Cor triatriatum is the rarest of all congenital cardiac diseases, accounting for 0.1–0.4% of congenital heart diseases. Atrial septal defect is the most common associated defect; however, cor triatriatum is sometimes associated with univentricular heart.

This single-centre retrospective study involved all patients who underwent the repair of cor triatriatum at Kobe Children’s Hospital between 2000 and 2020. Twenty-four patients were required surgery. We conducted a survey of survival rate, early and late pulmonary vein stenosis in each group.

The survival rate of 5 years after cor triatriatum resection was 100% in the biventricular group and 82.1% in the univentricular group, respectively. The free rate for pulmonary stenosis of 5 years after surgery was 100% in the biventricular group and 90.0% in the univentricular group, respectively. There was no statistical difference in survival rate and 5 years free rate for pulmonary stenosis after surgery.

The results showed that surgical correction offers good early and mid-term outcomes for both cor triatriatum with biventricular and univentricular physiologies.

Milrinone is a phosphodiesterase type 3 inhibitor that results in a positive inotropic effect in the heart through an increase in cyclic adenosine monophosphate. The purpose of this study was to evaluate circulating cyclic adenosine monophosphate and milrinone concentrations in milrinone treated paediatric patients undergoing congenital heart surgery.

Single-centre prospective observational pilot study from January 2015 to December 2017 including children aged birth to 18 years. Milrinone and circulating cyclic adenosine monophosphate concentrations were measured at four time points through the first post-operative day and compared between patients with and without low cardiac output syndrome, defined using clinical and laboratory criteria.

Fifty patients were included. Nine (18%) developed low cardiac output syndrome. For all patients, 22% had single ventricle heart disease. The density and distribution of cyclic adenosine monophosphate concentrations varied between those with and without low cardiac output syndrome but were not significantly different. Milrinone concentrations increased in all patients. Paired t-tests demonstrated an increase in circulating cyclic adenosine monophosphate concentrations during the post-operative period among patients without low cardiac output syndrome.

In this prospective observational study, circulating cyclic adenosine monophosphate concentrations increased in those without low cardiac output syndrome during the first 24 post-operative hours and milrinone concentrations increased in all patients. Further study of the utility of cyclic adenosine monophosphate concentrations in milrinone treated patients is necessary.

The relationship between different surrogates of insulin resistance and left ventricular geometry in obese children is still unclear.

We sought to explore the relationship between commonly used measures of insulin sensitivity/resistance (homeostatic model assessment index, serum uric acid, and triglycerides to high-density lipoprotein cholesterol ratio) and left ventricular geometry in normotensive obese children.

In this cross-sectional study, 32 normotensive obese children were examined. Transthoracic echocardiography was used to measure left ventricular mass index and relative wall thickness. Homeostasis model assessment index, serum uric acid level, and a ratio of triglycerides to high-density lipoprotein cholesterol were used as markers of the insulin resistance. Simple and partial correlation analyses (to control for the effects of body mass index) were conducted to explore relationship between studied variables and left ventricular mass index or relative wall thickness as outcome variables.

We found positive correlations between homeostasis model assessment index and relative wall thickness (r = 0.47, p = 0.03) which remained significant after controlling for the effect of body mass index, z-score (r = 0.48, p = 0.03). The cutoff level of homeostasis model assessment index with the optimum sensitivity (Sn) and specificity (Sp) derived from the receiver operating characteristic (ROC) curves for predicting concentric remodelling was ≥5.51 with Sn = 83.33 and Sp = 68.75.

There is a positive relationship between homeostasis model assessment index and relative wall thickness of obese normotensive children which may help to distinguish at risk obese normotensive children for the development of concentric left ventricular remodelling.

Although rare, coronary artery anomalies can have significant clinical implications. Total anomalous origin of the coronary arteries from the pulmonary artery (TCAPA) represents a rare subtype of coronary artery anomaly for which little is known. The aim of this review was to characterise the presentation, utilised diagnostic modalities, associated cardiac lesions, and treatment strategies in patients with TCAPA.

A systematic review was performed for cases of TCAPA using PubMed, Embase, and Web of Science. Keywords searched included “total anomalous origin of the coronary arteries from the pulmonary artery,” “single ostium anomalous coronary artery from the pulmonary artery,” and “anomalous origin of both coronary arteries from the pulmonary artery.”

Fifty-seven cases of TCAPA were identified in 50 manuscripts. Fifty-eight per cent of patients were male and the median age at presentation was 10 days (mean 1.71 ± 6.6 years, range 0 days–39 years). Most patients were symptomatic at the time of presentation; cyanosis (n = 22) and respiratory distress (n = 14) were the most common symptoms. Cases were most commonly diagnosed at autopsy (n = 26, 45.6%), but operative intervention was pursued in 22 cases (45.6%); aortic re-implantation (n = 14) and a Takeuchi-type repair (n = 7) were the most common routes of repair.

The clinical presentation of patients with TCAPA was found to be variable, likely related to the presence of associated cardiac lesions. TCAPA should be considered in patients with suspected anomalous origin of the left coronary artery from the pulmonary artery for the serious consequences that can occur if not promptly corrected.

Post-operative fever (POF) after cardiac surgery in a developing country is of great concern because of the associated morbidity and mortality. In our country, we experience this complication more because of a high rate of malaria infestations, gastroenteritis, and malnutrition. We also experience a low yield of positive diagnostic tests when POF develops; this is partly due to expense and the prioritisation of other essential items in our poorly equipped cardiac ICU.

To assess the causes of POF after cardiac surgery and determine its impact on the outcome on patients.

International Cardiac Surgery Mission teams visited our centre for 7 years (2013–2019). During this period, a significant number of cardiac surgeries were performed. A retrospective study of patients with POF was performed with data from our hospital’s database, and included standard demographics; types of surgery performed, and management protocols being used in the peri-operative care.

A total of 242 patients were treated during the study period and underwent 266 surgeries. Of these, 151 (56.8%) and 115 (43.2%) were adult and paediatric cases respectively. Ages ranged between 0 and 90 years; 34 (14.1%) had POF. When we evaluated the clinical and associated laboratory findings, pyrexia (temp >38.0 °C) and elevated white blood cell count with differential neutrophilia were present in 10 patients (29.4%). The time of onset and duration of POF were between <24 and >144 hours. In the invasive procedure related to POF, sternotomy infections were present in seven patients (18.4%). Malaria infestations and breach in sterility protocol were predominant.

The management of POF in the cardiac ICU was complicated by the requirement of co-operation from a larger number of specialties than is usually required in advanced centres with special expertise in malaria confirmation. We, therefore, introduced structured clinical, laboratory, and appropriate interventions to treat POF more aggressively. We found that more careful attention to peri-operative details to ensure strict observation of sterility protocol with anti-malaria prophylaxis led to improvements in our centre’s outcomes.

Increased risk donors in paediatric heart transplantation have characteristics that may increase the risk of infectious disease transmission despite negative serologic testing. However, the risk of disease transmission is low, and refusing an IRD offer may increase waitlist mortality. We sought to determine the risks of declining an initial IRD organ offer.

We performed a retrospective analysis of candidates waitlisted for isolated PHT using 20072017 United Network of Organ Sharing datasets. Match runs identified candidates receiving IRD offers. Competing risks analysis was used to determine mortality risk for those that declined an initial IRD offer with stratified Cox regression to estimate the survival benefit associated with accepting initial IRD offers. Overall, 238/1067 (22.3%) initial IRD offers were accepted. Candidates accepting an IRD offer were younger (7.2 versus 9.8 years, p < 0.001), more often female (50 versus 41%, p = 0.021), more often listed status 1A (75.6 versus 61.9%, p < 0.001), and less likely to require mechanical bridge to PHT (16% versus 23%, p = 0.036). At 1- and 5-year follow-up, cumulative mortality was significantly lower for candidates who accepted compared to those that declined (6% versus 13% 1-year mortality and 15% versus 25% 5-year mortality, p = 0.0033). Decline of an IRD offer was associated with an adjusted hazard ratio for mortality of 1.87 (95% CI 1.24, 2.81, p < 0.003).

IRD organ acceptance is associated with a substantial survival benefit. Increasing acceptance of IRD organs may provide a targetable opportunity to decrease waitlist mortality in PHT.

Paediatric cardiac surgery on cardiopulmonary bypass induces substantial physiologic changes that contribute to post-operative morbidity and mortality. Fluid overload and oedema are prevalent complications, routinely treated with diuretics. The optimal diuretic choice, timing of initiation, dose, and interval remain largely unknown.

To guide clinical practice and future studies, we used PubMed and EMBASE to systematically review the existing literature of clinical trials involving diuretics following cardiac surgery from 2000 to 2020 in children aged 0–18 years. Studies were assessed by two reviewers to ensure that they met eligibility criteria.

We identified nine studies of 430 children across four medication classes. Five studies were retrospective, and four were prospective, two of which included randomisation. All were single centre. There were five primary endpoints – urine output, acute kidney injury, fluid balance, change in serum bicarbonate level, and required dose of diuretic. Included studies showed early post-operative diuretic resistance, suggesting higher initial doses. Two studies of ethacrynic acid showed increased urine output and lower diuretic requirement compared to furosemide. Children receiving peritoneal dialysis were less likely to develop fluid overload than those receiving furosemide. Chlorothiazide, acetazolamide, and tolvaptan demonstrated potential benefit as adjuncts to traditional diuretic regimens.

Early diuretic resistance is seen in children following cardiopulmonary bypass. Ethacrynic acid appears superior to furosemide. Adjunct diuretic therapies may provide additional benefit. Study populations were heterogeneous and endpoints varied. Standardised, validated endpoints and pragmatic trial designs may allow investigators to determine the optimal diuretic, timing of initiation, dose, and interval to improve post-operative outcomes.

Technologically, advances in both transcatheter and surgical techniques have been continuing in the past 20 years, but an updated comprehensive comparison in device-based versus surgery in adults in terms of incidence of in-hospital mortality, perioperative stroke, and atrial fibrillation onset is still lacking. We investigate the performance of transcatheter device-based closure compared to surgical techniques by a systematic review and meta-analysis of the last 20 years literature data.

The analysis was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Literature search was performed based on Cochrane Library, Embase, PubMed, and Google Scholar to locate articles published between January 2000 and October 2020, regarding the comparison between short-term outcome and post-procedural complications of atrial septal defect patients receiving transcatheter or surgical closure. The primary outcome was the comparison of in-hospital mortality from all causes between patients treated with transcatheter closure or cardiac. Secondary outcomes were the onset of post-procedural atrial fibrillation or perioperative stroke.

A total of 2360 patients were included of which 1393 [mean age 47.6 years, 952 females (68.3%)] and 967 [mean age 40.3 years, 693 females (71.6%)] received a transcatheter device-based and surgery closure, respectively. In-hospital mortality [OR 0.16 (95% CI (0.66−0.44)), p = 0.0003, I2 = 0%], perioperative stroke [OR 0.51 (95% CI (0.31−0.84)), p = 0.003, I2 = 79%], and post-procedural atrial fibrillation [OR 0.14 (95% CI (0.03−0.61)), p = 0.009, I2 = 0%] significantly favoured transcatheter device-based closure

Transcatheter atrial septal defect closure resulted safer in terms of in-hospital mortality, perioperative stroke, and post-procedural atrial fibrillation compared to traditional surgery.

We sought to describe patient characteristics associated with prolonged post-operative length of stay in a contemporary cohort of infants who underwent isolated repair of aortic coarctation.

We reviewed patients less than 1 year of age who underwent isolated repair of aortic coarctation at our institution from 2009 to 2016. Prolonged post-operative length of stay was defined as length of stay within the upper tertile for the cohort. Bivariate and multi-variable analyses were performed to determine independent risk factors for prolonged length of stay.

We reviewed 95 consecutive patients who underwent isolated repair of aortic coarctation, of whom 71 were neonates at the time of diagnosis. The median post-operative length of stay was 6.5 days. The upper tertile for post-operative length of stay was greater than 10 days; 32 patients within this tertile and 1 patient who died at 8.5 days after surgery were analysed as having prolonged post-operative length of stay. In a multi-variable analysis, pre-maturity (odds ratio: 3.5, 95% confidence interval: 1.2, 10.7), genetic anomalies (odds ratio: 4.7, 95% confidence interval: 1.2, 18), absence of pre-operative oral feeding (odds ratio: 7.4, 95% confidence interval: 2.4, 22.3), and 12-hour vasoactive-ventilation-renal score greater than 25 (odds ratio: 7.4, 95% confidence interval: 1.9, 29) were independently associated with prolonged length of stay.

In neonates and infants who underwent isolated repair of aortic coarctation, pre-maturity, genetic anomalies, lack of pre-operative oral feedings, and 12-hour vasoactive-ventilation-renal score more than 25 were independent risk factors for prolonged post-operative length of stay. Further study on the relationship between pre-operative oral feedings and post-operative length of stay should be pursued.

Children with CHD carry an additional burden of pulmonary insufficiency, often necessitating prolonged ventilatory support, especially in the peri-operative phase. There has been an increase in the utilisation of non-invasive ventilatory support for these children. The objective of this study was to evaluate the utilisation, safety, and outcomes of RAM cannula as a tool for escalation and de-escalation of respiratory support in paediatric cardiac patients less than one year of age.

A single-centre retrospective cohort study of patients supported with RAM cannula.

A total of 275 instances of RAM use were included in the study, 81.1% being post-operative. Patients were stratified into escalation and de-escalation cohorts based on the indication of non-invasive ventilation. The success rate of using RAM cannula was 69.5% overall, 66.1% in the escalation group, and 72.8% in the de-escalation group. At baseline, age at cardiac ICU admission >30 days, FiO2 ≤ 40%, PaCO2 ≤ 50 mmHg; and after 12 hours of non-invasive ventilation support respiratory rate ≤ 60/min, PaO2 ≥ 50 mmHg, PaCO2 ≤ 50 mmHg; and absence of worsening on follow-up chest X-ray predicted the success with a sensitivity of 95% in the logistic regression model. Successful support was associated with a significantly shorter unit stay.

RAM cannula can be safely used to provide non-invasive support to infants in the cardiac ICU for escalation and de-escalation of respiratory support. Factors associated with success can be used to make decisions about candidacy and appropriate timing of non-invasive ventilation use to maximise effectiveness.