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Modified Blalock-Taussig shunt was used in 95 patients and right ventricle to pulmonary artery conduit in 58. Postoperatively, total caloric intake gradually increased, whereas weight-for-age-Z-score constantly decreased. Early and inter-stage mortality before stage II correlated with low caloric intake. Older age (p = 0.023) at Norwood, lower weight (p < 0.001) at Norwood, and longer intubation (p = 0.004) were correlated with low weight-for-age-Z-score (< –3.0) at 2 months of age. Patients with weight-for-age-Z-score < –3.0 at 2 months of age had lower survival after stage II compared to those with weight-for-age-Z-score of –3.0 or more (85.3 versus 92.9% at 3 years after stage II, p = 0.017). There was no difference between inter-stage weight gain and survival after bidirectional cavopulmonary shunt between the shunt types.

Weight-for-age-Z-score decreased continuously throughout the first 5 months after the Norwood procedure. Age and weight at Norwood and intubation time were associated with weight gain. Inter-stage low weight gain (Z-score < –3) was a risk for survival after stage II.

Head-up tilt test (HUTT) is an important tool in the diagnosis of pediatric vasovagal syncope. This research will explore the relationship between syncopal symptoms and HUTT modes in pediatric vasovagal syncope.

A retrospective analysis was performed on the clinical data of 2513 children aged 3–18 years, who were diagnosed with vasovagal syncope, from Jan. 2001 to Dec. 2021 due to unexplained syncope or pre-syncope. The average age was 11.76 ± 2.83 years, including 1124 males and 1389 females. The patients were divided into the basic head-up tilt test (BHUT) group (596 patients) and the sublingual nitroglycerine head-up tilt test (SNHUT) group (1917 patients) according to the mode of positive HUTT at the time of confirmed pediatric vasovagal syncope.

(1) Baseline characteristics: Age, height, weight, heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP), and composition ratio of syncope at baseline status were higher in the BHUT group than in the SNHUT group (all P < 0.05). (2) Univariate analysis: Age, height, weight, HR, SBP, DBP, and syncope were potential risk factors for BHUT positive (all P < 0.05). (3) Multivariate analysis: syncope was an independent risk factor for BHUT positive, with a probability increase of 121% compared to pre-syncope (P<0.001).

The probability of BHUT positivity was significantly higher than SNHUT in pediatric vasovagal syncope with previous syncopal episodes.

Acute asthma exacerbation is one of the most common reasons for paediatric emergency room visits and hospital admissions in the United States of America.

To assess the impact of CHD on outcomes of children hospitalised for acute asthma exacerbation.

Children primarily admitted for acute asthma exacerbation were sampled from 2006, 2009, 2012, and 2016 kid inpatient database of the Healthcare Cost and Utilization Project using ICD codes. The disease outcomes were compared between those with and without CHD using multivariate logistic regressions in Stata version 17.

There were a total of 639,280 acute asthma exacerbation admissions, of which 5,907 (0.92%) had CHD. The mortality rate was 0.079% for patients without CHD and 0.72% for those with co-existing CHD. Children with CHD had higher odds of mortality (5.51, CI 3.40–8.93, p < 0.001), acute respiratory failure (2.84, CI 2.53–3.20; p < 0.001), need for invasive mechanical ventilation (4.58, CI 3.80–5.52; p < 0.001), acute kidney injury (adjusted odds ratio 3.03, CI 3.03–7.44; p < 0.001), and in-hospital cardiac arrest (adjusted odds ratio 4.52, CI 2.49–8.19; p < 0.001) when compared with those without CHD. The adjusted mean length of hospital stays (CI 2.91–3.91; p < 0.001) and hospital charges (95% CI $31060–$47747) among children with acute asthma exacerbation and CHD were significantly higher than in those without CHD.

CHD is an independent predictor of mortality, more severe disease course, and higher hospital resource utilisation. Strategies that improve CHD care will likely improve the overall health outcomes of children with CHD hospitalised for acute asthma exacerbation.

Transcatheter pulmonary valve replacement serves as a successful alternative to surgical replacement of a right ventricle to pulmonary artery conduit. Guidelines for recommending transcatheter pulmonary valve replacement depend on MRI right ventricular volumes, which have been correlated to the echocardiographic measure of right ventricular annular tilt. We aim to assess whether right ventricular annular tilt can be a clinically useful alternative tool in the acute and long-term periods after transcatheter pulmonary valve replacement to assess right ventricular health.

We reviewed 70 patients who underwent transcatheter pulmonary valve replacement at a single institution. Echocardiographic measurements were obtained prior to transcatheter pulmonary valve replacement, immediately after transcatheter pulmonary valve replacement, and within 6 months to 1 year after transcatheter pulmonary valve replacement. Right ventricular annular tilt measures the angle of the tricuspid valve plane relative to the mitral valve plane at end-diastole in the apical four-chamber view. Right ventricular fractional area change, right ventricular systolic strain, tissue Doppler velocity, and tricuspid annular plane systolic excursion Z-scores were obtained using published methods.

Right ventricular annular tilt decreased significantly immediately after transcatheter pulmonary valve replacement (p = 0.0004), and this reduction in right ventricular volume persisted at the mid-term follow-up (p < 0.0001). Fractional area change did not change significantly after transcatheter pulmonary valve replacement while right ventricular global strain improved at mid-term follow-up despite no significant difference immediately after transcatheter pulmonary valve replacement.

Right ventricular annular tilt decreases both immediately after transcatheter pulmonary valve replacement and at mid-term follow-up. Right ventricular strain also improved after transcatheter pulmonary valve replacement, corresponding to the improved volume load. Right ventricular annular tilt can be considered as an additional echocardiographic factor to assess right ventricular volume and remodeling after transcatheter pulmonary valve replacement.

Carvajal syndrome is a very rare autosomal recessive cardiocutaneous disorder caused by a desmosomal mutation in exon 24 of the desmoplakin gene. It manifests with woolly hair, epidermolytic palmoplantar keratoderma, and arrhythmogenic right ventricular cardiomyopathy. We herein present a patient with heart failure and dilated cardiomyopathy who was diagnosed with Carvajal syndrome because of dermatologic manifestations.

A seven-year-old girl was referred to our clinic due to decompensated heart failure and clinical deterioration. The patient had severe weakness, tachycardia, and tachypnea. She had a complaint of getting tired quickly for three weeks, and she had shortness of breath and abdominal pain for the last two days. She had hepatomegaly and woolly hair. Mild keratoderma was present on the soles of her feet. Echocardiography demonstrated biventricular dilatation, significantly impaired left ventricular systolic function (ejection fraction 22%), and moderate to severe mitral and tricuspid regurgitation. Molecular genetic evaluation was performed because of cutaneous and cardiac findings, which demonstrated a desmoplakin gene mutation. Homozygous mutation c.4297C > T (p.Gln1433*) was identified in desmoplakin gene, and the diagnosis of Carvajal syndrome was confirmed.

Syndromic types of arrhythmogenic right ventricular cardiomyopathy such as Carvajal syndrome are rare diseases. Awareness about cutaneous manifestations and genetic evaluation would help diagnosis and prevention of sudden death. Genetic counselling is needed in familial cases.

The goal of this single-centre study was to evaluate long-term results of percutaneous closure of secundum-type atrial septal defect using Amplatzer Septal Occluder with the follow-up to 25 years.

A retrospective analysis of patients who underwent percutaneous closure of secundum-type atrial septal defect between September 1995 and October 2012 in our institution was performed. All procedures were performed after fulfilling strict indication criteria. More than 5 years follow-up was reached in 651/803 patients (81%) with median follow-up time of 12 (5–25) years.

The mean stretched defect diameter was 14,0 ± 5,2 mm. Early reintervention due to moderate or severe residual atrial septal defect had to be performed in 3/803 patients (0,03%). The incidence of long-term moderate or severe residual atrial septal defect was 0,0%. The complete closure rate at 10-year follow-up was 98,5%, as trivial residual shunts persisted in 8/508 patients (1,5%). A significant rate of the echocardiographic right ventricular end-diastolic diameter post-procedural normalisation (p < 0,05) was encountered. The rate of major complications was 0,5%. One device embolisation, one thrombus formation at the occluder surface, and one cardiac erosion in periprocedural or short post-procedural course were experienced. Only one late complication of infective endocarditis at the region of implanted occluder and the aortic valve was detected. The survival rate of all followed patients was 100%.

Percutaneous closure of secundum-type atrial septal defect using Amplatzer Septal Occluder is a safe and effective procedure accounting for a very low incidence of major complications in the long-term follow-up.

Pulmonary arterial hypertension, a common consequence of untreated CHD, is associated with a significant morbidity and mortality. Recent researches have demonstrated that patients with clinically severe cardiovascular illnesses, including pulmonary hypertension, have a greater mortality risk when their red cell distribution width is high. This work aimed to assess the predictive value of red cell distribution width in children with pulmonary arterial hypertension-CHD and to correlate red cell distribution width with various clinical and echocardiographic data.

Sixty patients with CHD associated with pulmonary arterial hypertension were enrolled as the patient group. Another 60 patients with CHD and no pulmonary arterial hypertension, matched for age and sex, were enrolled as the control group. Electrocardiography and echocardiographic evaluation were performed for all included children. Red cell distribution width as part of the complete blood count was also performed using a Coulter® LH 700 series haematology analyzer.

The red cell distribution width was significantly higher in the pulmonary arterial hypertension-CHD group than in the CHD-only group (P < 0.05). There was a significant positive correlation between the red cell distribution width and mean pulmonary artery pressure. Red cell distribution width was an independent predictor of mortality in children with pulmonary arterial hypertension-CHD. The best red cell distribution width cut-off for predicting mortality in children with pulmonary arterial hypertension-CHD was ≥ 17.6%.

Red cell distribution width was significantly higher in children with pulmonary arterial hypertension–CHD than in those without pulmonary arterial hypertension. Moreover, red cell distribution width could be a cheap easy predictive marker for mortality in children with pulmonary arterial hypertension-CHD.

CHDs correspond to 28% of all congenital anomalies, being the leading cause of infant mortality in the first year of life. Thus, it is essential to explore risk factors for CHDs presentation, allowing the detection of probable cases within a population.

We identified newborns with CHDs within a cohort from the Program for the Prevention and Monitoring of Congenital Defects in Bogota and Cali, 2002–2020. Cases were classified as isolated, complex isolated, polymalformed, and syndromic. Variables were analysed by comparing case and control averages with Student’s t test using a 95% confidence level.

Prevalence obtained was 19.36 per 10 000 live births; non-specified CHD, ventricular septal defect, and atrial septal defect were the most prevalent. As risk factors were found: paternal and maternal age above 45 years, pregestational diabetes, mother’s body mass index above 25, low educational level, and socio-economic status. As protective factors: folic acid consumption within the first trimester and pregestational period.

Different risk and protective factors associated with the presentation of CHDs have been described. We consider that public health strategies should be aimed to reduce risk factors exposure. Also, improving diagnosis and prognosis by having a close monitoring on high-risk patients.