The efficacy of a specialized pediatric cardiac rapid response team is unknown. We hypothesized that a specialized cardiac rapid response team would facilitate team-wide communication between the cardiac stepdown unit and cardiac intensive care unit (ICU) teams and improve patient care.

A specialized pediatric cardiac rapid response team was implemented in June 2015. All pediatric cardiac rapid response team activations and outcomes from implementation through December 2018 were reviewed. Cardiac arrests and unplanned transfers to the cardiac ICU were indexed to 1000 patient-days to account for inpatient volume trends and evaluated over time.

There were 202 cardiac rapid response team activations in 108 unique patients during the study period. After implementation of the pediatric cardiac rapid response team, unplanned transfers from the cardiac stepdown unit to the cardiac ICU decreased from 16.8 to 7.1 transfers per 1000 patient days (p = 0.012). The stepdown unit cardiac arrest rate decreased from 1.2 to 0.0 arrests per 1000 patient-days (p = 0.015). There was one death on the cardiac stepdown unit in the 5 years since the implementation of the cardiac rapid response team, compared to four deaths in the previous 5 years.

A reduction in unplanned cardiac ICU transfers, cardiac arrests, and mortality on the cardiac stepdown unit has been observed since the implementation of a specialized pediatric cardiac rapid response team. A specialized cardiac rapid response team may improve communication and empower the interdisciplinary care team to escalate care for patients experiencing clinical decline.

Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them.

We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology.

Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of −0.70 (−1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036).

The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

This study demonstrates the clinical and electrophysiological details of catheter ablation conducted in children with focal atrial tachycardia using three-dimensional electroanatomic mapping systems.

Electrophysiological procedures were performed using the EnSite™ system.

Between 2014 and 2020, 60 children (median age 12.01 years [16 days–18 years]; median weight 41.5 kg [3–98 kg]) with focal atrial tachycardia and treated with catheter ablation were evaluated retrospectively. Tachycardia-induced cardiomyopathy was developed in 15 patients (25%). Most of the focal atrial tachycardia foci were right-sided (75%), and more than one focus was found in four patients. Radiofrequency ablation was performed in 47 patients (irrigated radiofrequency ablation in seven cases), cryoablation in 9, and radiofrequency ablation and cryoablation in the same session in 4 patients. The median procedural time was 163.5 minutes (82–473 minutes). Fluoroscopy was used in 29 of (48.3%) patients (especially for left-side substrate) with a mean time of 8.6 ± 6.2 minutes. The acute success rate was 95%. The procedure failed in three patients, and recurrence was observed in 3.5% of patients (2/57) during a median follow-up of 17 months (2–69 months). The second ablation was performed in four cases, of which three were successful. Overall success rate was 96.6% with no major complications observed, except in one patient with minimal pericardial effusion.

Catheter ablation seems to be an effective and safe treatment in focal atrial tachycardia. Electroanatomic mapping system can facilitate the ablation procedure and minimise radiation exposure.

Catheter ablation is a safe and effective therapy for the treatment of supraventricular tachycardia in children. Current improvements in technology have allowed progressive reduction in radiation exposure associated with the procedure. To assess the impact of three-dimensional mapping, we compared acute procedural results collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to published results from the Prospective Assessment after Pediatric Cardiac Ablation study.

Inclusion and exclusion criteria from the Prospective Assessment after Pediatric Cardiac Ablation study were used as guidelines to select patient data from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry to compare acute procedural outcomes between cohorts. Outcomes assessed include procedural and fluoroscopy exposure times, success rates of procedure, and complications.

In 786 ablation procedures, targeting 498 accessory pathways and 288 atrioventricular nodal reentrant tachycardia substrates, average procedural time (156.5 versus 206.7 minutes, p < 0.01), and fluoroscopy time (1.2 versus 38.3 minutes, p < 0.01) were significantly shorter in the study group. Success rates for the various substrates were similar except for manifest accessory pathways which had a significantly higher success rate in the study group (96.4% versus 93.0%, p < 0.01). Major complication rates were significantly lower in the study group (0.3% versus 1.6%, p < 0.01).

In a large, multicentre study, three-dimensional systems show favourable improvements in clinical outcomes in children undergoing catheter ablation of supraventricular tachycardia compared to the traditional fluoroscopic approach. Further improvements are anticipated as technology advances.

In recent years, cardiac resynchronization therapy (CRT) has also started to be performed in the paediatric and CHD population. This study aimed to evaluate the efficacy of CRT in children with CHD.

Patients with CHD who underwent CRT treatment in our paediatric cardiology clinic between January, 2010 and January, 2020 were included in the study. Demographic findings, 12-lead electrocardiograms, echocardiograms, clinical characteristics, management strategies, and outcomes were reviewed systematically.

The study population consisted of 18 CHD patients who had been treated with CRT for 10 years in our institution. The median age was 11 years (2.2–18 years) and the median weight was 39 kg (10–81 kg). Systemic ventricle was left ventricle in 13 patients, right ventricle in 4 patients, and 1 patient had single-ventricle physiology. CRT implantation indications were as follows: dysfunction after permanent pacemaker in 11 patients, dysfunction after left bundle branch block in 4 patients, and systemic ventricular dysfunction in 3 patients. CRT implantation techniques were epicardial (n = 13), hybrid (n = 4), and transvenous (n = 1) methods. QRS duration significantly decreased after CRT implantation (160 versus 124 m/second, p < 0.05). Median systemic ventricle ejection fraction (EF) significantly increased after the procedure (30 versus 50%, p < 0.05). Fourteen patients (78%) were responders, two patients (11%) were superresponders, and two patients (11%) were non-responders after the CRT treatment. One patient deceased during follow-up. Median follow-up duration was 40 months (6–117 months).

When electromechanical dyssynchrony occurs in paediatric cases with CHD and developing heart failure, patients should be evaluated in terms of CRT to improve ventricular function. Alternative CRT therapy will be beneficial in these cases that do not improve clinically despite optimal medical treatment.

Arrhythmias are common in the post-operative course of patients with hypoplastic left heart syndrome. We sought to determine the types, incidence, risk factors, and impact of arrhythmias in patients with HLHS and anatomic variants.

We performed a retrospective chart review of 120 consecutive patients with HLHS and anatomical variants, who had single-ventricle palliation at our institution from January, 2006 to December, 2016.

A total of thirty-one patients (26%) had 37 episodes of arrhythmias over a median follow-up period of 3.5 years. Of the 37 episodes, 12 (32.4%) were ectopic atrial tachycardia, 9 (24.3%) were paroxysmal supraventricular tachycardia, 4 (10.8%) were junctional ectopic tachycardia, 5 (13.6%) were sinus node dysfunction, 3 (8.1%) were heart block, 2 (5.4%) were atrial flutter, and 2 (5.4%) were ventricular tachycardia. Twenty-four (65%) of the arrhythmias occurred at post-stage 1 surgery. Most (64.8%) of the arrhythmias were resolved. Arrhythmias that occurred at post-stage 1 surgery were more likely to resolve compared to post-stages 2 or 3 (p = 0.006). No anatomical, surgical, or clinical variables were associated with arrhythmia except for age (OR per unit decrease in age at stage 1 palliation: 1.12 (95% CI 1.003, 1.250); p = 0.0439). Arrhythmias were not associated with length of hospital stay or mortality.

Arrhythmias are common in patients with HLHS and anatomic variants, with EAT and PSVT being the most common types. Arrhythmias were associated with younger age at surgery, but did not affect mortality or length of hospital stay.

Improved survival has led to a growing population of adults with congenital heart disease (CHD), followed by numerous reports of late complications. Liver disease is a known complication in some patients, with most studies focusing on Fontan associated liver disease. Whether liver disease also exists in other patients with CHD is not fully investigated. Elevated central venous pressure is considered pivotal in the development of liver disease in Fontan associated liver disease, and other patients with alterations in central venous pressure may also be at risk for developing liver fibrosis. We wanted to see if liver fibrosis is present in patients with tetralogy of Fallot. Many patients with tetralogy of Fallot have severe pulmonary regurgitation, which can lead to elevated central venous pressure. Patients with tetralogy of Fallot may be at risk of developing liver fibrosis.

Ten patients (24–56 years) with tetralogy of Fallot and pulmonary regurgitation were investigated for liver fibrosis. All patients were examined with magnetic resonance elastography of liver, hepatobiliary iminodiacetic acid scan, indocyanine green elimination by pulse spectrophotometry, elastography via FibroScan, abdominal ultrasound including liver elastography, and blood samples including liver markers.

Three out of ten patients had findings indicating possible liver fibrosis. Two of these had a liver biopsy performed, which revealed fibrosis stage 1 and 2, respectively. The same three patients had an estimated elevated central venous pressure in previous echocardiograms.

Mild liver fibrosis was present in selected patients with tetralogy of Fallot and may be related to elevated central venous pressure.

To determine if triiodothyronine alters lactate, glucose, and pyruvate metabolism, and if serum pyruvate concentration could serve as a predictor of low cardiac output syndrome in children after cardiopulmonary bypass procedures.

This study was ancillary to the Oral Triiodothyronine for Infants and Children undergoing Cardiopulmonary bypass (OTICC) trial. Serum pyruvate was measured in the first 48 patients and lactate and glucose were measured in all 208 patients enrolled in the OTICC study on the induction of anaesthesia, 1 and 24 hours post-aortic cross-clamp removal. Patients were also defined as having low cardiac output syndrome according to the OTICC trial protocol.

Amongst the designated patient population for pyruvate analysis, 22 received placebo, and 26 received triiodothyronine (T3). Lactate concentrations were nearly 20 times greater than pyruvate. Lactate and pyruvate levels were not significantly different between T3 and placebo group. Glucose levels were significantly higher in the placebo group mainly at 24-hour post-cross-clamp removal. Additionally, lactate and glucose levels peaked at 1-hour post-cross-clamp removal in low cardiac output syndrome and non-low cardiac output syndrome patients, but subsequently decreased at a slower rate in low cardiac output syndrome. Lactate and pyruvate concentrations correlated with glucose only prior to surgery.

Thyroid supplementation does not alter systemic lactate/pyruvate metabolism after cardiopulmonary bypass and reperfusion. Pyruvate levels are not useful for predicting low cardiac output syndrome. Increased blood glucose may be regarded as a response to hypermetabolic stress, seen mostly in patients with low cardiac output syndrome.

To evaluate the reliability of balloon coronary compression testing during percutaneous pulmonary valve implantation.

Despite the widespread use of the ‘balloon coronary test’ as the preferable method to rule out the risk of coronary compression, this adverse event has been described after pulmonary valve implantation where coronary balloon test suggested no risk or low risk, calling into question the accuracy of the test.

We performed a retrospective chart review of 84 patients who underwent pulmonary valve implantation between January 2018 and December 2019 and selected 36 patients whose archived imaging was suitable to perform quantitative analysis of the ‘balloon coronary test’. We focused on the spatial disparity between the right ventricular outflow tract position defined by the inflated testing balloon and the eventual implanted valve position, to classify the test as inaccurate or accurate.

In total, 36.1% of cases were classified as having an inaccurate coronary balloon test. Among the baseline characteristics, right ventricular outflow tract substrate was identified as a significant predictor of test accuracy. Related to this characteristic, the type of testing balloon used and the size of the eventually implanted valve were found to be associated with test accuracy.

Based on our findings, balloon coronary testing is not an accurate method of predicting final valve position with respect to fixed structures in the thorax. This may translate to a high false positive rate for the likelihood of coronary compression in pulmonary valve implantation.

With the increased training loads at very early ages in European elite youth soccer, there is an interest to analyse coronary artery remodelling due to high-intensity exercise.

Prospective echocardiographic study in 259 adolescent elite male soccer players and 48 matched controls.

The mean age was 12.7 ± 0.63 years in soccer players and 12.6 ± 0.7 years in controls (p > 0.05). Soccer players had significant greater indexed left ventricular mass (93 ± 13 g/m2 versus 79 ± 12 g/m2, p = 0.001). Both coronary arteries origin could be identified in every participant. In soccer players, the mean diameter of the left main coronary artery was 3.67 mm (SD ± 0.59) and 2.61 mm (SD ± 0.48) for right main coronary artery. Controls showed smaller mean luminal diameter (left main coronary artery, p = 0.01; right main coronary artery, p = 0.025). In soccer players, a total of 91% (n = 196) and in controls a total of 94% (n = 45) showed left main coronary artery z scores within the normal range: −2.0 to 2.0. In right main coronary artery, a pattern of z score values distribution was comparable (soccer players 94%, n = 202 vs. controls 84%, n = 40). A subgroup of soccer players had supernormal z score values (>2.0 to 2.5) for left main coronary artery (9%, n = 19, p = 0.01) and right main coronary artery (6%, n = 10, p = 0.025), respectively.

Elite soccer training in early adolescence may be a stimulus strong enough to develop increased coronary arteries diameters. In soccer players, a coronary artery z score >2.0–2.5 might reflect a physiologic response induced by multiannual high-intensity training.

Vocal cord palsy is one of the recognised complications of complex cardiac surgery in the paediatric population. While there is an abundance of literature highlighting the presence of this complication, there is a scarcity of research focusing on the pathophysiology, presentation, diagnosis, and treatment options available for children affected by vocal cord palsy.

Electronic searches were conducted using the search terms: “Vocal Cord Palsy,” “VCP,” “Vocal Cord Injury,” “Paediatric Heart Surgery,” “Congenital Heart Surgery,” “Pediatric Heart Surgery,” “Vocal Fold Movement Impairment,” “VFMI,” “Vocal Fold Palsy,” “PDA Ligation.” The inclusion criteria were any articles discussing the outcomes of vocal cord palsy following paediatric cardiac surgery.

The two main populations affected by vocal cord palsy are children undergoing aortic arch surgery or those undergoing PDA ligation. There is paucity of prospective follow-up studies; it is therefore difficult to reliably assess the current approaches and the long-term implications of management options.

Vocal cord palsy can be a devastating complication following cardiac surgery, which if left untreated, could potentially result in debilitation of quality of life and in severe circumstances could even lead to death. Currently, there is not enough high-quality evidence in the literature to aid recognition, diagnosis, and management leaving clinicians to extrapolate evidence from adult studies to make clinical judgements. Future research with a focus on the paediatric perspective is necessary in providing evidence for good standards of care.

Shape-memory abnormalities are seen in some nitinol atrial septal occluders. Variably described as cobra-head, tulip, and others, their incidence, mechanisms, clinical impact, and outcome have not been systematically analysed.

We retrospectively reviewed all consecutive device closures in the last 6 years for deformations. Type and size of the occluder, deployment technique, size, and angulation/kinking of the delivery sheath were analysed. Procedural success, duration, and other complications were studied.

A total of 112 devices (11.8%) among 950 occluders used in 936 patients showed deformities. Fourteen of 936 received 2 devices. Deformities were transient and self-correcting in 40%. Multivariate analysis showed significant associations with oversized sheaths (p = 0.004), kinked/angulated sheaths (p < 0.001), special deployment techniques (p < 0.001), and twist in the device waist (p = 0.011). Despite more frequent deformities with Figulla (15.6%) and Amplatzer (13.9%) occluders than Cera occluders (6.6%) and larger devices (>24 mm – 14.6%) than smaller devices (less than or equal to 24 mm – 9.7%), they were not significant on multivariate analysis. In vivo manipulations corrected most deformities; nineteen needed in vitro reformations and four needed a change of device. Despite prolongation of the procedure, repeated attempts (mean 2.76 ± 1.7 attempts, with a range from 1 to 9 attempts), and supraventricular tachycardia in two patients, there were no serious adverse effects.

Deformations were frequent in 11.8% of atrial septal occluders on a targeted search. Oversized and angulated/kinked sheaths, special techniques like pulmonary vein deployment and twist in device waist during procedure predisposed to deformities. While most deformities were corrected with manipulations, removal of the device was infrequently needed and change of device was rarely required. Long procedural time and multiple attempts for deployment did not affect procedural success.

Percutaneous balloon pericardiotomy is a percutaneous procedure that creates a window in the parietal pericardium by balloon dilation. The use of percutaneous balloon pericardiotomy has not been reported well in children.

The objective of this study was to describe the single centre experience of percutaneous balloon pericardiotomy in children.

This was a retrospective study to describe all the children aged <20 years undergoing percutaneous balloon pericardiotomy during an 18-year period (2001–2019). Patient characteristics, technical and ultimate procedural success, and repeat interventions were collected.

A total of 13 percutaneous balloon pericardiotomy’s were performed in 11 children at the median age of 12 years (range 1.8–19). The etiologies of pericardial effusion were post-pericardiotomy syndrome (n = 4), restrictive cardiomyopathy (n = 1), autoimmune diseases (n = 3), malignancy (n = 2), and idiopathic (n = 1). Two patients received two percutaneous balloon pericardiotomy. The technical success of percutaneous balloon pericardiotomy was 100% with no acute adverse events (balloon rupture or local bleeding). Five (45%) required re-intervention and ultimately three required a surgical pericardial window 6 to 35 days after the percutaneous balloon pericardiotomy. As a result, ultimate procedural success rate was 73% (8/11).

Percutaneous balloon pericardiotomy was performed safely with high technical success in children. Percutaneous balloon pericardiotomy may be considered for recurrent and persistent pericardial effusion, before considering a surgical pericardial window.

Pulmonary arteriovenous malformations in single ventricle congenital heart disease are poorly understood. Previous studies investigating pulmonary arteriovenous malformations predominantly focus on patients with heterotaxy syndrome and interrupted inferior caval vein. It is unknown if development and resolution of pulmonary arteriovenous malformations are similar for patients with and without heterotaxy syndrome.

In this retrospective single-institution study, we identified patients with a history of single ventricle congenital heart disease and Fontan palliation. We then matched patients with heterotaxy syndrome (intact and interrupted inferior caval vein) and non-heterotaxy hypoplastic left heart syndrome. To compare development of pulmonary arteriovenous malformations, we identified the frequency of positive diagnoses pre-Fontan. To compare resolution of pulmonary arteriovenous malformations, we recorded oxygen saturation changes for 12 months following Fontan.

A total of 124 patients were included. Patients with heterotaxy and interrupted inferior caval vein were more likely to have a pre-Fontan contrast echocardiogram performed (p < 0.01) and more likely to be diagnosed with pulmonary arteriovenous malformations pre-Fontan (p < 0.01). There was no difference in oxygen saturation prior to Fontan, yet all patient groups had increased their oxygen saturations in the first year after Fontan discharge.

Pulmonary arteriovenous malformations are variably diagnosed prior to Fontan palliation; however, all study groups had increased oxygen saturations after Fontan discharge, potentially indicating resolution of pulmonary arteriovenous malformations in all groups. The prevalence of pulmonary arteriovenous malformations pre-Fontan is likely underestimated. A quantitative, systematic approach to diagnosis and follow-up of pulmonary arteriovenous malformations is needed to better understand susceptibility and pathophysiology.

To detect early left ventricular dysfunction in children with non-alcoholic fatty liver disease using three-dimensional speckle tracking echocardiography.

Forty obese children with non-alcoholic fatty liver disease were included as group I. Another 40 obese children without non-alcoholic fatty liver disease of matched age, sex, and weight were included as group II. Forty healthy controls of matched age and sex served as a control group. Anthropometric measurements, laboratory investigations, and echocardiographic examinations including three-dimensional speckle tracking echocardiography were measured for all included children.

Abnormal lipid profile was detected in children with non-alcoholic fatty liver disease. Troponin I levels were significantly higher in children with non-alcoholic fatty liver disease compared to obese children without non-alcoholic fatty liver disease and to healthy controls. Three-dimensional speckle tracking echocardiography examination revealed a significant reduction of left ventricular global longitudinal strain, circumferential strain, radial strain, and area strain in children with non-alcoholic fatty liver disease inspite of normal left ventricular fraction shortening measured by conventional echocardiography. All strains were negatively correlated with the grade of non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease is associated with subclinical left ventricular dysfunction. Three-dimensional speckle tracking echocardiography can be helpful in identifying early left ventricular dysfunction in children with non-alcoholic fatty liver disease even in the presence of normal left ventricular ejection fraction.