Identifying thrombus formation in Fontan circulation has been highly variable, with reports between 17 and 33%. Initially, thrombus detection was mainly done through echocardiograms. Delayed-enhancement cardiac MRI is emerging as a more effective imaging technique for thrombus identification. This study aims to determine the prevalence of occult cardiac thrombosis in patients undergoing clinically indicated cardiac MRI.

A retrospective chart review of children and adults in the Duke University Hospital Fontan registry who underwent delayed-enhancement cardiac MRI. Individuals were excluded if they never received a delayed-enhancement cardiac MRI or had insufficient data. Demographic characteristics, native heart anatomy, cardiac MRI measurements, and thromboembolic events were collected for all patients.

In total, 119 unique individuals met inclusion criteria with a total of 171 scans. The median age at Fontan procedure was 3 (interquartile range 1, 4) years. The majority of patients had dominant systemic right ventricle. Cardiac function was relatively unchanged from the first cardiac MRI to the third cardiac MRI. While 36.4% had a thrombotic event by history, only 0.5% (1 patient) had an intracardiac thrombus detected by delayed-enhancement cardiac MRI.

Despite previous echocardiographic reports of high prevalence of occult thrombosis in patients with Fontan circulation, we found very low prevalence using delayed-enhancement cardiac MRI. As more individuals are reaching adulthood after requiring early Fontan procedures in childhood, further work is needed to develop thrombus-screening protocols as a part of anticoagulation management.

Perioperative immunisation administration surrounding congenital heart surgery is controversial. Delayed immunisation administration results in children being at risk of vaccine-preventable illnesses and is associated with failure to complete immunisation schedules. Among children with CHD, many of whom are medically fragile, vaccine-preventable illnesses can be devastating. Limited research shows perioperative immunisation may be safe and effective.

We surveyed Pediatric Acute Care Cardiology Collaborative member centres and explored perioperative immunisation practices. We analysed responses using descriptive statistics.

Complete responses were submitted by 35/46 (76%) centres. Immunisations were deferred for any period prior to surgery by 23 (66%) centres and after surgery by 31 (89%) centres. Among those who deferred post-operative immunisation, 20 (65%) required deferral only for patients whose operations required cardiopulmonary bypass. Duration of deferral in the pre- and post-operative periods was variable. Many centres included exceptions to their policy for specific vaccine-preventable illnesses. Almost all (34, 97%) centres administer routine childhood immunisation to patients who remain admitted for prolonged periods.

Most centres defer routine childhood immunisation for some period before and after congenital heart surgery. Centre specific practices vary. Immunisation deferral confers risk to patients and may not be warranted in this population. Further research would be necessary to understand the immunologic impact of these practices.

Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge.

We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation.

Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9–18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively.

In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension.

Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006–2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott’s shunt), lung transplant, or death.

Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1–44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28–52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6–10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0–4.7] and right ventricular stroke work index was 915 [715–1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott’s shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each).

Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

Malignant vasovagal syncope in children seriously affects their physical and mental health. Our study aimed to explore the efficacy of catheter ablation in ganglionated plexus with malignant vasovagal syncope children.

Catheter ablation of ganglionated plexus was safe and effective in children with malignant vasovagal syncope and can be used as a treatment option for these children.

A total of 20 children diagnosed with malignant vasovagal syncope were enrolled in Beijing Children’s Hospital, affiliated with Capital Medical University. All underwent catheter ablation treatment of ganglionated plexus. Ganglionated plexuses of the left atrium were identified by high-frequency stimulation and/or anatomic landmarks being targeted by radiofrequency catheter ablation. The efficacy of the treatment was evaluated by comparing the remission rate of post-operative syncopal symptoms and the rate of negative head-up tilt results. Safety and adverse events were evaluated.

After follow-up for 2.5 (0.6–5) years, the syncope symptom scores were decreased significantly compared with before treatment [3 (2–4) versus 5 (3–8) scores, P < 0.01]. Eighty-five per cent (17/20) children no longer experienced syncope, whilst 80% (16/20) children showed negative head-up tilt test after treatment. No adverse effects such as cardiac arrhythmia occurred in the children.

Infants with congenital heart disease and increased pulmonary blood flow frequently suffer from feeding difficulties and growth failure. Providing expressed breast milk by spoon has been hypothesised to decrease energy expenditure in these infants as compared to breastfeeding. This study assessed the effect of supplemental feeding of expressed breast milk on weight gain in infants with unoperated congenital heart disease.

This was a prospective open-label randomised control trial. In total, 50 infants with post tricuspid left to right shunt were enrolled in the study. In the intervention group, apart from breastfeeding, a minimum predetermined volume of expressed breast milk was targeted to be given by spoon. 30-50 kcal/kg/day was given by expressed breast milk by spoon-feeding. In the control group, the infants were given at least 8 feeds per 24 hours by direct breastfeeding. Both groups were followed up for 1 month and assessed for weight gain.

Despite a high rate of protocol breach in both groups (30% overall), infants in the intervention group had better weight gain at one-month follow-up compared to those in the control group, 780 ± 300 versus 530 ± 250 gm (p = 0.01).

In infants with left to right shunts, supplemental feeding of expressed breast milk by spoon along with breastfeeding resulted in significantly higher average weight gain at 30 days compared to the control group who received breastfeeding alone. Future studies with larger sample sizes and longer follow-ups need to be done to confirm the findings of this study.

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.

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.