The aim of this study was to evaluate the association of bicuspid aortic valve on contemporary outcomes, including reoperation rates, after one-stage correction for interrupted aortic arch with ventricular septal defect or for aortic coarctation with hypoplastic aortic arch and ventricular septal defect.

Seventy-four consecutive patients (35 boys, 47% and 39 girls, 53%) with interrupted aortic arch (n = 41, 55%) or aortic coarctation with hypoplastic aortic arch (n = 33, 45%) with ventricular septal defect underwent early one-stage correction. Twenty (27%) patients had bicuspid aortic valve, and the remaining 54 (73%) had a tricuspid aortic valve. The median aortic valve annulus diameter was 6.0 mm (IQR: 2.0). Patients’ median age was 7 ± 29 days (range, 2–150); median weight was 3.3 ± 0.7 kg (range, 1.5–6.0), with 21 (28%) patients <3.0 kg. Selective brain perfusion through the innominate artery and selective coronary perfusion through the aortic root during aortic arch reconstruction were used in all patients. Statistical analysis was performed using SPSS version 20.0 software (SPSS Inc., Chicago, IL, USA).

The early mortality was 1.3%. One premature neonate died in the hospital with extracorporeal membrane oxygenation after aortic coarctation plus ventricular septal defect repair. There was no further mortality. Median follow-up was 5.7 years (IQR: 10.48). Reinterventions occurred in 36 (49%) patients: balloon angioplasty in 18 (24%) patients, reoperations in 4 (5%) patients, and both in 14 (19%) patients. A total of 86 follow-up procedures were required in these 36 (49%) patients: aortic valve valvulopasty (n = 6, 8%), stent implantation (n = 8, 11%), balloon dilatation (n = 39, 53%), and reoperation (n = 33, 45%). The median time to reinterventions was 9.094 years (SE 0.890). A potential risk factor for reintervention after interrupted aortic arch and aortic coarctation with ventricular septal defect repair was bicuspid aortic valve (p = 0.019, Chi2 (1) = 5.457). In addition, a multivariate Cox analysis with backward selection and significance level <0.015 was applied to all variables that showed significant effects in univariable analyzes. This regression confirmed that bicuspid aortic valve (HR = 0.381, p = .016), and interrupted aortic arch (HR = 0.412, p = 0.043) were predictors of late reintervention. All patients had no obvious neurologic impairment in routine examinations at last follow-up.

Bicuspid aortic valve was a significant risk factor for valve-related reintervention after one-stage repair for aortic arch obstruction with ventricular septal defect due to later development of stenosis associated with higher late morbidity and mortality. Particularly neonates with bicuspid aortic valve will possibly require reintervention in the future. Regular lifelong cardiac follow-up is recommended.

To characterise the current approach to sedation, analgesia, iatrogenic withdrawal syndrome and delirium in paediatric cardiac ICUs.

A convenience sample survey of practitioners at institutions participating in the Pediatric Cardiac Critical Care Consortium conducted from September to December 2020.

Paediatric cardiac ICUs.

Survey responses were received from 33 of 42 institutions contacted. Screening for pain and agitation occurs commonly and frequently. A minority of responding centres (39%) have a written analgesia management protocol/guideline. A minority (42%) of centres have a written protocol for sedation. Screening for withdrawal occurs commonly, although triggers for withdrawal screening vary. Only 42% of respondents have written protocols for withdrawal management. Screening for delirium occurs “always” in 46% of responding centres, “sometimes” in 36% of centres and “never” 18%. Nine participating centres (27%) have written protocols for delirium management.

Our survey identified that most responding paediatric cardiac ICUs lack a standardised approach to the management of analgesia, sedation, iatrogenic withdrawal, and delirium. Screening for pain and agitation occurs regularly, while screening for withdrawal occurs fairly frequently, and screening for delirium is notably less consistent. Only a minority of centres use written protocols or guidelines for the management of these problems. We believe that this represents an opportunity to significantly improve patient care within the paediatric cardiac ICU.

This study aimed to evaluate electrocardiographic and echocardiographic findings, Holter recordings of the multisystem inflammatory syndrome in children, and to identify prognostic factors for cardiac involvement.

We retrospectively reviewed demographic characteristics, medical data, laboratory findings, electrocardiogram and echocardiographic findings, 24-hour Holter recordings, need for an ICU, and extracorporeal membrane oxygenation in multisystem inflammatory syndrome in children. Acute left ventricular systolic dysfunction was defined as left ventricular ejection fraction (EF) ≤%55 on echocardiography.

Sixty-seven children were included in the study. 24-hour Holters were recorded in 61.2% of the patients and 49.2% were normal. On echocardiographic examination, 14.9% of the patients had systolic dysfunction (EF ≤ 55%). While 32.8% of patients had mild mitral regurgitation, 3% had moderate mitral regurgitation, and 6% had mild aortic regurgitation. There was no statistically significant difference in EF values between the group with arrhythmia in Holter and the group with normal Holter results (p ≥ 0.05). B-type natriuretic peptide was positively correlated with C-reactive protein, ferritin, and fibrinogen. Significant effectivity of the B-type natriuretic peptide value was observed in the differentiation of those with EF ≤ and > 55%. Extracorporeal membrane oxygenation support was needed for three (4.5%) patients. One patient who died had systemic juvenile idiopathic arthritis.

Neutrophil/lymphocyte ratio, C-reactive protein, D-dimer, ferritin, troponin, and B-type natriuretic peptide were found to be significantly higher in patients with systolic dysfunction. Also, the cut-off value of 1700 pg/ml for B-type natriuretic peptide was significantly effective. These parameters may indicate the severity of the disease but should be supported by prospective studies.

Electroanatomic mapping systems are increasingly used during ablations to decrease the need for fluoroscopy and therefore radiation exposure. For left-sided arrhythmias, transseptal puncture is a common procedure performed to gain access to the left side of the heart. We aimed to demonstrate the radiation exposure associated with transseptal puncture.

Data were retrospectively collected from the Catheter Ablation with Reduction or Elimination of Fluoroscopy registry. Patients with left-sided accessory pathway-mediated tachycardia, with a structurally normal heart, who had a transseptal puncture, and were under 22 years of age were included. Those with previous ablations, concurrent diagnostic or interventional catheterisation, and missing data for fluoroscopy use or procedural outcomes were excluded. Patients with a patent foramen ovale who did not have a transseptal puncture were selected as the control group using the same criteria. Procedural outcomes were compared between the two groups.

There were 284 patients in the transseptal puncture group and 70 in the patent foramen ovale group. The transseptal puncture group had a significantly higher mean procedure time (158.8 versus 131.4 minutes, p = 0.002), rate of fluoroscopy use (38% versus 7%, p < 0.001), and mean fluoroscopy time (2.4 versus 0.6 minutes, p < 0.001). The acute success and complication rates were similar.

Performing transseptal puncture remains a common reason to utilise fluoroscopy in the era of non-fluoroscopic ablation. Better tools are needed to make non-fluoroscopic transseptal puncture more feasible.

The burden of pulmonary disease in children with CHD remains under-recognised. Studies have examined children with single ventricle and two ventricle heart disease and documented a decreased forced vital capacity. Our study sought to further explore the pulmonary function of children with CHD.

A retrospective review was performed of spirometry in CHD patients over a 3-year period. Spirometry data were corrected for size, age, and gender and analysed using z-scores.

The spirometry of 260 patients was analysed. About 31% had single ventricle (n = 80, 13.6 years (interquartile range 11.5-16.8)) and 69% had two ventricle circulation (n = 180, 14.4 years (interquartile range 12.0-17.3)). Single ventricle patients were found to have a lower median forced vital capacity z-score compared to two ventricle patients (p = 0.0133). The prevalence of an abnormal forced vital capacity was 41% in single ventricle patients and 29% in two ventricle patients. Two ventricle patients with tetralogy of Fallot and truncus arteriosus had similar low forced vital capacity comparable to single ventricle patients. The number of cardiac surgeries predicted an abnormal forced vital capacity in two ventricle patients except tetralogy of Fallot patients.

Pulmonary morbidity in patients with CHD is common with a decreased forced vital capacity noted in single ventricle and two ventricle patients. Forced vital capacity is lower in patients with single ventricle circulation; however, two ventricle patients with tetralogy of Fallot or truncus arteriosus have similar lung function in comparison to the single ventricle group. The number of surgical interventions was predictive of forced vital capacity z-score in some but not all two ventricle patients and not predictive in single ventricle patients suggesting a multifactorial to pulmonary disease in children with CHD.

To electrophysiologically determine the impact of moderate to severe chronic hypoxia (H) resulting from a wide array of CHD (HCHD) conditions on the integrity of brainstem function.

Applying brainstem auditory-evoked response methodology, 30 chronically afflicted HCHD patients, who already had undergone heart surgery, were compared to 28 healthy control children (1–15 yo) matched by age, gender and socioeconomic condition. Blood oxygen saturation was clinically determined and again immediately before brainstem auditory-evoked response testing.

Among HCHD children, auditory wave latencies (I, III and V) were significantly longer (medians: I, 2.02 ms; III, 4.12 ms, and; V, 6.30 ms) compared to control (medians: I, 1.67ms; III, 3.72 ms, and; V, 5.65 ms), as well as interpeak intervals (HCHD medians: I-V, 4.25 ms, and; III-V, 2.25ms; control medians: I-V, 3.90 ms and, III-V, 1.80 ms) without significant differences in wave amplitudes between groups. A statistically significant and inverse correlation between average blood oxygen saturation of each group (control, 94%; HCHD, 78%) and their respective wave latencies and interpeak intervals was found.

As determined by brainstem auditory-evoked responses, young HCHD patients manifestly show severely altered neuronal conductivity in the auditory pathway strongly correlated with their hypoxic condition. These observations are strongly supported by different brainstem neurological and image studies showing that alterations, either in microstructure or function, result from the condition of chronic hypoxia in CHD. The non-altered wave amplitudes are indicative of relatively well-preserved neuronal relay nuclei.

Feeding difficulty is common in infants with CHD.

The purpose of this study was to describe the feeding skills and behaviours of infants with CHD at 6–12 months of age and explore relationships between feeding, gastrointestinal distress, and gastroesophageal reflux.

Parents of 30 infants with CHD completed online surveys when their infant was 6, 8, 10, and 12 months old. Surveys included parent-report measures of feeding skills (Child Oral and Motor Proficiency Scale), feeding behaviours (Pediatric Eating Assessment Tool), symptoms of gastrointestinal distress (Infant Gastrointestinal Symptoms Questionnaire), and gastroesophageal reflux (Infant Gastroesophageal Reflux Questionnaire-Revised).

We found that 95, 32, 67, and 30% of infants were delayed in their feeding skill development at 6, 8, 10, and 12 months, respectively. Symptoms of problematic feeding behaviours were similarly high, with 90, 62, 29, and 38% of infants meeting criteria for problematic feeding at 6, 8, 10, and 12 months, respectively. Feeding skills and behaviours were related but unique contributors. Feeding behaviours were related to both gastrointestinal distress and gastroesophageal reflux, but feeding skills were rarely related to either.

Delayed acquisition of feeding skills and problematic feeding were common in infants with CHD. Infants with more gastrointestinal and gastroesophageal reflux symptoms had more symptoms of problematic feeding behaviours.

Comprehensive assessment, targeted interventions, and early involvement of feeding specialists are important to support feeding in infants with CHD through at least the first year of life.

Prolonged effusions post-Fontan procedure are associated with morbidity. Fontan patients have higher pro-inflammatory cytokines in chest tube drainage compared to controls. Colchicine, an anti-inflammatory medication, decreases effusions in adults after cardiac surgery. We hypothesised that patients post-Fontan treated with colchicine would have decreased pro-inflammatory cytokine levels and shorter duration of chest tube drainage.

This pilot clinical trial enrolled nine patients (intention to treat); five completed the protocol (per protocol). Post-operative Fontan patients 20 months to 5 years receiving colchicine were compared to a previously published control cohort (n = 25). Per protocol patients received 0.6 mg colchicine daily starting post-operative day 2, ending 1 day after chest tube removal. Chest tube samples were taken on days 1–4, 7 and 10, or until removal and analysed with a 17-cytokine Bio-Plex Assay. Descriptive statistics and basic univariate comparisons were made.

There was no difference in duration of chest tube drainage or length of stay between intention to treat patients and controls. Per protocol patients had shorter duration of chest tube drainage compared to controls (6 days [interquartile range 4.7–7], versus 10 days [7–11], p = 0.007) and shorter length of stay (7 days [5.5–9] versus 9 days [9–13], p = 0.005). Pro-inflammatory cytokines trended lower in per protocol patients.

In this pilot cohort, patients who completed the colchicine protocol post-Fontan procedure had shortened duration of chest tube drainage and length of stay. A decrease of pro-inflammatory cytokines may contribute to the mechanism of this change.

ClinicalTrials.gov: Colchicine in Postoperative Fontan Patients (CPFP); NCT03575572; https://clinicaltrials.gov/ct2/show/NCT03575572.

Children with right ventricle outflow tract anomalies require repeated heart surgeries, thereby needing regular preoperative assessments throughout their lifetime. This situation puts a heavy burden on these children. Thus, the aim of this study was to explore how children diagnosed with right ventricle outflow tract anomalies experience their heart disease and their everyday life during the preoperative assessment and after the decision on whether to perform a new cardiac surgery.

Individual interviews were conducted with nine children between 9 to 17 years of age on three occasions from 2014 to 2016. In total there were 27 interviews which all were analyzed with thematic analysis.

The analysis yielded three themes and eight subthemes. The theme Me and my heart disease concerns children’s experiences of the heart disease. Almost all described symptoms and how they adapt in their everyday life. The theme Being me concerns the children’s sense of self, where their heart disease was not prominent. The theme Being placed in someone else’s hands describes how the assessment was more of a safety net at least until the decision of heart surgery.

The children’s symptoms, their experiences during the assessment, their future surgeries and how the heart disease affects their everyday life could be better understood as elements of their adaptation to the heart disease. In order to achieve individualized support based on the child’s experiences and to ensure that these children are involved in their own care a child-centered approach is recommended.

To characterise the use of peripherally inserted central catheters in paediatric cardiac patients and to identify risk factors associated with their complications.

Observational retrospective cohort study in paediatric cardiac patients who underwent peripherally inserted central catheter placement in a tertiary children’s hospital from January 2000 to June 2018.

1822 cardiac patients underwent 2952 peripherally inserted central catheter placements in the study period. Median age was 29 days, with survival to hospital discharge of 96.4%. Successful placement achieved 94.5% of attempts, with a median line duration of 12 days. Factors associated with successful placement were the use of general anaesthesia (odds ratio 7.52, p < 0.001) and year of placement (odds ratio 1.08, p < 0.001). The incidence of complications was 28.6%, with thrombosis/occlusion being the most frequent (33%). Thrombosis/occlusion were associated with two and three lumens (odds ratio 1.96, p < 0.001 and 4.63, p = 0.037, respectively). Lines placed by interventional radiology had decreased infiltration (odds ratio 0.20, p = 0.002) and lower migration/malposition (odds ratio 0.36, p < 0.001). The use of maintenance intravenous fluids (odds ratio 3.98, p = 0.008) and peripheral tip position (odds ratio 3.82, p = 0.001) were associated with increased infiltration. The probability of infection decreased over time (odds ratio 0.79, p < 0.001).

Peripherally inserted central catheters in paediatric cardiac patients have complication rates similar to other paediatric populations. A prospective assessment of the factors associated with their complications in this patient population may be beneficial in improving outcomes.

Nutritional assessment appears to be an essential component of the evaluation of children with CHD undergoing surgery because nutritional status may impact corrective surgery-associated morbidity.

A prospective single-centre cohort study with children between 6 and 24 months of age. Patients who had genetic syndromes or those who were premature or low birthweight at birth were excluded. Pre-operative nutritional parameters included anthropometric measurements and serum concentrations of total protein, vitamin D, iron, and ferritin. Outcome measures included ICU length of stay, mechanical ventilation, vasoactive-inotropic score, and duration of inotropes. Linear regression analysis was performed to determine whether pre-operative variables were associated with outcomes.

Analysis was performed on 120 patients (median age of 8 months), of whom 67 were male. Prior to surgery, 50.8% of patients had reduced (z ≤ −2.0) weight-for-age z score, 23.3% had reduced length-for-age z score, and 59.2% had reduced mid-upper arm circumference z score. Pre-operative serum total protein levels were 59.36 ± 9.16 g/L. Multiple regression analysis showed that low serum protein was associated with longer ICU length of stay and length of mechanical ventilation, while mid-upper arm circumference z score ≤ −2 was associated with longer ICU length of stay and mechanical ventilation and inotropes duration.

Pre-operative assessment of nutritional status by performing anthropometric and biochemical measurements including mid-upper arm circumference z score and serum protein concentrations in children undergoing CHD surgery appears to be predictors of some post-operative short-term outcomes and could be used as a guide to highlight patients needing appropriate perioperative nutritional interventions.

To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries.

Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit.

Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00–08:00 and weekends) versus in-hours between 2015 and 2020.

The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy.

Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42–32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03–0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37–10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00–23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003).

Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

To investigate the opinion and personal experience of parents of children born with Hypoplastic Left Heart Syndrome and what advice they would give to other parents who have to decide between treatment options.

We conducted a qualitative, descriptive and retrospective study by means of a survey directed to parents of children born with Hypoplastic Left Heart Syndrome in a tertiary hospital in Buenos Aires (Argentina). Their answers and data regarding medical procedures were analysed.

Parents of thirteen out of sixteen patients with Hypoplastic Left Heart Syndrome were surveyed. Norwood surgery had been performed in all the patients, many had received other procedures, and five had died. In relation to the decision-making process, sixty-one percent of parents would recommend other parents to remain at peace after having done everything possible and 54% would suggest to not feel guilt despite the final result. None of the parents would recommend rejecting surgical treatment and choosing comfort care.

The majority of parents of children with Hypoplastic Left Heart Syndrome would recommend continuing with the therapeutic effort in order to feel at peace and reduce feelings of guilt.

We investigated frequency and risk factors of acute loss of the arterial pulse and permanent femoral arterial occlusion in neonates with CHD who underwent ultrasound-guided femoral arterial access.

We divided the patients into groups according to the presence of acute loss of the arterial pulse and permanent femoral arterial occlusion. We obtained data related to patient characteristics and access variables of ultrasound-guided femoral arterial access from our database of cardiac catheterisation between August, 2017 and May, 2021. We used an echocardiography-S6, 12-MHz linear probe, 21-gauge needle, 0.018"guidewire, and a 4F sheath for arterial access.

Ultrasound-guided femoral arterial access was obtained in 323 (98.8%) of the 327 neonates. We identified acute loss of the arterial pulse in 130 (40.2%) patients and permanent femoral arterial occlusion in 19 (5.9%) patients. Median weight was 3.05 (Interquartile range (IQR): 2.80–3.40) kg, first attempt success rate was 88.2%, and median access time was 46 sec (IQR: 23–94). Logistic regression analysis identified coarctation of the aorta (odds ratio: 2.46; 95% CI: 1.30–4.66; p = 0.006) as independent risk factor for acute loss of the arterial pulse, but did not identify any independent risk factors for permanent femoral arterial occlusion.

This study showed coarctation of the aorta as an independent risk factor for acute loss of the arterial pulse, but did not identify any independent factors for permanent femoral arterial occlusion in neonates with CHD. Although most cases of acute loss of the arterial pulse resolve in the early period, the frequency of permanent femoral arterial occlusion remains high despite effective treatment.