Infants who require open heart surgery are at increased risk for developmental delays including gross motor impairments which may have implications for later adaptive skills and cognitive performance. We sought to evaluate the feasibility and efficacy of a tummy time intervention to improve motor skill development in infants after cardiac surgery.

Infants <4 months of age who underwent cardiac surgery were randomly assigned to tummy time with or without outpatient reinforcement or standard of care prior to hospital discharge. The Alberta Infant Motor Scale (AIMS) was administered to each infant prior to and 3 months after discharge. Groups were compared, and the association between parent-reported tummy time at home and change in motor scores at follow-up was examined.

Parents of infants (n = 64) who had cardiac surgery at a median age of 5 days were randomly assigned to tummy time instruction (n = 20), tummy time + outpatient reinforcement (n = 21) or standard of care (n = 23). Forty-nine (77%) returned for follow-up. At follow-up, reported daily tummy time was not significantly different between groups (p = 0.17). Fifteen infants had <15 minutes of tummy time daily. Infants who received >15 minutes of tummy time daily had a significantly greater improvement in motor scores than infants with <15 minutes of tummy time daily (p = 0.01).

In infants following cardiac surgery, <15 minutes of tummy time daily is associated with increased motor skill impairment. Further research is needed to elucidate the best strategies to optimise parental compliance with tummy time recommendations.

Systemic venous hypertension and low cardiac output are believed to be important mediators of liver injury after the Fontan procedure. Pulmonary vasodilators have the potential to improve such haemodynamics. The aim of this study was to assess the acute effects of exercise on liver stiffness and venous pressures and to assess the impact of inhaled Treprostinil on this response.

In this prospective, double-blind, placebo-controlled, crossover trial, 14 patients with a Fontan circulation were randomised to inhalation of placebo and Treprostinil. Incremental and constant work rate exercise tests were performed to assess the effect of Treprostinil on exercise tolerance. Venous pressures were measured throughout and liver stiffness at rest and immediately after peak exercise.

Mean age was 27.8 ± 7.9 years and 66% were females. Exercise acutely increased liver stiffness by 30% (mean shear wave speed: 2.38 ± 0.71 versus 2.89 ± 0.51 ms, p = 0.02). Peripheral venous pressures increased acutely during both incremental (12.1 ± 2.4 versus 22.6 ± 8.0 mmHg, p < 0.001) and constant work rate exercise (12.5 ± 2.5 versus 23.4 ± 5.2 mmHg, p < 0.001). Overall, Treprostinil failed to attenuate exercise-induced increases in liver stiffness. Compared with placebo, Treprostinil did not significantly impact venous pressure responses, VO2peak, nor exercise endurance times.

Peripheral venous pressure increased acutely during exercise by an average of 88% above baseline and was not altered by administration of inhaled Treprostinil. Liver stiffness measured immediately post-exercise increased acutely by an average of 30%, with no attenuation following Treprostinil inhalation.

The purpose of this study was to evaluate the mid-term outcome of aortic valve replacement for bicuspid aortic valve and tricuspid aortic valve and the related risk factors.

From January 2014 to June 2019, 177 tricuspid aortic valve patients and 101 bicuspid aortic valve patients who underwent aortic valve replacement in our hospital were collected. 1:1 propensity score matching analysis was used to control the bias in patient selection. The perioperative and follow-up data between the two groups were compared. Independent risk factors which were associated with the continued dilatation of the ascending aorta were identified by univariate or multivariate logistic regression analysis.

After the matching procedure, 160 patients were included in the analysis (80 in each group). Baseline characteristics, intraoperative, and perioperative outcomes were similar between the two groups (all p > 0.05). Moreover, 67 patients in the tricuspid aortic valve group and 70 in the bicuspid aortic valve group completed the follow-up. The ascending aorta change, annual change rate, and the proportion of continuous dilation of ascending aorta in bicuspid aortic valve group were significantly higher than those in the tricuspid aortic valve group (p < 0.05). Multivariate logistic regression analysis showed that type 1 in bicuspid aortic valve (OR 5.173; 95% CI 1.772, 15.101; p = 0.003), aortic regurgitation (OR 3.673; 95% CI 1.133, 11.908; p = 0.030), and aortic valve stenosis with regurgitation (OR 6.489; 95% CI 1.726, 24.404; p = 0.006) were independent risk factors for the continued dilatation of the ascending aorta in all AV patients. Furthermore, the multivariate logistic regression analysis showed that type 1 in bicuspid aortic valve (OR 5.157; 95% CI 1.053, 25.272; p = 0.043), age ≥ 40 years (OR 6.956; 95% CI 1.228, 39.410; p = 0.028), and aortic regurgitation (OR 4.322; 95% CI 1.174, 15.911; p = 0.028) were independent risk factors for the continued dilatation of the ascending aorta in bicuspid aortic valve patients.

Compared with tricuspid aortic valve patients, the ascending aorta of bicuspid aortic valve patients is more likely to continue to enlarge after aortic valve replacement. Type 1 in bicuspid aortic valve, age ≥ 40 years, and aortic regurgitation were the independent risk factors.

Rhabdomyoma is the most common cardiac tumour in children. It is usually associated with tuberous sclerosis complex caused by mutations in TSC-1 or TSC-2 genes. This tumour typically regresses by unknown mechanisms; however, it may cause inflow or outflow obstruction that necessitates urgent surgery. Here we investigate the clinical features and the genetic analysis of patients with tuberous sclerosis complex presenting with large rhabdomyoma tumours. We also investigate the potential role of autophagy and apoptosis in the pathogenesis of this tumour.

All the patients with cardiac rhabdomyoma referred to Aswan Heart Centre from 2010 to 2018 were included in this study. Sanger sequencing was performed for coding exons and the flanking intronic regions of TSC1 and TSC2 genes. Histopathological evaluation, immunohistochemistry, and western blotting were performed with P62, LC3b, caspase3, and caspase7, to evaluate autophagic and apoptotic signaling.

Five patients were included and had the clinical features of tuberous sclerosis complex. Three patients, who were having obstructive tumours, were found to have pathogenic mutations in TSC-2. The expression of two autophagic markers, P62 and LC3b, and two apoptotic markers, caspase3 and caspase7, were increased in the tumour cells compared to normal surrounding myocardial tissue.

All the patients with rhabdomyoma were diagnosed to have tuberous sclerosis complex. The patients who had pathogenic mutations in the TSC-2 gene had a severe disease form necessitating urgent intervention. We also demonstrate the potential role of autophagy and apoptosis as a possible mechanism for tumourigenesis and regression. Future studies will help in designing personalised treatment for cardiac rhabdomyoma.

Phrenic nerve injury is a common complication of cardiac and thoracic surgical procedures, with potentially severe effects on the health of a child. This review aims to summarise the available literature on the diagnosis and management of PNI post-cardiac surgery in paediatric patients with CHD.

The presence of injury post-surgery can be difficult to detect and may present with non-specific symptoms, emphasising the importance of an effective diagnostic strategy. Chest X-ray is usually the first investigation for a suspected diagnosis of PNI, which is usually confirmed using fluoroscopy, ultrasound scan, or phrenic nerve stimulation (gold standard). Management options include supportive ventilation and/or invasive diaphragmatic plication surgery. While the optimal timing of plication surgery remains controversial, it is now the most widely accepted treatment for PNI in children post-CHD surgery, especially for very young patients who cannot be weaned off supportive ventilation. Further research is needed to determine the optimal timing of surgical intervention for positive outcomes and to explore the benefits of using minimally invasive surgical techniques in children.

PNI is a common and serious complication of CHD surgery, therefore, its diagnosis and management in the paediatric population are of major importance. Further research is needed to determine the optimal timing of surgical intervention for positive outcomes and to explore the benefits of using minimally invasive surgical techniques in children.

To evaluate the discriminative ability of hyperlactataemia for early morbidity and mortality in neonates with CHD following cardiac surgery.

Retrospective, observational study of neonates who underwent cardiac surgery on cardiopulmonary bypass at a tertiary care children’s hospital from June 2015 to June 2019. The primary predictor was lactate. The primary composite outcome was defined as ≥1 of the following: cardiac arrest or extracorporeal membrane oxygenation within 72 hours or 30-day mortality post-operatively. The secondary outcome was the presence of major residual lesions, according to the Technical Performance Score.

Of 432 neonates, 28 (6.5%) sustained the composite outcome. On univariate analysis, peak lactate within 48 hours, increase in lactate from ICU admission through 12 hours, and single ventricle physiology were significantly associated with the composite outcome. The peak lactate occurred at a median of 2.9 hours (interquartile range: 1, 35) before the event. Through multi-variable analysis, a multi-variable risk algorithm was created. Predicted probabilities demonstrated an increasing risk based on single ventricle status and delta lactate, ranging from 1.8% (95% CI: 0.9, 3.9) to 52.4% (95% CI: 32.4, 71.7). The model had good discriminative ability for the composite outcome on receiver operating characteristic analysis (area under the curve = 0.79; 95% CI: 0.75, 0.89). Moreover, a peak lactate of 7.3 mmol/l or greater was significantly associated with the presence of a major residual lesion (odds ratios: 5.16, 95% CI: 3.01, 8.87).

We present a simple, two-variable model, including delta lactate in the immediate post-operative period and single ventricle status, to prognosticate the risk of early morbidity and mortality in neonates undergoing cardiac surgery for potential intervention.

Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery.

Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time.

Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up.

Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

During tetralogy of Fallot repair, leaving or even create an interatrial communication may facilitate post-operative course particularly with right ventricle restrictive physiology. The aim of our study is to assess the influence of atrial communication on post-operative course of tetralogy of Fallot repair.

Retrospectively, we studied all children who had tetralogy of Fallot repair (2003–2018). We divided them into two groups: tetralogy of Fallot repair with interatrial communication (TOFASD) group and tetralogy of Fallot repair with intact atrial septum (TOFIAS) group. We performed propensity match score for specific pre- or intra-operative variables and compared groups for post-operative outcome variables. Secondarily, we looked for right ventricle restrictive physiology incidence and influence of early repair performed before 3 months of age on post-operative course.

One hundred and sixty children underwent tetralogy of Fallot repair including (93) cases of TOFIAS (58%) and (67) cases of TOFASD (42%). With propensity matching score, 52 patients from each group were compared. Post-operative course was indifferent in term of positive pressure ventilation time, vasoactive inotropic score, creatinine and lactic acid levels, duration and amount of chest drainage and length of intensive care unit and hospital stay. Right ventricle restrictive physiology occurred in 38% of patients with no effects on outcome. 12/104 patients (12%) with early repair needed longer pressure ventilation time (p = 0.003) and intensive care unit stay (p = 0.02).

Leaving interatrial communication in tetralogy of Fallot repair did not affect post-operative course. As well, right ventricle restrictive physiology did not affect post-operative course. Infants undergoing early tetralogy of Fallot repair may require longer duration of positive pressure ventilation time and intensive care unit stay.

We aimed to compare the results of two surgical methods for the treatment of congenital supravalvular aortic stenosis.

From May 2004 to January 2020, 29 patients underwent surgical repair for supravalvular aportic stenosis in a single centre. The perioperative evaluation of the patients was retrospectively reviewed.

Fifteen (51.7%) and 14 (48.2%) patients were treated with the Doty and the McGoon methods, respectively. The median age of our cohort was 4.5 (3.0–9.9) years. Ten (34.5%) patients had Williams–Beuren syndrome, and pulmonary stenosis was observed in 12 (41.3%) patients. The median follow-up time was 2.5 (0.7–7.3) years. On follow-up, five patients had residual stenosis with the McGoon technique and one with the Doty technique (p = 0.05). One patient died early in the post-operative period in the Doty group, and three patients were re-operated on due to restenosis in the McGoon group. Freedom from re-operation in the Doty group at 1, 3, 5, and 10 years was 100%. In the McGoon group, freedom from re-operation rates at the 1-, 3-, and 7-year follow-up were 100, 88.9, and 44.4%, respectively (p = 0.08).

Our results with both surgical techniques suggest that supravalvular aortic stenosis can be treated with good results. The Doty method provided better relief for the supravalvular aortic segment, considering the residual stenosis and the re-operation rates.

After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation.

Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days–6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8–154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months.

The median duration of ICU stay after tracheostomy was 27 days (range 2–93 days). Follow-up time in ward was median 30 days (2–156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week–11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients.

HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.