Cardiac catheterization during extracorporeal membrane oxygenation (ECMO) in pediatric patients with congenital heart disease is increasingly used to evaluate residual lesions and perform transcatheter interventions. However, evidence from Latin American populations remains scarce. We conducted a retrospective, single-center cohort study including patients under 18 years who underwent cardiac catheterization while on ECMO support between January 2012 and December 2024. Demographic and procedural variables were collected. Outcomes included successful ECMO decannulation, procedure-related complications, and survival to hospital discharge. Among 140 pediatric patients supported with ECMO, 36% (n = 51) underwent cardiac catheterization, totaling 59 procedures. Most patients (92%) had complex congenital heart disease, with a predominance of single-ventricle physiology. In 64% of procedures, one or more transcatheter interventions were performed, most frequently balloon angioplasty or stenting of the pulmonary arteries. The median time from ECMO cannulation to catheterization was 1.25 days; 67% of procedures were performed within the first 24 h. Overall survival to hospital discharge was 43%. Major complications were infrequent (3%), and no procedure-related mortality was observed. Cardiac catheterization during ECMO was feasible and safe in this cohort. Most procedures were performed early and included transcatheter interventions. Although multivariable analysis did not identify independent predictors of survival, catheterization provided essential diagnostic and therapeutic information that influenced management. These findings support its role in selected pediatric patients supported with ECMO.

This study aimed to evaluate the characteristics, difficulties, and outcomes of patients who underwent transcatheter ablation treatment due to arrhythmia with a diagnosis of CHD. A total of 166 patients (189 substrates) with CHD who underwent catheter ablation between November 2013 and 2023 were evaluated retrospectively. EnSite™ (St Jude Medical Inc., St Paul, MN, USA) was used in all patients. The mean age was 14.8 ± 7.9 years (2.9-43 years). The most common CHD's were Ebstein anomaly (n: 40), tetralogy of Fallot (n: 31), atrial septal defect (n: 25), ventricular septal defect (n: 22), great artery transposition (D/L TGA, n: 12), and complex CHD in single ventricle physiology (n: 9). The most common arrhythmia mechanisms were Wolf-Parkinson-White syndrome (WPW, n: 50), intraatrial reentrant tachycardia (IART, n: 39), typical atrioventricular nodal reentrant tachycardia (AVNRT, n: 37), and ventricular tachycardia-ventricular extrasystoles (VT/VES, n: 23). There was more than one arrhythmia in 23 patients and multiple manifest accessory pathways in 10 patients. The average procedure time was 174 ± 69.3 minutes, and the average fluoro time was 8.3 minutes. While successful ablation was performed in 176/189 (acute success 93.1%) substrates, the procedure was unsuccessful in five patients and suboptimal in eight patients. Recurrence was observed in 11 patients (6.4%) during a mean follow-up period of 49.2 ± 30.1 months. A second ablation was performed on 13 patients. Acute success was achieved in all except one patient. A total of 11 patients are being followed up with medical treatment. Despite the complex anatomy, age, operations, and limited vascular access possibilities in patients diagnosed with CHD, transcatheter ablation treatment with advances in electrophysiology, the introduction of different energy types, special ablation catheters, multipolar mapping catheters, and 3D nonfluoroscopic mapping systems seems to be a safe and effective option.

This study evaluates the short- and long-term outcomes of patients with single-ventricle morphology with arrhythmias prior to Fontan surgery, assessing the association between early arrhythmias and subsequent clinical outcomes. Staged surgical palliation for single-ventricle physiology involves complex operations with a high incidence of arrhythmias. The optimal duration of antiarrhythmic medication use and its impact on surgical outcomes and long-term prognosis remain unclear. A single-center retrospective analysis was conducted on patients treated between August 2011 and January 2024. Patients with single-ventricle physiology undergoing staged surgical palliation with documented arrhythmias were selected for detailed analysis. Data on patient characteristics, arrhythmia type, antiarrhythmic medication use, and outcomes were collected and analyzed. The study included 109 patients, predominantly male, with hypoplastic left heart syndrome being the most common morphology. Arrhythmias were primarily encountered during Stage 1 admission (97%), with no initial arrhythmia occurrence during interstage times. Pre-Fontan electrophysiology studies showed inducible tachycardia in 33% of patients. The need for ablation was seen in conjunction with heterotaxy syndrome and patients with longer duration and complexity of tachycardia prior to Fontan. Overall survival to Fontan was poor, with significant mortality at each stage of palliation. Subsequent post-Fontan arrhythmia was rare and there was no recurrence of patients undergoing ablation. Early arrhythmias are common in single-ventricle patients undergoing staged palliation and this cohort is associated with significant morbidity and mortality. Pre-Fontan electrophysiology studies and ablation may improve outcomes, particularly in high-risk cohorts, highlighting the need for tailored arrhythmia monitoring and management strategies. This study evaluates the outcomes of patients with single-ventricle physiology and arrhythmias occurring before Fontan surgery, examining the relationship between early arrhythmias and clinical outcomes. Conducted as a single-center retrospective analysis from August 2011 to January 2024, it includes 109 patients with hypoplastic left heart syndrome. Arrhythmias were common during Stage 1 admission (97%). Pre-Fontan electrophysiology studies showed inducible tachycardia in 33% of patients, with ablation needed in those with heterotaxy syndrome and complex tachycardia. Survival to Fontan was poor, with significant mortality at each stage. Tailored arrhythmia monitoring and management strategies are essential.

Single-ventricle physiology is a critical cardiac condition requiring early diagnosis and intervention. The objectives of this study were to report on the management and outcomes of patients diagnosed with single-ventricle physiology in central South Africa. This study was a retrospective, observational analysis of patients presenting with single-ventricle physiology at the Universitas Academic Hospital in central South Africa between November 1997 and June 2021. Patients were referred from the Free State (54%) and Northern Cape (29%) provinces and Lesotho. One hundred and fifty-four patients presented with single-ventricle physiology: 114 received interventions and 40 were not eligible for intervention. Patients presented for the first time at a median age of 34.5 days, with patients from nearby districts presenting within a few days of birth. However, patients from outlying areas presented much later. Eighty-seven patients received systemic-to-pulmonary artery shunting or pulmonary artery banding. Sixty-three patients proceeded to bidirectional Glenn procedures, and 30 patients (26%) had full palliation to Fontan. Twenty-one patients died after stage 1, six after the Glenn procedure and two after the Fontan procedure. Overall, 34 (29.8%) patients were lost to follow up. Patients in our study presented late and follow up of these patients was a challenge. The highest mortality rate occurs during the first stage of palliation. Outcomes from this study are comparable to other sub-Saharan studies.

Galectin-3 (Gal-3) is a biomarker associated with myocardial fibrosis, a key factor in the dysfunction of the functionally single ventricle (FSV) in patients after the Fontan procedure. This study aimed to evaluate the relationship between serum Gal-3 levels and echocardiographic and cardiopulmonary exercise parameters in this population. Thirty-seven patients (23 males, 14 females) with Fontan circulation were included. All underwent speckle-tracking echocardiography (STE), cardiopulmonary exercise testing (CPET), and serum Gal-3 measurement using ELISA. Correlations between Gal-3 and clinical, echocardiographic, and CPET parameters were analyzed. Gal-3 levels correlated positively with patient age and time since Fontan completion (p < 0.05). No significant associations were found between Gal-3 and ejection fraction, global longitudinal strain (GLS), or free wall strain. However, Gal-3 showed a significant correlation with the transmural strain gradient (p < 0.05). No association was observed between Gal-3 and CPET parameters, including peak VO2. Galectin-3 may reflect fibrotic remodeling of the FSV, as suggested by its correlation with the transmural strain gradient. The absence of a relationship with exercise capacity highlights the complexity of Fontan-related dysfunction. Gal-3 shows promise as a noninvasive biomarker of myocardial fibrosis in this unique patient group.

We describe 2 successful cases of children with single ventricle physiology (SVP) who underwent dental rehabilitation under general anaesthesia, a scenario that carries significant challenges. Both patients received intranasal dexmedetomidine as premedication, which provided effective anxiolysis, facilitated intravenous access, and contributed to perioperative haemodynamic stability. One patient was maintained on sevoflurane, while the other received total intravenous anaesthesia with propofol and remifentanil. In both cases, deep extubation was performed safely, aided by dexmedetomidine’s sedative and sympatholytic properties and careful titration of anaesthetic depth. These cases highlight important anaesthetic considerations in SVP, including the role of dexmedetomidine as premedication, careful titration of anaesthetic agents to achieve haemodynamic goals, particularly in reducing pulmonary vascular resistance, the potential to omit muscle relaxants, goal-directed fluid therapy, and the importance of smooth extubation.

In pediatric cardiac critical care, patients often require specific anesthesia and sedation considerations due to unique physiological vulnerabilities. This study assessed the incidence of propofol-related infusion syndrome (PRIS) in Pediatric Cardiac Intensive Care Unit (PCICU) patients receiving continuous propofol infusion for non-procedural sedation. We conducted a retrospective review of post-operative congenital heart disease patients < 18 years admitted to the PCICU from 1/1/2000-9/30/2024, who received continuous propofol infusions ≥ 12hours and/or ≥ 2.4mg/kg/hour. The primary outcome was the incidence of PRIS. Secondary outcomes included intensive care unit (ICU) length of stay and survival to hospital discharge. Statistical analyses included chi-square tests and the Kruskal-Wallis test, stratified by physiological complexity (single vs. double ventricle) and surgical mortality risk scores (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery [STAT] score). A total of 641 patients were included. No statistically significant differences in propofol characteristics were found across physiologic or surgical risk groups. The median infusion rate was 1.2mg/kg/hour, with a median duration of 22.7hours. No cases of PRIS occurred. Survival to hospital discharge was 97%. The median ICU length of stay was 7.1 days. In this single center, retrospective study of post-operative congenital heart disease patients, continuous propofol infusion was not associated with any cases of PRIS. These findings support the cautious use of propofol in pediatric cardiac critical care. Further prospective studies are needed to evaluate safety across the heterogenous congenital heart disease population, including those with single ventricle physiology.

Associations between cardiorespiratory fitness, activity behavior, physical literacy and parental sports participation in children with a critical congenital heart disease.

IV calcium is used frequently in the pediatric cardiovascular ICU (CVICU) for neonates and infants undergoing congenital heart surgery (CHS). Since critical illness is associated with abnormal cellular calcium handling and adverse effects induced by hypercalcemia, we aimed to: describe calcium use across three CVICUs; determine explanatory factors related to hypercalcemia and calcium administration; and evaluate associations with outcome. Retrospective cohort analysis from January 2020 to December 2022. Three university affiliated CVICUs. Children younger than 6 months undergoing CHS receiving postoperative monitoring of ionized calcium (iCa). None. Average iCa for every 24-hour period was calculated from tests drawn during the first 72 hours after CHS. Of 276 infants evaluated, 119 (43%) were neonates, 62 (23%) underwent The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category 4 and 5 surgery, and 44 (16%) had single ventricle physiology. Median (interquartile range) age was 50 days (8-113 d). IV calcium utilization differed between centers, with calcium administered in 21%, 62%, and 96% of cohorts ( p < 0.001) at respective hospitals. Hypercalcemia was more prevalent in neonates ( p = 0.02), patients with longer cardiopulmonary bypass times ( p = 0.02), and patients with higher postoperative Vasoactive-Inotrope Scores ( p = 0.001). Children receiving top 10% of total calcium administration (compared with those receiving some calcium and no calcium) were younger ( p < 0.001), experienced higher rates of cardiac arrest ( p = 0.02), longer CVICU length of stay (LOS; p < 0.001), and lower survival rates ( p < 0.001). In multivariable analyses, we failed to identify associations between hypercalcemia and receiving top 10% calcium administration with LOS or mechanical ventilation duration. In 2020-2022, post-CHS calcium management in neonates/infants varied across our three CVICUs. Increased calcium administration and hypercalcemia occurred in high-risk populations (e.g., neonates, STAT ≥ 4 category). Future experimental designs are needed to better understand these relationships and optimize CVICU postoperative calcium management.

Association Between Congenital Heart Disease Complexity, Mental Health Conditions and Opioid Use Disorder.

Defining diastolic dysfunction post-Fontan: Threshold, risk factors, and associations with outcomes.

Computational fluid dynamics-based risk stratification of modified Blalock-Taussig-Thomas shunt thrombogenicity.

Insights from Ventricular Leadless Pacemaker Implantation in Two Critically Ill Patients with Unpalliated Single Ventricle Physiology: A Case Series

ObjectivesMechanical valve replacement is often used as the therapeutic option in valvular heart surgery in children. Evidence suggests that this should change to provide optimal long-term survival for this growing population. We reviewed our current practice in valve repair in congenital heart disease and analysed its outcomes.MethodsA total of 90 patients (30 semilunar valve and 60 atrioventricular [AV] valve) underwent valve repair between September 2020 and December 2024. Operative data and follow-up information were gathered retrospectively. Kaplan-Meier calculations were used for survival and freedom from reoperation analysis. Cox regression analysis was used to assess risk factors for mortality (single ventricle physiology, age and weight at time of surgery, bicuspid aortic valve, and complexity of repair). Complexity of repair was defined as the application of 3 or more repair techniques.ResultsEstimated survival at 12 months for semilunar and AV valve groups was 95.2% and 95%, respectively. Estimated freedom from reoperation at 12 months for repair of semilunar and AV valves was 95.8% and 95.6%, respectively. Single ventricle morphology (HR [hazard ratio], 5.2; 95% confidence interval [CI], 1.3-20.8; P = .0198) and younger age at time of surgery (HR, 0.7; 95% CI, 0.6-0.9; P = .0223) were associated with increased risk of mortality.ConclusionsValve repair in congenital heart disease provides reliable early outcomes in this complex population. The worst outcomes are expected in patients with single ventricle requiring surgery in early life.

This case describes a 32-year-old adult male who was incidentally diagnosed with isolated dextrocardia, Double inlet left ventricle (l-looped), l-transposed great arteries with subpulmonary stenosis during evaluation for an orbital abscess. This case highlights protective factors that enabled this patient's survival into adulthood without cardiac surgeries or medications despite single ventricle physiology, namely his "self-banded" pulmonary flow.

IntroductionUnplanned readmissions to the pediatric intensive care unit (PICU) following cardiac surgery remain poorly understood. Such readmissions are associated with increased morbidity and present significant challenges to healthcare. This study aimed to identify characteristics associated with unplanned PICU readmission during the same hospitalization following cardiac surgery.MethodWe conducted a retrospective analysis using prospectively collected data from a tertiary care children's hospital from January 2015 to January 2021. All pediatric patients who underwent cardiac surgery and were transferred alive from the PICU were included. Each patient readmitted to the PICU was matched to two controls based on age and PELOD score at the time of initial PICU admission postsurgery to identify factors associated with readmission among 72 variables.ResultsOf the 956 patients discharged alive from the PICU after cardiac surgery, 23 (2.4%) required unplanned readmission. Over half (12/23, 52.2%) of these readmissions occurred within 48 h of PICU discharge. Univariate analysis identified preoperative arrhythmias (P = .037) and postoperative single-ventricle physiology (P = .024) as potential risk factors for readmission. Male sex appeared to be a protective factor against unplanned PICU readmission (P = .026). Although preoperative vasopressor requirement, postoperative altered ventricular function and known pleural effusion at PICU discharge were not significantly associated with PICU readmission, these factors showed a trend toward significance. After multivariate analysis, postoperative single-ventricle physiology remained the sole independent risk factor associated with unplanned PICU readmission with an odds ratio of 8.6 (95% confidence interval, 1.9-46.5).ConclusionWhile unscheduled PICU readmissions following cardiac surgery are rare, postoperative single-ventricle physiology emerged as an independent risk factor.

Patients with dextrocardia, heterotaxy syndrome, and single ventricle physiology typically undergo multiple staged palliative operations culminating in a Fontan circulation. Despite these palliative procedures, heart transplantation may become the inevitable final option.We report the case of a 12-year-old female with dextrocardia, heterotaxy syndrome with right atrial isomerism, and single ventricle physiology who had previously undergone staged Fontan palliation. Due to progressive Fontan failure and clinical deterioration, she was evaluated and listed for OHT.She underwent successful OHT without intraoperative complications. Her postoperative course was uneventful, and she showed significant clinical improvement during follow-up.This case highlights that heart transplantation is a viable and life-saving treatment option for patients with complex congenital heart disease following Fontan failure. Although transplantation in patients with dextrocardia poses technical challenges, it can be performed safely with favorable outcomes.

Assessment of the systemic right ventricle (RV) is critical for patients with hypoplastic left heart syndrome (HLHS). Traditional imaging metrics fail to capture the RV's complex geometry and remodeling in HLHS, limiting risk stratification. We aimed to apply statistical shape modeling to a large multicenter cohort of cardiac magnetic resonance data sets to define RV shape variants and evaluate associations with clinical outcomes. Cardiac magnetic resonance from the FORCE (Fontan Outcomes Registry Using CMR Examinations) was analyzed for patients with HLHS post-Fontan. Three-dimensional RV models were segmented at end-diastole and processed using statistical shape modeling (ShapeWorks). Shape modes were extracted via principal component analysis and correlated with RV function, tricuspid regurgitation, remnant left ventricular morphology, and clinical outcomes, including mortality, transplant, and a composite adverse outcome including heart failure. The mean RV shape template of 329 patients with HLHS (mean age, 14.7±6.3 years) depicted a circumferentially dilated RV with loss of septal concavity. RV end-diastolic volume was independently associated with composite adverse outcome (odds ratio, 6.50; P=0.001). Distinct shape modes were identified, including an apical bulge phenotype that was independently associated with composite adverse outcome (odds ratio, 2.45; P=0.047) and mortality/transplant (odds ratio, 4.24; P=0.004). This variant also correlated with RV dilation, hypertrophy, and impaired regional strain. A spheroidal shape was associated with ≥moderate tricuspid regurgitation and tricuspid annular dilation. Remnant left ventricular morphology influenced RV shape and function but not transplant-free survival. Our statistical shape modeling analyses provide novel insights into RV geometric remodeling in HLHS and identify specific shape phenotypes associated with dysfunction and adverse outcomes. Shape-based metrics offer additive prognostic value beyond conventional volumetric analysis, with potential implications for risk stratification and surgical decision-making in single-ventricle physiology.

BACKGROUND: Multimodal Electronic Health Record (EHR) data for congenital heart disease (CHD) is challenging to integrate and requires subspecialty expertise across several medical domains. Single ventricle physiology (SVP), a CHD subtype, exemplifies this challenge due to its heterogeneous traits and variable outcomes. HYPOTHESIS: An integrative, multimodal EHR platform informed by subspecialty domain knowledge can improve SVP outcome analysis by enabling intuitive visualizations, survival analysis, disease trajectory tracking, and predictive modeling—thereby reducing the complexity and manual integration effort. METHODS: Using an in-house prototype, we analyzed 25,000 CHD patients from UCLA Health. We identified 732 SVP patients using a phenotype algorithm with adjudication. International Classification of Diseases (ICD) and CPT codes were collected, and the CHD patient landscape was visualized using UMAP. Kaplan-Meier curves compared survival across SVP subtypes over three years. Additional analyses included outcomes, including arrhythmias, heart failure, stroke, liver disease, protein-losing enteropathy, and surgical procedures (Norwood, Glenn, Fontan, heart transplantation). RESULTS: A prototype platform was developed to study SVP outcomes. Figure 2A showed 732 SVP patients among 25,000 CHD cases, and Figure 2B highlighted the top 15 ICD codes. Four SVP subtypes were summarized in Figure 2C , while Figure 2D displayed patient encounters from 2013–2023. Survival analysis by birth decade ( Figure 2E ) identified 167 patients with complete records, 138 with at least 36 months of follow-up. Fontan and Double Inlet Left Ventricle patients showed higher survival rate ( Figures 2F–2G ). Disease trajectory analysis ( Figure 2H ) revealed reduced arrhythmias and heart failure severity in the first year, underscoring the importance of early intervention. DISCUSSION: This study demonstrated that an integrative, multimodal EHR-based platform can streamline the analysis of SVP patient outcomes, enabling visualization, survival analysis, disease trajectory tracking, and predictive modeling. This approach reduces the domain-specific complexity and highlights the potential of data-driven tools to assist physicians with integration of multimodal data to enhance patient care delivery. CONCLUSION: An EHR-based platform can enhance SVP outcome analysis by integrating multimodal data and alleviating complexity. Future work will incorporate imaging data and extend to other rare diseases.

Introduction: For infants born with single ventricle physiology, the initial palliation involves the implantation of a systemic to pulmonary vascular graft, typically a modified Blalock-Taussig shunt (mBTS) or a Sano shunt. However, irregular blood flow through the life-sustaining shunt can be life-threatening, and state-of-the-art monitoring approaches are either invasive or indirect. Alternative approaches for periodic monitoring of blood flow in a nonclinical setting are needed. Methods: A novel contactless blood flow sensor was developed for integration on the shunt exterior for early detection of flow irregularities. This sensing strategy avoids device-related thrombosis by combining the capacitively-coupled contactless conductivity detection (C4D) principle with time-of-flight measurements to quantify blood flow. Five metal rings are positioned along the shunt exterior, each capacitively-coupled to the conductive blood. The first and last electrodes are used to impose a sinusoidal driving signal and voltage differences are measured between adjacent pairs among the three central electrodes. Time varying conductivity can be tracked and by cross-correlation of the two recordings, in which the downstream measurement is a time-delayed copy of the upstream one, and knowing the precise spacing of the pairs, flow rate can be computed. Results: Sensor prototypes consisted of platinum rings fitted on polytetrafluoroethylene tubing. Capacitive coupling of the rings to blood was evaluated first. Response to varying solution conductivities was then characterized, demonstrating a linear response with the highest sensitivity at lower conductivities. Following successful sensor calibration measurements in test fluid, ex vivo flow measurements using bovine blood were collected at 30 and 50 mL/min. Based on the electrode locations, the delay for the flow rates was expected to be 100 and 70 ms, respectively. Peaks were observed at 100 ms and 60 ms, corresponding to 30 mL/min (0% error) and 42.9 mL/min (14.2% error), respectively. Errors were comparable to current “gold-standard” devices. Conclusions: We successfully demonstrated the feasibility of non-contact flowing monitoring in a format suitable for pediatric vascular shunts. This low power approach can support a fully implantable device to provide early warning of inadequate blood flow and inform timely intervention to reduce morbidity and mortality in infants with single ventricle physiology.