Tetralogy of Fallot patients face an elevated risk of developing chylothorax and pleural effusions post-surgery. This patient group exhibits risk factors known to compromise the lymphatic system, such as elevated central venous pressure, pulmonary flow changes, and hypoxia. This study investigates the morphology and function of the lymphatic system in tetralogy of Fallot patients through lymphatic magnetic resonance imaging and near-infrared fluorescence imaging, respectively.

Post-repair tetralogy of Fallot patients aged 6–18 years were recruited, along with age and gender-matched controls. Magnetic resonance imaging was used to assess the morphology of the thoracic lymphatic vessels and the thoracic, while near-infrared fluorescence imaging was used to assess lymphatic activity utilising lymph rate, velocity, and pressure.

Nine patients and 10 controls were included. Echocardiography revealed that 2/3 of the patients had moderate-severe pulmonary regurgitation, while none displayed signs of elevated central venous pressure. Magnetic resonance imaging identified three patients with type 3 (out of 4 types) lymphatic abnormalities, while controls had none. The thoracic ducts showed severe (one patient) and moderate (one patient) tortuosity. Mean thoracic duct diameters were 3.3 mm ±1.1 in patients and 3.0 mm ± 0.8 in controls (p-value = 0.53). Near-infrared fluorescence imaging revealed no anomalous patterns.

Despite no presence of clinical lymphatic disease, 3/9 of the repaired tetralogy of Fallot patients exhibited lymphatic morphological abnormalities. The significance of these anomalies remains uncertain currently. Further research is needed to determine whether these lymphatic alterations in this patient cohort are a result of congenital malformations, haemodynamic shifts, or prenatal and early-life saturation levels.

Mobile health has been shown to improve quality, access, and efficiency of health care in select populations. We sought to evaluate the benefits of mobile health monitoring using the KidsHeart app in an infant CHD population.

We reviewed data submitted to KidsHeart from parents of infants discharged following intervention for high-risk CHD lesions including subjects status post stage 1 single ventricle palliation, ductal stent or surgical shunt, pulmonary artery band, or right ventricular outflow tract stent. We report on the benefits of a novel mobile health red flag scoring system, mobile health growth/feed tracking, and longitudinal neurodevelopmental outcomes tracking.

A total of 69 CHD subjects (63% male, 41% non-white, median age 28 days [interquartile range 20, 75 days]) were included with median mobile health follow-up of 137 days (56, 190). During the analytic window, subjects submitted 5700 mobile health red flag notifications including 245 violations (mean [standard deviation] 3 ± 3.96 per participant) with 80% (55/69) of subjects submitting at least one violation. Violations precipitated 116 interventions including hospital admission in 34 (29%) with trans-catheter evaluation in 15 (13%) of those. Growth data (n = 2543 daily weights) were submitted by 63/69 (91%) subjects and precipitated 31 feed changes in 23 participants. Sixty-eight percent of subjects with age >2 months submitted at least one complete neurodevelopment questionnaire.

In our initial experience, mobile health monitoring using the KidsHeart app enhanced interstage monitoring permitting earlier intervention, allowed for remote tracking of growth feeding, and provided a means for tracking longitudinal neurodevelopmental outcomes.

Given increased survival for adults with CHD, we aim to determine outcome differences of infective endocarditis compared to patients with structurally normal hearts in the general population.

We conducted a retrospective cross-sectional study identifying infective endocarditis hospitalisations in patients 18 years and older from the National Inpatient Sample database between 2001 and 2016 using International Classification of Disease diagnosis and procedure codes. Weighting was used to create national annual estimates indexed to the United States population, and multivariable logistic regression analysis determined variable associations. Outcome variables were mortality and surgery. The primary predictor variable was the presence or absence of CHD.

We identified 1,096,858 estimated infective endocarditis hospitalisations, of which 17,729 (1.6%) were adults with CHD. A 125% increase in infective endocarditis hospitalisations occurred for adult CHD patients during the studied time period (p < 0.001). Adults with CHD were significantly less likely to experience mortality (5.4% vs. 9.5%, OR 0.54, CI 0.47–0.63, p < 0.001) and more likely to undergo in-hospital surgery (31.6% vs. 6.7%, OR 6.49, CI 6.03–6.98, p < 0.001) compared to the general population. CHD severity was not associated with increased mortality (p = 0.53). Microbiologic aetiology of infective endocarditis varied between groups (p < 0.001) with Streptococcus identified more commonly in adults with CHD compared to patients with structurally normal hearts (36.2% vs. 14.4%).

Adults with CHD hospitalised for infective endocarditis are less likely to experience mortality and more likely to undergo surgery than the general population.

Neurodevelopmental delay is common in children who undergo surgery for Congenital Heart Disease (CHD) in infancy. Cardiac surgery associated acute kidney injury (CS-AKI) occurs frequently in the paediatric cardiac Intensive care unit (ICU). Cardiac surgery associated acute kidney injury (CS-AKI) is associated with worse neurodevelopmental scores and delay in cognitive, language, and motor domains in children with CHD. No known data exist regarding the association of CS-AKI and motor and language subscales. In this study, we explored the relationship between CS-AKI and receptive and expressive language, as well as gross and fine motor delay.

This was a single centre retrospective observational cohort study. Children who underwent surgery for CHD and developed recurrent CS-AKI in the first year of life who had follow-up neurodevelopmental testing using the Bayley Scale of Infant Development Version III were included. Neurodevelopmental delay subscales assessed included: receptive and expressive language, fine and motor skills.

The study cohort included 203 children. Recurrent CS-AKI was significantly associated with lower scores in receptive and expressive language, as well as fine and gross motor on unadjusted analyses. On adjusted analyses, recurrent CS-AKI was significantly associated with severe receptive language delay.

The independent association of recurrent CS-AKI with severe language delay in children who undergo surgery for CHD in infancy is novel. Our findings may contribute to the understanding of language impairment in this population. Further studies are required to better understand this relationship and any potentially modifiable factors.

Modified Blalock-Taussig shunt (BT shunt) is a palliative operation used for cyanotic heart diseases with decreased pulmonary blood supply. The definitive management of tetralogy of Fallot (TOF) is total corrective surgery, but these patients can be palliated with BT shunt. In the modern world, the BT shunt is getting out of favour in patients with TOF. In this article, we will share our 5-year experience at our institute, which also shows a decreasing trend.

It is a retrospective study. Files of all the patients admitted in our department from January 2019 to December 2023 were reviewed. Age, weight, hospital stay, inotropic support duration, mechanical ventilation duration, and outcomes were studied.

From January 2019 to December 2023, 173 patients underwent BT shunt for TOF. The mean age was 31 months, and the mean weight was 9.3 kg. The overall mortality for BT shunt was 15% after BT shunt. Hypercyanotic spell not controlled by medical management was the most common indication for BT shunt in our setup. Most of the patients with hypercyanotic spells were also candidates for total correction but due to the emergency, BT shunt was performed.

The role of BT shunt in patients with TOF is decreasing due to PDA/RVOT stenting, it is likely that the BT shunt in TOF will become a thing of the past in the future even in developing countries like ours.

When young patients with an oval fossa defect present early with symptoms of heart failure and pulmonary hypertension, surgical treatment is recommended in small bodyweight rather than transcatheter closure.

Outcomes of device closure in consecutive symptomatic children weighing under 10 kg were compared with young children aged below 4 years but weighing above 10 kg. Transthoracic echocardiography under conscious sedation guided the procedure in all without need for balloon sizing, transesophageal echocardiogram, and intubation anaesthesia. Symptoms, anthropometry, shunt ratio, pulmonary pressures, defect and device size, percentage oversizing, device/body weight ratio, complications, and post-procedural growth spurt were compared.

Ninety-six patients weighing under 10 kg were compared with 160 patients weighing above 10 kg. In total, 83.3% of patients in the study group and 25% of controls were severely malnourished. The median indexed defect size was 35.2 mm/sq.m and 27.4 mm/sq.m, and the device was oversized by 8.7% and 14.2% in the study group and controls, respectively. The device/body weight ratio was 1.93 in study group and 1.4 in controls. Procedure was successful in all except one patient weighing under 10 kg who had a device embolisation. Both groups showed significant growth spurts and proportion, with severe malnutrition reduced to 42% and 11% in the two groups.

Device closure was feasible and safe in patients under 10 kg. Transthoracic echocardiographic imaging on conscious sedation provided adequate guidance. Symptoms and growth significantly improved after intervention. Despite a larger defect size, smaller patients had comparable outcomes. In symptomatic children under 10 kg needing early closure, transcatheter intervention should not be deferred.

An aberrant right subclavian artery represents the most common aortic arch vascular anomaly. Conventional wisdom states that these anomalies do not result in dysphagia, but rather serve as “red herrings”. Clearly, in the vast majority of cases, this holds true. Nonetheless, one should never say never.

Herein, we present a cohort of four children with debilitating dysphagia resulting from an aberrant right subclavian artery. Subclavian reimplantation via a right posterolateral thoracotomy was performed successfully in all cases.

Dysphagia resolved postoperatively, and all patients were able to advance to a normal diet. They were able to gain appropriate weight postoperatively and continue to do well at most recent clinical follow-up.

This case series suggests that aberrant right subclavian artery anatomy should be considered a potential aetiology of dysphagia, albeit rarely. Surgical intervention for select patients can provide dramatic resolution of symptoms.

Bicuspid aortic valve is the most common CHD and commonly associated with activity restrictions that may lead to a sedentary lifestyle known to increase obesity risk. It is unknown whether obesity is associated with changes in aortic dimensions or aortic valve function in young people with bicuspid aortic valve. This study investigates whether overweight and obese children with bicuspid aortic valve have worse aortic valve function or increased aortic dimensions compared to healthy weight children with bicuspid aortic valve.

This was a single centre retrospective cohort study comprised of patients 5 to 25 years old with a diagnosis of bicuspid aortic valve between 1 January, 2019 and 31 December, 2020. Patients were classified as healthy weight or overweight/obese. Values for aortic dimensions as well as peak and mean aortic valve gradients were obtained from echocardiogram reports.

About 251 patients were analysed. Demographics were similar between groups. When indexed to height, the aortic valve annulus (1.28 ± 0.14 vs. 1.34 ± 0.15, p = 0.001) and sinotubular junctions (1.44 ± 0.21 vs. 1.49 ± 0.24, p = 0.038) were larger in the overweight/obese group, with no differences in aortic root or ascending aorta sizes. The obese/overweight group had a higher peak aortic valve gradient (23.03 ± 1.64 mmHg vs. 16.17 ± 1.55 mmHg, p = 0.003) compared to the healthy weight group.

Healthy weight patients did not have larger aortic dimensions compared to the overweight/obese patients. There was evidence of worsening aortic valve stenosis in overweight/obese patients compared to those at a healthy weight.

Society of Thoracic Surgeons Congenital Heart Surgery Database is the largest congenital heart surgery database worldwide but does not provide information beyond primary episode of care. Linkage to hospital electronic health records would capture complications and comorbidities along with long-term outcomes for patients with CHD surgeries. The current study explores linkage success between Society of Thoracic Surgeons Congenital Heart Surgery Database and electronic health record data in North Carolina and Georgia.

The Society of Thoracic Surgeons Congenital Heart Surgery Database was linked to hospital electronic health records from four North Carolina congenital heart surgery using indirect identifiers like date of birth, sex, admission, and discharge dates, from 2008 to 2013. Indirect linkage was performed at the admissions level and compared to two other linkages using a “direct identifier,” medical record number: (1) linkage between Society of Thoracic Surgeons Congenital Heart Surgery Database and electronic health records from a subset of patients from one North Carolina institution and (2) linkage between Society of Thoracic Surgeons data from two Georgia facilities and Georgia’s CHD repository, which also uses direct identifiers for linkage.

Indirect identifiers successfully linked 79% (3692/4685) of Society of Thoracic Surgeons Congenital Heart Surgery Database admissions across four North Carolina hospitals. Direct linkage techniques successfully matched Society of Thoracic Surgeons Congenital Heart Surgery Database to 90.2% of electronic health records from the North Carolina subsample. Linkage between Society of Thoracic Surgeons and Georgia’s CHD repository was 99.5% (7,544/7,585).

Linkage methodology was successfully demonstrated between surgical data and hospital-based electronic health records in North Carolina and Georgia, uniting granular procedural details with clinical, developmental, and economic data. Indirect identifiers linked most patients, consistent with similar linkages in adult populations. Future directions include applying these linkage techniques with other data sources and exploring long-term outcomes in linked populations.

Accurate measurement of transcutaneous oxygen saturation is important for the assessment of cyanosis in CHD. Aim of this study was the evaluation of a supplementary transcutaneous oxygen saturation measurement with an Apple watch® in children with cyanotic heart disease.

During a six-minute walk test, measurement of transcutaneous oxygen saturation was performed simultaneously with an Oximeter (Nellcor, Medtronic, USA) and an Apple watch® Series 7 (Apple inc, USA) in 36 children with cyanotic heart disease.

Median age was 9.2 (IQR 5.7–13.8) years. Transcutaneous oxygen saturation measurement with the Apple watch® was possible in 35/36 and 34/36 subjects before and after six-minute walk test. Children, in whom Apple watch® measurement was not possible, had a transcutaneous oxygen saturation < 85% on oximeter. Before six-minute walk test, median transcutaneous oxygen saturation was 93 (IQR 91–97) % measured by oximeter and 95 (IQR 93–96) % by the Apple watch®. After a median walking distance of 437 (IQR 360–487) m, transcutaneous oxygen saturation dropped to 92 (IQR 88–95, p < 0.001) % by oximeter and to 94 (IQR 90–96, p = 0.013) % measured with the Apple watch®.

In children with mild cyanosis measurement of transcutaneous oxygen saturation with an Apple watch® showed only valid results if transcutaneous oxygen saturation was > 85%, with higher values being measured with the smart watch. In children with moderate or severe cyanosis transcutaneous oxygen saturation, measurement with the Apple watch® was not reliable and cannot be recommended to monitor oxygen saturation at home.

Myocardial infarction is rare in children but frequently occurs unexpectedly with atypical presentation. It can cause a progressive lethal course unless prompt treatment is initiated.

Paediatric cases of myocardial infarction diagnosed by the presence of ischaemic myocardial insults and symptomatic ventricular dysfunction were reviewed retrospectively.

Eighteen patients (5 days to 14 years of age; median 3 months) with myocardial infarction were studied. The aetiology was variable, including congenital coronary anomalies: anomalous left coronary artery from pulmonary artery (five patients), pulmonary atresia with intact ventricular septum with right ventricle-dependent coronary circulation (four), anomalous aortic origin of left coronary artery (three), and Williams syndrome with supravalvar aortic stenosis (one). Two acquired coronary anomalies occurred in one patient with undiagnosed Kawasaki disease with complete thrombotic obstruction of the left coronary artery and another with post-transplant cardiac allograft microangiopathy. Three patients developed thromboembolic coronary artery incidents in normal coronary anatomy. Fourteen patients were less than 1 year of age (78%). Electrocardiographic abnormalities were noted in all patients. Four patients required extracorporeal membrane oxygenation support for severe ventricular dysfunction, none of whom survived. Five patients underwent heart transplant. Five patients died during the same hospitalisation, and one patient died after discharge (overall mortality 33%). Transplant-free survival was 39%.

Most myocardial infarction occurred in infants who presented with abrupt onset of non-specific clinical manifestations with progressively deteriorating haemodynamic status resulting in poor transplant-free survival rate. Early diagnosis and treatment are essential to prevent catastrophic outcomes.

Gastrointestinal bleeding is a potential complication in paediatric patients undergoing cardiopulmonary bypass, as it develops secondary to low gastrointestinal perfusion. This study aimed to examine the incidence of gastrointestinal bleeding and identify its risk factors in these patients.

This retrospective study was undertaken to examine the demographic features, clinical findings, and operative data of paediatric patients under years old who had undergone congenital heart surgery with cardiopulmonary bypass between November 1, 2021, and November 1, 2023. The study aimed to investigate the incidence of gastrointestinal bleeding associated with cardiopulmonary bypass and to identify potential risk factors for gastrointestinal bleeding. The obtained results were statistically evaluated.

The study period included 1100 patients who underwent congenital heart surgery with cardiopulmonary bypass. Fifty-two percent of the total participants were male. The median weight of the patients was 4.4 kg, with an interquartile range of 3.5–5.8 kg. The patients were categorised by age, revealing that 62% were newborns, 24% were infants, and 14% were children. Forty-four (4.2%) of the total number of patients experienced gastrointestinal bleeding. Newborns had a significantly higher incidence of bleeding (6% or 34 patients) compared to infants (3% or 8 patients) and children (1.5% or 2 patients) (p < 0.05). Patients who experienced gastrointestinal bleeding had a longer median hospital stay of 24 days compared to those who did not, with a median hospital stay of 14 days. Moreover, patients who suffered from bleeding had a significantly higher mortality rate (30%) in comparison to those who did not (9.9%) (p < 0.05). The incidence of gastrointestinal bleeding was found to be associated with several risk factors, such as low operative age and weight, high surgical score, presence of low cardiac output syndrome, extracorporeal membrane oxygenation (ECMO) usage, high lactate levels, and low platelet count.

Gastrointestinal bleeding is a potential complication for patients who undergo cardiopulmonary bypass. It is particularly relevant for newborns who have undergone prolonged surgery, have a high surgical complexity score, exhibit high lactate levels, display low cardiac output, utilise ECMO, and possess low platelet counts. In such cases, there may be a heightened incidence of gastrointestinal bleeding. It is important to consider this possibility in order to ensure the best possible patient outcomes.

Congenital heart patients undergoing congenital heart surgery in the first year of life are at high risk of having a neurodevelopmental disorder. The most common difficulties are related to executive functioning. The following questions were assessed in the current project: Are patients having congenital heart surgery after one year of life at lower risk for neurodevelopmental disorders? At what age do executive function deficits manifest?

We evaluated executive function in four groups of congenital heart patients who had undergone congenital heart surgery. These groups were high-risk patients with and without a genetic syndrome associated with a neurodevelopmental disorder and low-risk patients with and without a genetic syndrome associated with a neurodevelopmental disorder. We evaluated executive function using the Behavior Rating Inventory of Executive Function – Preschool Version, Behavior Rating Inventory of Executive Function-2, and Minnesota Executive Function Scale at various ages. We compared the rates of executive function deficits in the high- and low-risk groups as well as compared that to the published norms for age. We also assessed at what age these deficits become apparent.

We found that both high- and low-risk groups had higher levels of executive functioning deficits compared to the norms for age. The low-risk group’s degree of executive function deficits appeared a little lower than the high-risk group. However, it was difficult to comment on the statistical significance. We also saw that executive function deficits often do not become apparent for many years after surgery. This finding highlights the need for continued evaluation of functioning as these kids mature.

It is unclear how extracorporeal membrane oxygenation use varies across paediatric cardiac surgical programmes and how it relates to post-operative mortality. We aimed to determine hospital-level variation in post-operative extracorporeal membrane oxygenation use and its association with case-mix adjusted mortality.

Retrospective analysis of 37 hospitals contributing to the Pediatric Cardiac Critical Care Consortium clinical registry from 1 August 2014 to 31 December 2019. Hospitalisations including cardiothoracic surgery and post-operative admission to paediatric cardiac ICUs were included. Two-level multivariable logistic regression with hospital random effect was used to determine case-mix adjusted post-operative extracorporeal membrane oxygenation use rates and in-hospital mortality. Hospitals were grouped into extracorporeal membrane oxygenation use tertiles, and mortality was compared across tertiles.

There were 43,640 eligible surgical hospitalisations; 1397 (3.2%) included at least one post-operative extracorporeal membrane oxygenation run. Case-mix adjusted extracorporeal membrane oxygenation rates varied more than sevenfold (0.9–6.9%) across hospitals, and adjusted mortality varied 10-fold (0–5.5%). Extracorporeal membrane oxygenation rates were 2.0%, 3.5%, and 5.2%, respectively, for low, middle, and high extracorporeal membrane oxygenation use tertiles (P < 0.0001), and mortality rates were 1.9%, 3.0%, and 3.1% (p < 0.0001), respectively. High extracorporeal membrane oxygenation use hospitals were more likely to initiate extracorporeal membrane oxygenation support intraoperatively (1.6% vs. 0.6% low and 1.1% middle, p < 0.0001). Extracorporeal membrane oxygenation indications were similar across hospital tertiles. When extracorporeal cardiopulmonary resuscitation was excluded, variation in extracorporeal membrane oxygenation use rates persisted (1.5%, 2.6%, 3.8%, p < 0.001).

There is hospital variation in adjusted post-operative extracorporeal membrane oxygenation use after paediatric cardiac surgery and a significant association with adjusted post-operative mortality. These findings suggest that post-operative extracorporeal membrane oxygenation use could be a complementary quality metric to mortality to assess performance of cardiac surgical programmes.

Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients.

This study was conducted retrospectively in term newborns and infants younger than six months of age who underwent congenital cardiac surgery operation from 1 November 2022 to 1 November 2023 and were followed in the paediatric cardiac ICU. Patients who were receiving mechanical ventilator support at least 12 hours postoperatively were evaluated for the development of postoperative atelectasis and reintubation in the first 3 days of extubation. The patients were grouped as HFNC and non-HFNC users. Demographic characteristics, surgery type, and ICU clinical follow-up data were obtained from medical records. The results were statistically evaluated.

A total of 40 patients who did not use HFNC in the early postoperative period and 40 patients with HFNC in the late period during the study period were included in the study. The median age was 1 month (IQR 15 days–2 months) with equal gender distribution. Among patients, 70% of them were in the neonatal age group. Reintubation rates in the first 72 hours in HFNC users and non-HFNC users were 2.5% and 12.5%, respectively (p < 0.05). The median postoperative atelectasis scores at 24, 48, and 72 hours of extubation were 2 versus 2.5 (p > 0.05), 1.5 versus 3.5 (p < 0.05), and 1 versus 3 (p < 0.05) in HFNC users and non-HFNC users, respectively.

HFNC therapy may have a positive effect on preventing atelectasis and reducing the reintubation rate in the early postoperative period.