Background/Objectives: In the present study, we hypothesized that cardiac surgery in pediatric patients with congenital heart disease (CHD) leads to profound endothelial glycocalyx degradation, measured as the increase in plasma syndecan-1 concentration, and that this endothelial damage is more pronounced in patients with cyanotic CHD. Methods: A total of 40 infants (24 with acyanotic and 16 with cyanotic CHD) were enrolled in this prospective study. A total of 39 cardiac surgeries were performed with cardiopulmonary bypass (CPB), 38 with CPB and aortic clamping, and 3 with CPB, aortic clamping, and deep hypothermic circulatory arrest. Results: Syndecan-1 concentrations increased significantly post-surgery compared to the baseline in both groups (cyanotic: 24.4 to 48.0 ng/mL, p < 0.0001; acyanotic: 28.8 to 59.8 ng/mL, p < 0.0001). However, there was no significant difference in syndecan-1 concentrations at any timepoint between children with cyanotic and those with acyanotic CHD. Baseline syndecan-1 showed no correlation with preoperative arterial oxygen saturation (r = 0.26, p = 0.102), hemoglobin (r = −0.3, p = 0.06), age (r = 0.15, p = 0.36), and weight (r = 0.13, p = 0.42). Of note, CPB time (r = 0.08, p = 0.63) and AC time (r = 0.03, p = 0.86) were not related to syndecan-1 concentrations at the end of surgery. Conclusions: Cardiac surgery leads to profound glycocalyx degradation in children with CHD detected by increased plasma syndecan-1 concentrations. Regardless of major pathophysiological differences, children with cyanotic and acyanotic CHD presented similar plasma syndecan-1 values throughout the study.

IntroductionParagangliomas (PGLs) are rare neuroendocrine tumors. Atypical manifestations such as skin lesions mimicking vasculitis secondary to catecholamine-induced vasoconstriction are extremely rare. Both hypoxia observed in cyanotic congenital heart disease, like tetralogy of Fallot (TOF), and succinate dehydrogenase B (SDHB) germline mutations can activate hypoxia-inducible factor (HIF) pathways, leading to tumor proliferation.Clinical CaseA 34-year-old female patient, with a history of TOF repair, hypothyroidism, hypertension, carotid body tumor surgery, and autoamputation of two toes in the right foot, presented with necrotic, ulcerative lesions on her extremities. The patient was treated with steroids and methotrexate for 3 months. Examination revealed blood pressure of 100/70 mmHg, a diastolic murmur in the pulmonary area, pretibial edema, right third toe gangrene, and multiple skin lesions (Figure 1). Rheumatologic tests for vasculitis were negative, and the skin biopsy was inconclusive. Abdominopelvic CT performed for vascular imaging and detected a retroperitoneal mass (80x46 mm) in the posterior of the pancreas. 18F-FDG PET/CT showed intense uptake. Significant increase in urinary normetanephrine (10-fold) and norepinephrine (18-fold) levels was detected. 68Ga Dotatate PET/CT revealed intense somatostatin receptor activity. Focal uptake was seen on the MIBG scan. The tumor was inoperable due to involvement of the superior mesenteric and celiac arteries. Genetic analysis identified a pathogenic heterozygous SDHB gene variant (c.262A>C; p.Thr88Pro).Given the ineffectiveness of vasculitis treatment, it was discontinued. The gangrenous toe was amputated, and the patient was managed with hyperbaric oxygen therapy along with alpha blockade therapy. The treatment resulted in the healing of necrotic lesions. 177Lu-Dotatate therapy resulted in partial tumor regression and biochemical response, but it was discontinued due to pancytopenia. Therapy was resumed with thrombopoietin receptor agonist support but later discontinued due to recurrent cytopenia.131I-MIBG therapy was administered. However, despite these interventions, the patient developed progressive cardio-renal complications and died due to sudden arrhythmia.ConclusionThis case highlights the rare hypoxia-related PGL phenotype in a patient with TOF and SDHB mutation. Although SDHB mutations are sufficient to initiate PGL development via pseudohypoxia, the accompanying chronic systemic hypoxia due to TOF may have exerted a synergistic effect by further stabilizing HIF signaling, thereby accelerating tumorigenesis and contributing to the unusual clinical presentation. Cutaneous lesions mimicking vasculitis may be attributed to catecholamine-induced vasoconstriction and ischemia. Recognition of atypical findings may allow for early diagnosis and intervention. Multidisciplinary management is essential in inoperable cases with complex comorbidities.Figure 1:Necrotic cutaneous lesions of the patient’s lower extremity

Patients with cyanotic CHD and those with metabolic dysfunction-associated steatotic liver disease are at risk of liver fibrosis. We compared hepatic extracellular volumes and native T1 values to better understand the burden of liver disease in these populations. The sample comprised 136 patients in 5 groups: control (n = 23), metabolic dysfunction-associated steatotic liver disease [mild (F0-F1) and significant (F2-F4) fibrosis; n = 45], repaired tetralogy of Fallot (n = 30), and Fontan circulation (n = 38). Differences were assessed using linear regression models, with adjustment for the body mass index and sex. The hepatic extracellular volume was significantly larger in the Fontan group (43.96% ± 4.22%) than in the other groups, even with adjustment. Patients with Fallot had significantly larger extracellular volumes (36.77% ± 5.63%) than did controls and mild liver disease (p < 0.001 and p = 0.011, respectively), although smaller extracellular volumes than patients with significant liver disease (p = 0.042). These trends were corroborated by native T1 values, which were highest in patients with Fontan (1013.7 ± 86.1 ms), although not significantly different from patients with F2-F4 steatotic liver disease. The potential burden of CHD-related hepatic injury and steatotic liver disease highlights the importance of early identification. Given the possible additional risk of liver fibrosis in patients with coexisting metabolic dysfunction and CHD, comprehensive clinical management should prioritise regular metabolic risk assessment and the promotion of a healthy lifestyle to reduce the likelihood of liver disease development in this vulnerable population.

We report the case of a male in his late teens who presented to our emergency department with acute generalised tonic-clonic seizures. Neurological examination, neuroimaging and laboratory tests confirmed severe hypocalcaemia, secondary to hypoparathyroidism, as the cause of his seizures. A detailed history was significant for recurrent urinary tract infections and epilepsy, accompanied by impaired academic performance and an intracardiac repair 8 years prior to presentation for a congenital cyanotic heart disease. Dysmorphic features on clinical examination, accompanied by the chronicity of complaints, led us to consider a genetic syndrome. On genomic microarray analysis (GMA), a 22q11.21 deletion was detected. The present case aims to highlight how commonly encountered laboratory findings, such as hypocalcaemia, can facilitate the identification of genetic associations like 22q11.2 deletion syndrome (22q11.2DS), a rare diagnosis in adulthood. In many cases, clinical problems may be managed in isolation without triggering a unifying diagnosis.

To evaluate whether complete hilum-to-hilum bilateral pulmonary artery (PA) mobilization at the time of systemic-to-pulmonary artery shunt (APS) is associated with greater two-year PA growth than conventional APS without mobilization in cyanotic congenital heart disease (CCHD), particularly in patients receiving 3.5-4.0-mm conduits.We retrospectively analyzed 245 consecutive infants who underwent APS between January 2018 and September 2025. Patients were stratified by technique: Group A (n = 128) received complete bilateral PA mobilization with a 3.5- or 4.0-mm shunt; Group B (n = 117) underwent standard APS without mobilization. The primary endpoint was change in McGoon index (sum of branch PA diameters normalized to the diameter of the descending aorta at the diaphragm) from baseline to 24 months, assessed by transthoracic echocardiography, CT angiography, or cardiac MRI. Secondary endpoints included shunt patency and hospital length of stay (LOS).Baseline characteristics were similar (age 1.4 ± 0.7 vs. 1.4 ± 0.6 years; weight 6.1 ± 1.8 vs. 6.2 ± 1.8kg; all p > 0.05). Group A showed a greater increase in McGoon index (1.26 ± 0.11 to 1.62 ± 0.14; p < 0.001) than Group B (1.22 ± 0.09 to 1.42 ± 0.12; p < 0.001), with a significant between-group difference at 24 months (p < 0.001; Cohen's d = 1.49). ΔMcGoon correlated inversely with LOS (ρ=-0.844; p < 0.001). Shunt patency was higher with mobilization (96.1% vs. 88.9%; p = 0.03).In this two-year cohort, complete bilateral PA mobilization during APS was associated with greater PA growth, higher shunt patency, and a shorter hospital stay in the mobilization group, although the era-based design precludes definitive causal inference. These findings support incorporation of hilum-to-hilum mobilization as a standardized, modifiable operative step, while prospective, adjusted analyses are needed to confirm durability and subgroup effects.

Organ-level radiation dose estimations from cardiac catheterizations in neonates with tetralogy of Fallot.

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease requiring lifelong surveillance post-repair. Cardiovascular magnetic resonance (CMR) is the gold standard for assessing right ventricular function and pulmonary regurgitation, but limited data exists from South Asian populations. This study evaluates CMR findings in repaired TOF patients and assesses its role in pulmonary valve replacement (PVR) decision-making in a Pakistani cohort. Methods: This cross-sectional study included 80 consecutive patients with surgically repaired TOF who underwent CMR evaluation ≥2 years post-repair at Hayatabad Medical Complex, Peshawar (January 2020-December 2024). CMR was performed using 1.5 Tesla scanners with comprehensive protocols. Primary outcomes included right ventricular volumes, ejection fraction, and pulmonary regurgitation severity. Results: The cohort comprised 80 patients (mean age 14.5 ± 3.2 years, 56.25% male) with median 8.9 years since repair. Right ventricular dysfunction was prevalent with mean RVEF of 39 ± 11% and dilatation in 87.5% (mean RVEDVi - right ventricular end-diastolic volume index) 128.6 ± 32.4 mL/m²). Left ventricular function remained preserved (Left ventricular ejection fraction LVEF 53 ± 6%). Severe pulmonary regurgitation (&gt;40%) occurred in 56.25% with mean regurgitation fraction of 36 ± 13%. Strong correlations existed between pulmonary regurgitation severity and right ventricular parameters (p &lt; 0.001). PVR (Pulmonary valve replacement) was recommended in 81.3% of patients with 92.3% guideline adherence. Residual anatomical defects significantly worsened outcomes. Time since surgery correlated with progressive right ventricular deterioration (p &lt; 0.001). Conclusions: High prevalence of right ventricular dysfunction and severe pulmonary regurgitation exists in Pakistani repaired TOF patients. CMR demonstrated strong correlations with clinical outcomes and proved essential for treatment planning. These findings emphasize CMR's critical role in guiding timely interventions and optimizing long-term management in resource-limited settings.

Background: Pregnancy complicated by maternal heart disease remains a leading cause of morbidity and mortality for both mothers and neonates. However, information regarding the factors influencing cardiovascular events in neonates and mothers within 6 months postpartum is limited. This study aimed to identify predictors of adverse maternal and neonatal outcomes in pregnancies of women with pre-existing heart disease. Methods: The study conducted a retrospective cross-sectional analysis of all pregnancies with a history of heart disease from January 2017 to January 2024, using secondary data from medical records at Dr M. Djamil Padang Hospital, Indonesia. We analysed the data using the χ2/Fisher’s exact test and logistic regression. Results: A total of 110 pregnant women with heart diseases were hospitalised during the study period, with an average age of 30.52 ± 6.00 years. The most common types of heart disease were valvular heart disease (28%), chronic hypertension (24%) and congenital heart disease (23%). In 27.2% of cases, adverse maternal outcomes included mortality (10%), heart failure (8.2%), arrhythmia (4.5%), rehospitalisation (3.6%) and thromboembolic event (0.9%). Meanwhile, 57.3% of cases had neonatal adverse outcomes, including mortality (5.4%), low Apgar score (16.4%), prematurity (28.2%), low birthweight (6.4%) and congenital heart disease (0.9%). The multivariate analysis discovered that moderate-to-severe left heart obstruction (OR 14.184; 95% CI [2.725–73.836]; p=0.002) and reduced ejection fraction &lt;40% (OR 16.211; 95% CI [1.712–153.507]; p=0.015) were the main factors that led to adverse maternal outcomes. Meanwhile, cyanotic congenital heart disease (OR 8.449; 95% CI [1.030-69.328]; p=0.047) was the most important determinant of adverse neonatal outcomes. Conclusion: This study revealed that moderate-severe left heart obstruction and reduced ejection fraction &lt;40% were the most significant factors influencing adverse maternal outcomes. Meanwhile, cyanotic congenital heart disease was the most important determinant of adverse neonatal outcomes.

This study aims to examine the incidence and clinical outcomes of critical congenital heart disease (CCHD) in neonates in China, with a specific focus on prognostic indicators following surgical intervention. A retrospective analysis was conducted on neonates who underwent surgical correction of congenital heart disease (CHD) involving cardiopulmonary bypass (CPB) at a single tertiary care hospital between January 2022 and December 2024. Documented variables included patient weight, type of cardiac anomaly, duration of CPB, aortic cross-clamp time, and systemic circulation duration. Prognostic indicators, length of hospital stay, and duration of mechanical ventilation in the intensive care unit (ICU) were also recorded. A total of 234 neonates underwent cardiac surgery with CPB during the study period. The cohort comprised 146 males and 88 females, with a mean weight of 3.64 ± 3.43kg and a mean age of 12.10 ± 7.77 days. The mean duration of CPB time was 159.19 ± 67.51min, mean cross-clamp duration was 95.53 ± 44.20min, and mean bypass duration was 37.54 ± 28.98min. The mean length of hospital stay was 25.43 ± 13.61 days, and the mean mechanical ventilation duration was 3.64 ± 2.71 days. Among the patients, 139 were diagnosed with cyanotic CHD, and 95 with acyanotic CHD. Emergency procedures were performed in 39 cases, while 195 cases underwent elective surgery. Radical surgery was performed in 215 cases, single-stage palliation in 4 cases, and intrapartum surgery in 15 cases. 222 neonates were discharged following recovery, and 12 deaths were recorded. In China, CCHD remains prevalent among newborns, posing a significant threat to their lives. With advancements in surgical and cardiopulmonary bypass techniques, the treatment system for these critically ill newborns is being progressively refined. This has led to continuously improving success rates and an increasingly diverse range of treatment options available.

Background: In cardiology, vasoregulation is one of the most important targets of pharmacotherapy. SOMNOtouch™-NIBP (SOMNOmedics AG, Randersacker, Germany) is a cuffless device designed for continuous, non-invasive blood pressure measurements, and it appears to be ready for use in infants and children with congenital heart disease. For infants, minor methodological modifications are required due to their small body size. Methods: Using this device, we demonstrate fluctuations in diastolic blood pressure in three patients: an infant with hypoplastic left heart syndrome after Norwood stage 1 and 2 operations; an infant with Tetralogy of Fallot with heart failure due to pulmonary overcirculation after an aorto-pulmonary shunt implantation; and a 13-year-old girl with chronic cyanosis due to a congenitally corrected transposition of the great arteries (ccTGA) with a ventricular septal defect and pulmonary stenosis. The measurement procedures are completely non-invasive and feasible in an outpatient setting. Results: The results demonstrate strong correlations between blood pressure and oxygen saturation levels as well as heart rate variability. We discuss our results in relation to current concepts of hypoxic pulmonary/systemic vasoconstriction and hypoxemia-related pathways. Conclusions: The cuffless device for continuous, non-invasive blood pressure measurement seems to be useful for infants with and without congenital heart defects who receive pharmacotherapies that modulate vasoregulation. These patients should also be non-invasively monitored for safety reasons and for a better understanding of their pathophysiology.

Vascular endothelial growth factor (VEGF) is a factor that is responsible for cell proliferation, growth of vascular endothelial cells, and angiogenesis. Changes in the level of this factor are associated with the pathology of structural disorders such as CHD. This systematic study assesses previous studies in order to find the VEGF influences on congenital heart disorders. This systematic review was written based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) criteria, and the principle of non-bias was respected. All the articles from 2014 to 2024 were extracted from Web of Science, PubMed, and Scopus databases. We investigated the role of VEGF in the pathology of cardiovascular structural disorders, the therapeutic and diagnostic effects of VEGF, and related factors that are influenced by this factor. Studies assessed based on PRISMA search steps and 22 were included in our study. Any disturbance in the production and functioning of VEGF is known as a genetic disorder in tetralogy of Fallot (TOF). VEGF caused abnormal elongation of the heart tubes, as well as disproportionate growth of cardiovascular tissue just before full formation. The increase of Hypoxia-inducible factor (HIF) with the increase of VEGF function precedes the development of the fetal heart. HIF also mediates endothelial formation through endothelial nitric oxide synthases (eNOS); HIF in children with cyanotic CHD (CCHD) and acyanotic CHD (ACHD) is significantly higher than in the control group, and its value is higher in complex CHD children than in the other groups. EGFR, inducible NOS (iNOS), and ET-1 were more in ACHD than in CCHD, and their amounts showed a positive correlation with HIF. The increase in the level of VEGF and HIF before the completion of the heart tissue is the main cause of CHD pathology; after the completion of the heart tissue, these factors help in the regeneration of the heart tissue. The regulation of VEGF and HIF levels during the fetal period is of great importance for the diagnosis and pathological aspect of CHD.

SDHB Mutation and Chronic Hypoxia: Dual Drivers of Paraganglioma in Cyanotic Congenital Heart Disease

Abdominal compartment syndrome in the pediatric population - Case series and review of the literature.

Neurodevelopmental impairments in congenital heart disease (CHD) are the most frequent long-term morbidity. Adverse neurodevelopmental outcomes may start in the prenatal period. Maternal mental health may be a potentially modifiable risk factor for the optimisation of neurodevelopment in CHD. We propose to assess the impact of prenatal maternal mental health on 1-year neurodevelopmental outcomes in complex CHD. Neuro-Moms CHD is a national multi-centre, prospective study of prenatal maternal mental health and neurodevelopmental outcomes in children with complex CHD who undergo neonatal open-heart surgery. Participants (n=87 mother-child dyads) will be recruited from five major French paediatric cardiology centres (Necker Children's Hospital in Paris, Bordeaux Cardiology Hospital, Marseille Children's Hospital, Montpellier University Hospital and Saint-Pierre Institute). Expecting women who receive a prenatal diagnosis of fetal complex cyanotic CHD that requires a neonatal open-heart surgery for the newborn are eligible to participate. They will complete self-reports on mental health, anxiety, depression and coping skills and will participate in a semi-structured psychological interview. Mothers will provide information on medical, sociodemographic and lifestyle factors. They will be enrolled during the third trimester of pregnancy and will participate at three time points: prenatal, T1; after the newborn's cardiac surgery, T2; and between 12 and 18 months after birth of the child with CHD, T3. Children with CHD will undergo a standardised neurodevelopmental assessment when they turn 12-18 months old. The father or co-parent of the child with CHD will also participate in T1 and will complete mental health self-reports. We will use a structural equation model to estimate simultaneously the relationships among maternal mental health, prenatal factors and child neurodevelopment outcomes. This study is sponsored by the French National Institute of Health and Medical Research. It was approved by the Ethics Committee on 5 November 2024 and is registered in a public trials registry (NCT06711666). Neuro-Moms CHD targets a public health question with important societal implications. Results are expected to be broadly communicated with the scientific community and the lay public. Dissemination of findings will be in the form of scientific articles in peer-reviewed journals and presentations at conferences. Any publication or communication will comply with the international recommendations: 'Recommendations for the Conduct, Reporting, Editing, and Publication of Scholarly work in Medical Journals' (http://www.icmje.org/recommendations). All participants will give written informed consent or assent to participate. The anonymised data to be collected in this study will be available within the manuscripts published. NCT06711666; pre-results.

Arrhythmias in Congenital Heart Disease: Tetralogy of Fallot.

Objectives: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in infancy, frequently requiring early surgical repair. Detailed pre-operative imaging is essential for assessing cardiovascular and extracardiac anatomy to guide surgical planning. While conventional invasive catheter angiography (ICA) has traditionally been used for this purpose, computed tomography angiography (CTA) has emerged as a promising non-invasive alternative with lower risk, shorter procedure times, and reduced radiation exposure. Given the vulnerability of pediatric patients, optimizing radiation dose and understanding its determinants – such as body size – are critical. The objective of the study is to compare radiation exposure, contrast agent usage, and procedural duration between CTA and ICA in the pre-operative evaluation of TOF patients under 1 year of age and to evaluate the correlation between patient anthropometric measurements and radiation dose. Methods: This retrospective single-center study included 120 TOF patients who underwent complete surgical repair between 2021 and 2024. Seventy-four patients underwent CTA and 46 underwent ICA. Data on radiation dose, contrast volume, and procedure time were collected. Correlations between effective dose and patient age, weight, height, and BMI were analyzed. Results: CTA significantly reduced effective radiation dose (1.21±0.19 mSv) compared to ICA (5.28±1.71 mSv, p&lt;0.01), required less contrast agent (8.02±2.38 cc vs. 38.8±13.01 cc), and had a shorter procedure time (3.1±0.58 min vs. 21.03±10.79 min; p&lt;0.001). Radiation dose was positively correlated with weight (CTA: r=0.41, p=0.0006; ICA: r=0.52, p=0.0009), height (CTA: r=0.32, p=0.0012; ICA: r=0.24, p=0.0014), and BMI (CTA: r=0.35, p=0.0008; ICA: r=0.39, p=0.0053), while no significant correlation was observed with age. Conclusion: CTA is a safe, non-invasive, and time-efficient imaging modality for the pre-operative evaluation of TOF patients, offering significant reductions in radiation dose and contrast use compared to ICA. The observed correlation between radiation dose and body size underscores the need for individualized imaging protocols in pediatric practice.

Background: Insulin-like growth factor-1 (IGF-1) and its binding protein, IGFBP-3, are crucial mediators of childhood growth and development. Alterations in their serum levels have been implicated in congenital heart disease (CHD), especially in relation to chronic hypoxia and growth failure. Aim: To evaluate serum IGF-1 and IGFBP-3 levels in children with CHD, to compare variations between cyanotic and acyanotic subtypes, and to explore correlations with anthropometric and clinical parameters. Methods: A cross-sectional comparative study was conducted on 82 children aged 2 months to 5 years: Group 1 (cyanotic CHD, n=12), Group 2 (acyanotic CHD, n=30), and Group 3 (control group, n=40). Serum IGF-1 and IGFBP-3 were measured using enzyme-linked immunosorbent assay (ELISA). Statistical analysis was performed using ANOVA and Pearson's correlation tests.

Infective Endocarditis Risk After Invasive Dental Procedures

Abstract Chordomas are rare slow-growing malignant bone tumors of notochord origin, with incidence of 0.08 per 100,000/year and median 5-year survival of 67%. Fewer than 5% occur before age 20. Most pediatric chordomas arise from the skull-base. Tetralogy of Fallot, a heterogeneous congenital cyanotic heart disease comprised of a ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy, is also a rare disease, with incidence of approximately 1 in 2,000 live births. Here we describe a girl with Tetralogy of Fallot who at age 10 years presented with 3 months of dysarthria, difficulty swallowing, morning headaches, and tongue paresthesia and fasciculations. Brain MRI showed a clival chordoma infiltrating into the pons and medulla. The tumor was partially resected. Pathology of the tumor showed cords and nests of epithelioid cells in a chondromyxoid background, expressing brachyury, cytokeratin (CK AE1/AE3), and S100 and no DNA sequence variants or RNA gene fusions of clinical significance by Onco-Kids somatic panel. Patient received 73.5 Gy to the clivus with IMRT (brainstem dose of 62 Gy to 0.03 cc), with the partially-resected tumor remaining stable, now 4 years following diagnosis. To date, this is the first description of these two rare conditions, chordoma and Tetralogy of Fallot, occurring in the same patient. Interestingly, both clival chordoma and Tetralogy of Fallot are associated with the T-box gene family of developmental transcription factors: Brachyury (TBXT) is expressed in chordomas and alterations in TBX1, TBX5 and TBX20 can contribute to Tetralogy of Fallot. While random co-occurrence is not impossible (likelihood of approximately 1 in 2.08 ×109), presentation of this rare skull-based tumor of embryological origin within the context of dysregulated cardiac development suggests the intriguing possibility of a yet-unknown biological connection between the two conditions, potentially via connection of T-box developmental transcription factors.

Background: Cyanotic congenital heart disease (CCHD) poses significant perioperative challenges, particularly during emergency neurosurgical procedures. Double outlet right ventricle (DORV) is a rare cyanotic congenital heart disease that complicates anesthetic management due to chronic hypoxemia and right-to-left shunting. Case Presentation: We report the case of a 5-year-old boy with uncorrected DORV who sustained severe traumatic brain injury after a fall. Head CT revealed a large right temporoparietooccipital epidural hematoma with subdural and subarachnoid hemorrhage and a 1.7 cm midline shift. Emergency craniotomy was performed under general anesthesia with invasive hemodynamic monitoring and cautious ventilatory management to balance cerebral and cardiac demands. Although intraoperative hemodynamics remained stable and the hematoma evacuation was technically successful, the patient developed persistent postoperative hypoxemia and multiorgan failure, resulting in death on postoperative day three. Despite a technically successful evacuation of the hematoma and stable intraoperative parameters, the patient’s postoperative course was complicated by persistent hypoxemia due to right-to-left shunting, culminating in multiorgan failure and death after three days of intensive care. This case underscores the profound anesthetic and surgical challenges posed by the coexistence of severe traumatic brain injury and uncorrected cyanotic congenital heart disease, highlighting the importance of multidisciplinary collaboration, tailored perioperative strategies, and the urgent need for early cardiac corrective interventions to improve outcomes in similar high-risk scenarios. Conclusion:This case demonstrates the profound anesthetic and surgical challenges of emergency neurosurgery in pediatric patients with uncorrected cyanotic congenital heart disease. Effective management requires multidisciplinary collaboration, individualized anesthetic strategies, and early cardiac correction to improve survival in such high-risk scenarios.