Manuscripts pertaining to paediatric cardiology and CHD have been published in a variety of different journals. Some of these journals are journals dedicated to paediatric cardiology, while others are focused on adult cardiology. Historically, it has been considered that manuscripts published in journals devoted to adult cardiology have greater citation potential. Our objective was to compare citation performance between manuscripts related to paediatric cardiology and CHD published in paediatric as opposed to adult cardiology journals.

We identified manuscripts related to paediatric cardiology and CHD published in five journals of interest during 2014. Of these journals, two were primarily concerned with adult cardiology, while the other three focused on paediatric cardiology. The number of citations for these identified manuscripts was gathered from Google Scholar. We compared the number of citations (median, mean, and 25th, 75th, 90th, and 95th percentiles), the potential for citation, and the h-index for the identified manuscripts.

We identified a total of 828 manuscripts related to paediatric cardiology and congenital heart as published in the 5 journals during 2014. Of these, 783 (95%) were published in journals focused on paediatric cardiology, and the remaining 45 (5%) were published in journals focused on adult cardiology. The median number of citations was 41 in the manuscripts published in the journals focused on adult cardiology, as opposed to 7 in journals focused on paediatric cardiology (p < 0.001). The h-index, however, was greater for the journals dedicated to paediatric cardiology (36 versus 27).

Approximately one-twentieth of the work relating to paediatric cardiology and CHD is published in journals that focus predominantly on adult cardiology. The median number of citations is greater when manuscripts concerning paediatric cardiology and CHD are published in these journals focused on adult cardiology. The h-index, however, is higher when the manuscripts are published in journals dedicated to paediatric cardiology. While such publications in journals that focus on adult cardiology tend to generate a greater number of citations than those achieved for works published in specialised paediatric cardiology journals, the potential for citation is no different between the journals. Due to the drastically lower number of manuscripts published in journals dedicated to adult cardiology, however, median performance is different.

Systemic-to-pulmonary shunts are used as a source of pulmonary blood flow in palliated Congenital Heart Disease in neonates and young infants. Shunt thrombosis, often requiring shunt interventions during index hospitalisation, is associated with poor outcomes. We hypothesised that extensive use of perioperative pro-coagulant products may be associated with shunt thrombosis.

Children (≤18 years) undergoing systemic-to-pulmonary shunts with in-hospital shunt reinterventions between 2016 and 2020 were reviewed retrospectively. Perioperative associations to shunt thrombosis were examined by univariate logistic regression and Wilcoxon rank sum tests as appropriate. Cox and log transformed linear regression were used to analyse postoperative ventilation duration, length of stay, and cost.

Of 71 patients requiring in-hospital shunt intervention after systemic-to-pulmonary shunts, 10 (14%) had acute shunt thrombosis, and among them five (50%) died. The median age was four (interquartile range: 0-15) months. There were 40 (56%) males, 41 (58%) had single ventricle anatomy, and 29 (40%) were on preoperative anticoagulants. Patients with acute shunt thrombosis received greater volume of platelets (p = 0.04), cryoprecipitate (p = 0.02), and plasma (p = 0.04) postoperatively in the ICU; experienced more complications (p = 0.01) including re-exploration for bleeding (p = 0.008) and death (p = 0.02), had longer hospital length of stays (p = 0.004), greater frequency of other arterial/venous thrombosis (p = 0.02), and greater hospital costs (p = 0.002).

Patients who develop acute shunt thrombosis receive more blood products perioperatively and experience worse hospital outcomes and higher hospital costs. Future research on prevention/early detection of shunt thrombosis is needed to improve outcomes in infants after systemic-to-pulmonary shunt surgery.

The aim of this study is to report on the short-term and mid-term outcomes of preterm infants who underwent patent ductus arteriosus ligation through anterior mini-thoracotomy.

Data for 103 preterm infants who underwent patent ductus arteriosus clipping through an anterior mini-thoracotomy at the 2nd intercostal space between 2009 and 2019 were retrospectively reviewed. The patients were divided into two groups according to their weight at the time of surgery. The complications, morbidity, and mortality rates of each group were compared at postoperative day 30 and at the end of 1 year after surgery.

During the operation, the median weight of the patients was 900 g (IQR 800–1125 g), the median age was 21 days (IQR 14.5–29 days). The lowest body weight was 460 g. In three patients (3%), there was intraoperative bleeding from the patent ductus arteriosus that required transition to median sternotomy. In one patient (1%) a residual patent ductus arteriosus that required reoperation was observed. Twelve patients (12%) died in the first 30 days postoperatively. Six patients (6%) died between the postoperative day 30 and 1 year. There was no statistically significant difference in the rates of mortality, morbidity, and complication between the groups.

Based on our observations of over a hundred preterm infants with patent ductus arteriosus over a decade, ligation through anterior mini-thoracotomy is the main surgical procedure of choice for this patient group in our clinic. Our findings demonstrate the safety of this approach and we believe that it can be successfully replicated in other institutions.

Paediatric residents are often taught cardiac anatomy with two-dimensional images of heart specimens, or via imaging such as echocardiography or computed tomography. This study aimed to determine if the use of a structured, interactive, teaching session using heart specimens with CHD would be effective in teaching the concepts of cardiac anatomy.

The interest amongst paediatric residents of a cardiac anatomy session using heart specimens was assessed initially by circulating a survey. Next, four major cardiac lesions were identified to be of interest: atrial septal defect, ventricular septal defect, tetralogy of Fallot, and transposition. A list of key structures and anatomic concepts for these lesions was developed, and appropriate specimens demonstrating these features were identified by a cardiac morphologist. A structured, interactive, teaching session was then held with the paediatric residents using the cardiac specimens. The same 10-question assessment was administered at the beginning and end of the session.

The initial survey demonstrated that all the paediatric residents had an interest in a cardiac anatomy teaching session. A total of 24 participated in the 2-hour session. The median pre-test score was 45%, compared to a median post-test score of 90% (p < 0.01). All paediatric residents who completed a post-session survey indicated that the session was a good use of educational time and contributed to increasing their knowledge base. They expressed great interest in future sessions.

A 2-hour hands-on cardiac anatomy teaching session using cardiac specimens can successfully highlight key anatomic concepts for paediatric residents.

Although the right jugular vein approach for percutaneous pulmonary valve implantation is well described, there are no reports that describe a percutaneous pulmonary valve implantation through a left superior caval vein to coronary sinus pathway.

A 14-year-old female with tetralogy of Fallot, mesocardia, left superior caval vein draining into the coronary sinus, and hemiazygos continuation of the inferior caval vein underwent ventricular septal defect closure, with homograft insertion from the right ventricle to the pulmonary artery, patch augmentation of the left pulmonary artery, and creation of an atrial communication. Thereafter followed numerous catheterisations and interventions with stent implantation for stenosis of the left pulmonary artery and the homograft, as did device closure of the atrial communication. When she was a 12-year-old, the indications for a percutaneous pulmonary valve implantation were fulfilled and she underwent implantation of a 22 mm Melody® valve through the left superior caval vein. The extra-stiff exchange wire was pre-formed into a “U-spiral”-type configuration, according to the underlying anatomy, in order to provide a smooth route for the delivery of stents, to create the landing zone, and for the implantation of the Melody “ensemble”. The procedure was performed under deep sedation according to our standard protocol. The duration of the procedure was 172 min and the radiation time was 24.9 min.

On the basis of this unique experience, percutaneous pulmonary valve implantation is safe and feasible even in patients with unusual anatomy. Crucial is the “U-spiral” shaped configuration of the guide wire.

The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery.

This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days).

The study cohort had a median age of 25.6 years (18–60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay.

Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.

Recent changes in surgical education have had an impact on our congenital training programmes. The mandate of the 8-hour workweek, a rapidly expanding knowledge base, and a host of other mandates has had an impact on the readiness of the fellows who are entering congenital programmes. To understand these issues completely, we interviewed the top congenital experts in the United States of America. The purpose of this paper is to share their insight and offer suggestions to address these challenges.

We used a qualitative thematic analysis approach and performed phone interviews with the top five congenital experts in the United States of America.

Experts unanimously felt that duty-hour restrictions have negatively affected congenital training programmes in the following ways: current fellows do not seem as conditioned as fellows in the past, patient handoffs are not consistent with excellent performance, the mentor–mentee relationship has been affected by duty-hour restrictions, and fellows may be less prepared for real-world practice. Three positive themes emerged in response to duty-hour restrictions: fellows appear to be doing less menial task work, fellows are now better rested for learning, and we are attracting more individuals into the speciality. Experts agreed that congenital fellowships should be increased to 2 years. There was support for both the traditional and integrated residency pathways.

We are in a new era of education and must work together to overcome the challenges that have arisen in recent years.

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.