Children with left aortic arch and aberrant right subclavian artery may present with either respiratory or swallowing symptoms beyond the classically described solid-food dysphagia. We describe the clinical features and outcomes of children undergoing surgical repair of an aberrant right subclavian artery.

This was a retrospective review of children undergoing repair of an aberrant right subclavian artery between 2017 and 2022. Primary outcome was symptom improvement. Pre- and post-operative questionnaires were used to assess dysphagia (PEDI-EAT-10) and respiratory symptoms (PEDI-TBM-7). Paired t-test and Fisher’s exact test were used to analyse symptom resolution. Secondary outcomes included perioperative outcomes, complications, and length of stay.

Twenty children, median age 2 years (IQR 1–11), were included. All presented with swallowing symptoms, and 14 (70%) also experienced respiratory symptoms. Statistically significant improvements in symptoms were reported for both respiratory and swallowing symptoms. Paired (pre- and post-op) PEDI-EAT-10 and PEDI-TBM-7 scores were obtained for nine patients, resulting in mean (± SD) scores decreasing (improvement in symptoms) from 19.9 (± 9.3) to 2.4 (± 2.5) p = 0.001, and 8.7 (± 4.7) to 2.8 (± 4.0) p = 0.006, respectively. Reoperation was required in one patient due to persistent dysphagia from an oesophageal stricture. Other complications included lymphatic drainage (n = 4) and transient left vocal cord hypomobility (n = 1).

Children with a left aortic arch with aberrant right subclavian artery can present with oesophageal and respiratory symptoms beyond solid food dysphagia. A thorough multidisciplinary evaluation is imperative to identify patients who can benefit from surgical repair, which appears to be safe and effective.

Outcomes for children with heart disease improved over the past decades. Quality improvement (QI) research in paediatric cardiac critical care is a key driver of improvement. The availability and variability of QI research across the field is unknown. This project represents a step in understanding the role. The Pediatric Cardiac Intensive Care Society (PCICS) can serve to support institutions’ needs, drive collaborations, and utilise available infrastructure at member institutions for improvement work.

The PCICS Quality Improvement and Safety Committee developed a survey to assess the state of QI research. The survey was disseminated over several months and available via QR code at the World Congress of Pediatric Cardiology and Cardiac Surgery in 2023.

Fifty-eight respondents completed the survey representing at least 38 unique institutions. Most respondents participated in QI research (52/58, 90%). Most QI projects were single centre (41% of respondents), and of those, the majority were from a minority of institutions (13 institutions [34% of total institutions]). QI support is available at slightly more than half of units, and 55% (32/58) have access to a QI specialist. QI support and rate of publications is significantly lower for small/medium units as compared to large units. Respondents suggested most interest from PCICS in networking with other members with similar project ideas (50/58, 86%).

PCICS member institutions are committed to QI research, with limitations in support, local specialists, and networking. Increasing connectivity and accessibility to QI resources may reduce burden to individual members and institutions to achieve QI research.

Ductal stenting in late presenters with transposition of great arteries with intact ventricular septum retrains the left ventricle before arterial switch operation. However, the experience is limited for its efficacy and safety. This study aims to highlight the efficacy and safety of ductal stenting for retraining the left ventricle.

Eight children with transposition of great arteries-intact ventricular septum and regressed left ventricle underwent ductal stenting. Serial echocardiographic measurements of left ventricle shape, mass, volume, free wall thickness, and function were done, and arterial switch operation was performed once the left ventricle was adequately prepared. Post-operative outcome in terms of duration of mechanical ventilation, ICU stay, and improvement in left ventricle function were monitored.

The procedure was successful in all patients. Babies were divided into two groups on basis of age at ductal stenting (group 1 age less than 90 days and group 2 age more than 90 days) and were evaluated for the degree of left ventricle retraining as evidenced by echocardiographic parameters and post-operative variables. The left ventricle posterior wall thickness and mass index after ductal stenting increased significantly in both the groups. Postoperatively, one baby of group two expired after seven days due to severe left ventricle dysfunction. Rest babies had an uneventful post-operative ICU stay with no statistical difference in the duration of invasive mechanical ventilation or ICU stay. On six-month follow-up, all surviving babies were doing well with normal left ventricle function.

Ductal stenting is a good alternative measure as compared to surgical procedures for left ventricle retraining in transposition of great arteries with regressed left ventricle.

Thromboembolism is a complication in paediatric patients with CHD requiring cardiac surgery. Previous research has focused on post-operative thromboembolism. This study aimed to describe thromboembolism frequency before or after cardiac surgery in children with CHD.

We performed a single-centre retrospective study from October 2020 to June 2023 (inclusive). Patients were eligible for inclusion if they were <21 years of age and underwent cardiac surgery. Outcomes of interest included the occurrence and characteristics of thromboembolism in the 12 months before and after surgery, antithrombotic therapies, recurrent thromboembolism, and clinically significant bleeding.

Among 260 patients included, 35 (13.5%) developed an index thromboembolism. Twelve (34.3%) patients had an index thromboembolism <12 months before surgery and 23 (65.7%) had an index thromboembolism <12 months after surgery, including 8 (22.9%) patients who had thromboembolism during both exposure periods. The median interquartile range (IQR) time of thromboembolism relative to cardiac surgery was –26 (–4 to –140) days and 15 (4 to 41) days, respectively. Seven (20%) patients had arterial, 18 (51.4%) venous, and 3 (8.6%) had both arterial and venous thromboembolism. Median (IQR) antithrombotic therapy duration was 49 (24–84) days. Nine (25.7%) patients developed recurrent thromboembolism and five (14.3%) patients experienced clinically significant bleeding.

The risk of thromboembolism and recurrence is high both before and after cardiac surgery among paediatric patients with CHD. Prospective multi-centre studies should seek to identify risk factors for preoperative and postoperative thromboembolism to inform the design of future risk-stratified thromboembolism prevention trials in children with CHD.

New drugs to target different pathways in pulmonary hypertension has resulted in increased combination therapy, but details of this use in infants are not well described. In this large multicenter database study, we describe the pharmacoepidemiology of combination pulmonary vasodilator therapy in critically ill infants.

We identified inborn infants discharged home from a Pediatrix neonatal ICU from 1997 to 2020 exposed to inhaled nitric oxide, sildenafil, epoprostenol, or bosentan for greater than two consecutive days. We compared clinical variables and drug utilisation between infants receiving simultaneous combination and monotherapy. We reported each combination’s frequency, timing, and duration and graphically represented drug use over time.

Of the 7681 infants that met inclusion criteria, 664 (9%) received combination therapy. These infants had a lower median gestational age and birth weight, were more likely to have cardiac and pulmonary anomalies, receive cardiorespiratory support, and had higher in-hospital mortality than those receiving monotherapy. Inhaled nitric oxide and sildenafil were most frequently used, and utilisation of combination and monotherapy for all drugs increased over time. Inhaled nitric oxide and epoprostenol were used in infants with a higher gestational age, earlier postnatal age, and shorter duration than sildenafil and bosentan. Dual therapy with inhaled nitric oxide and sildenafil was the most common combination therapy.

Our study revealed an increased use of combination pulmonary vasodilator therapy, favouring inhaled nitric oxide and sildenafil, yet with considerable practice variation. Further research is needed to determine the optimal combination, sequence, dosing, and disease-specific indications for combination therapy.

The risk factors for reoperation and mortality after partial and intermediate atrioventricular canal defect repair are unclear. This study assessed the mid-term outcomes and risk factors for reoperation and mortality after partial and intermediate atrioventricular canal defect surgery.

Ninety-seven patients who underwent primary repair of intermediate (n = 45) or partial (n = 52) atrioventricular canal defect between 2005 and 2019 were included in this single-centre study.

The median age was 5 years (2.7–8.9 years). The median follow-up time was 32 months (1.6–90.8 months). The estimated freedom from reoperation at 1, 5, and 10 years was 97%, 91%, and 73%, respectively.

In multivariable analyses, post-operative left atrioventricular valve regurgitation of grade II or higher (odds ratio [OR]: 5.3, 95% confidence interval [CI]: 1.8–15.5, p = 0.01) and post-operative residual intracardiac shunt (OR: 11.6, 95% CI: 1.6–85.8, p = 0.02) were risk factors for reoperation.

In multivariable analyses, perioperative reoperation (OR: 93.4, 95% CI: 3.9–218.7, p = 0.01) and the need for right atrioventricular valve repair (OR: 11.2, 95% CI: 1.0 – 123.3, p = 0.04) were risk factors for mortality. Mortality was higher in patients under 2.6 years of age.

For patients undergoing repair of partial or intermediate atrioventricular canal defect, those with post-operative left atrioventricular valve regurgitation of grade II or higher and post-operative residual intracardiac shunt have an increased reoperation risk. Higher mortality can be expected after a perioperative reoperation, and in patients requiring right atrioventricular valve repair during the index procedure.

Haemodynamic instability is common after surgical repair of CHDs in infants and children. Monitoring cardiac output in addition to traditional circulation parameters could improve the postoperative care of these patients. Echocardiography and transpulmonary thermodilution are the two most common methods for measuring cardiac output in infants.

To compare the results of cardiac output measurements using echocardiography and a transpulmonary thermodilution setup after paediatric cardiac surgery.

Forty children, scheduled for elective repair of a ventricular septal defect or of an atrio-ventricular septal defect using cardiopulmonary bypass, were enrolled in this prospective, observational study. Cardiac output was simultaneously measured using echocardiography and a commercially available transpulmonary thermodilution method (PiCCO™) at 18 h after the end of surgery.

At 18 h after surgery, PiCCO™ gave a mean of 3.0% higher cardiac output than echocardiography. This difference was not statistically significant. 95% of the observations fell within –50.0 to 82.6%.

The methods were found to have a good agreement on average, with no statistically significant difference between them. However, the spread of the results was large. It is questionable whether the methods can be used interchangeably in clinical practice.

Myocarditis represents a diverse group of inflammatory diseases affecting the heart muscle, with both infectious and non-infectious etiologies. Among the non-infectious causes, drug-induced hypersensitivity reactions are rare but serious. Isoniazid, a cornerstone in tuberculosis treatment, is known for its hepatotoxicity but has rarely been documented to cause hypersensitivity myocarditis.

We present a case of a 15-year-old girl from Eastern Turkmenistan who was admitted to our emergency department with altered consciousness and seizure activity. She was diagnosed with status epilepticus and treated accordingly. The patient, with no prior medical history, was found to have hypotensive shock and myocarditis upon further examination. A detailed history revealed she had ingested 45 tablets of expired isoniazid in a suicide attempt. She was treated with pyridoxine and supportive therapies, resulting in a gradual recovery.

This case underscores the critical need to consider drug-induced hypersensitivity myocarditis in the differential diagnosis of myocarditis, especially in patients with recent medication use. Prompt recognition and appropriate treatment with pyridoxine, steroid, and supportive cardiac care can be lifesaving. This case also highlights the importance of awareness regarding the potential cardiotoxic effects of isoniazid overdose.

Emerging evidence suggests that routine physical activity may improve exercise capacity, long-term outcomes, and quality of life in individuals with Fontan circulation. Despite this, it is unclear how active these individuals are and what guidance they receive from medical providers regarding physical activity. The aim of this study was to survey Fontan patients on personal physical activity behaviours and their cardiologist-directed physical activity recommendations to set a baseline for future targeted efforts to improve this.

An electronic survey assessing physical activity habits and cardiologist-directed guidance was developed in concert with content experts and patients/parents and shared via a social media campaign with Fontan patients and their families.

A total of 168 individuals completed the survey. The median age of respondents was 10 years, 51% identifying as male. Overall, 21% of respondents spend > 5 hours per week engaged in low-exertion activity and only 7% spend > 5 hours per week engaged in high-exertion activity. In all domains questioned, pre-adolescents reported higher participation rates than adolescents. Nearly half (43%) of respondents reported that they do not discuss activity recommendations with their cardiologist.

Despite increasing evidence over the last two decades demonstrating the benefit of exercise for individuals living with Fontan circulation, only a minority of patients report engaging in significant amounts of physical activity or discussing activity goals with their cardiologist. Specific, individualized, and actionable education needs to be provided to patients, families, and providers to promote and support regular physical activity in this patient population.

This study aims to assess the surgical outcome of borderline hypoplastic left ventricle before and after the induction of the left ventricle rehabilitation strategy.

A retrospective review investigated patients with borderline hypoplastic left ventricle who underwent surgical intervention between 2012 and 2022. The patient cohort was stratified into two groups based on the initiation of left ventricle rehabilitation: an early-era group (E group, 2012–2017) and a late-era group (L group, 2018–2022). Left ventricle rehabilitation was defined as palliation combined with other procedures aimed at promoting left ventricular growth such as restriction of atrial septal defect, relief of inflow/outflow obstructive lesions, and resection of endocardial fibroelastosis.

A total of 58 patients were included. Primary diagnosis included 12 hypoplastic left heart syndromes, 11 critical aortic valve stenosis, and others. A total of 9 patients underwent left ventricle rehabilitation, 8 of whom underwent restriction of atrial septal defect. As for clinical outcomes, 9 of 23 patients achieved biventricular repair in the E group, whereas in the L group, 27 of 35 patients achieved biventricular repair (39% vs. 77%, p = 0.004). Mortality did not differ statistically between the two groups (log-rank test p = 0.182). As for the changes after left ventricle rehabilitation, left ventricular growth was observed in 8 of 9 patients. The left ventricular end-diastolic volume index (from 11.4 to 30.1 ml/m2, p = 0.017) and left ventricular apex-to-right ventricular apex ratio (from 86 to 106 %, p = 0.014) significantly increased after left ventricle rehabilitation.

The introduction of the left ventricle rehabilitation strategy resulted in an increased proportion of patients achieving biventricular repair without a concomitant increase in mortality. Left ventricle rehabilitation was associated with enhanced left ventricular growth and the formation of a well-defined left ventricle apex. Our study underscores the significance of left ventricle rehabilitation strategies facilitating successful biventricular repair. The data suggest establishing restrictive atrial communication may be a key factor in promoting left ventricular growth.

The time constant of isovolumic relaxation is an established index of ventricular relaxation, a major component of diastolic function, even in a single right ventricle. However, the specific echocardiographic parameters for estimating diastolic dysfunction are insufficient for a single right ventricle. This study aimed to investigate the echocardiographic indices associated with time constant of isovolumic relaxation in post-Fontan operation patients with a single right ventricle.

We included 39 patients with hypoplastic left heart syndrome after Fontan operation with an ejection fraction ≥45% and preserved valve function. First, the correlation between echocardiographic parameters and time constant of isovolumic relaxation was examined, and partial correlation coefficients were calculated using age and heart rate as covariates. Next, univariate regression analysis was performed using time constant of isovolumic relaxation as the objective variable and echocardiographic parameters as independent variables, followed by multivariate regression analysis incorporating parameters with p < 0.10.

Among the echocardiographic parameters, global longitudinal strain correlated most strongly with time constant of isovolumic relaxation (r = 0.778, p < 0.001). This was consistent with the partial correlation coefficients (r = 0.707, p < 0.001). Using stepwise multivariate regression analysis, only global longitudinal strain was found to be an independent predictor of time constant of isovolumic relaxation (adjusted R2 = 0.551).

Global longitudinal strain could be used as a surrogate marker of time constant of isovolumic relaxation, an invasive indicator of relaxation impairment, in post-Fontan operation patients with a single right ventricle, preserved ejection fraction, and valve function.

Echocardiography is essential for the evaluation of pulmonary hypertension. We determined the feasible quantitative parameter for screening and monitoring pulmonary hypertension in preterm infants.

This secondary analysis of a prospective cohort single-centre study was conducted between August 2019 and September 2020. Serial echocardiography was performed 7 and 28 days after birth and at 36 weeks postmenstrual age. The data of infants who developed pulmonary hypertension at 36 weeks postmenstrual age were compared with those without pulmonary hypertension. We also modelled the parameters’ trend and performed an interaction test using multi-level Gaussian regression.

Out of 30 infants enrolled in the study, 79 echocardiograms were analysed. Left ventricular eccentric index was obtainable in all infants, while tricuspid jet velocity was measurable in 44.1%. Left ventricular eccentric index correlated well with tricuspid regurgitation jet velocity (r = 0.77, P < 0.001). Six infants were diagnosed with newly developed or persistent pulmonary hypertension at 36 weeks postmenstrual age. Serial left ventricular eccentric index showed a significantly different increasing trend in the pulmonary hypertension group (change per day: +0.004; P = 0.090) from the decreasing trend among a non-pulmonary hypertension group (change per day: –0.001; P = 0.041) (P for interaction = 0.007). Right ventricular systolic function and right ventricular isovolumic systolic velocity revealed a reducing trend in the pulmonary hypertension group, which was different from the improving trend in non-pulmonary hypertension infants. Infants with low current weight, low postmenstrual age, and requiring high-flow oxygen therapy at day 28 of life trended to increase the risk of late pulmonary hypertension.

Left ventricular eccentric index and right ventricular isovolumic systolic velocity were feasible for assessing pulmonary hypertension and should be incorporated into pulmonary hypertension evaluation. Serial left ventricular eccentric index and right ventricular isovolumic systolic velocity may help predict late pulmonary hypertension and early detection of right ventricular dysfunction.