Cirugía congénitos

Acta Paediatr. 2025 Oct 8. doi: 10.1111/apa.70332. Online ahead of print.

ABSTRACT

AIMS: To evaluate growth and body composition at NICU discharge in term-born neonates with structural congenital anomalies and identify predictors of body fat percentage.

METHODS: This prospective observational study included 61 term neonates (≥ 37 weeks' gestation) with cardiac or non-cardiac congenital anomalies requiring surgery. Anthropometric measures at birth and discharge and body composition at discharge were measured.

RESULTS: Mean z-scores declined significantly between birth and discharge for weight (-0.38 to -1.53), length (-0.02 to -0.64), and head circumference (-0.04 to -0.79) (all p < 0.001). Infants with congenital heart disease demonstrated a more pronounced weight z-score decline. At discharge, mean body fat was 9% (SD 4.9), with no significant differences between cardiac and non-cardiac anomalies or sexes. Fat-free mass was relatively preserved (91%, 910 g/kg). Multivariate analysis identified time of full enteral feeds (p = 0.02) and maternal ethnicity (p = 0.03) as independent predictors of body fat.

CONCLUSION: Term neonates with congenital anomalies experience significant postnatal growth restriction and reduced fat mass by NICU discharge. Maternal ethnicity and time to full enteral feeds independently influence body fat percentage. These findings support the need for individualised nutrition and routine body composition monitoring to optimise outcomes in this high-risk group.

PMID:41059561 | DOI:10.1111/apa.70332

J Rural Med. 2025 Oct;20(4):311-313. doi: 10.2185/jrm.2025-006. Epub 2025 Oct 1.

ABSTRACT

OBJECTIVE: Venous malformations (VMs) are low-flow blood reservoir mass lesions with spongy or cystic venous lumens caused by dilation of the venous system vasculature. A VM is a congenital lesion that presents at birth and enlarges during childhood as the host grows. Subcutaneous lesions due to VMs are rarely diagnosed at an advanced age. We report a case of axillary VM in an older patient.

PATIENT: A 92-year-old man was suspected of having left axillary lymphadenopathy (single nodule, 23 mm long) on chest computed tomography (CT) scan. Seven years earlier, a chest CT scan showed a small, 13 mm nodule in the same area.

RESULTS: On ultrasound elastography, the lesion did not appear hard, however, color Doppler imaging revealed abundant blood flow, suggesting a malignant lesion. Histologically, the lesions were consistent with VM.

CONCLUSION: VMs should be considered a differential diagnosis for superficial lymphadenopathy, even in older patients.

PMID:41059371 | PMC:PMC12497984 | DOI:10.2185/jrm.2025-006

Crit Care Explor. 2025 Oct 3;7(10):e1327. doi: 10.1097/CCE.0000000000001327. eCollection 2025 Oct.

ABSTRACT

OBJECTIVE: To train and test supervised machine learning (ML) models to predict low cardiac output syndrome (LCOS) within the first 48 postoperative hours in neonates undergoing cardiothoracic surgery.

DESIGN: Retrospective observational study. An efficient tree-based gradient-boosting algorithm (LightGBM) ML models were developed to predict LCOS occurrence at 2-, 4-, 6-, and 12-hour forecasting horizons, incorporating data from the prediction time and the two preceding hours. SHapley Additive exPlanations (SHAP) analyses were used for feature importance analyses.

SETTING: Single center, January 2012 to April 2023.

PATIENTS: Neonates 28 days old or younger who underwent cardiothoracic surgery.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: A total of 181 neonates were included, with 14.9% experiencing LCOS. A multivariate time-series dataset was constructed using hourly clinical and laboratory variables recorded during the first 48 postoperative hours. The LightGBM ML models achieved area under the receiver operating characteristic curve values ranging from 0.91 to 0.98 and area under the precision-recall curve values ranging from 0.60 to 0.80 for LCOS prediction across 2-, 4-, 6-, and 12-hour forecasting horizons. SHAP analyses identified higher vasoactive inotrope score, lower urine output, and higher serum lactate as the most influential predictors.

CONCLUSIONS: This study demonstrates that the supervised machine learning models can accurately predict LCOS in neonates, offering high interpretability. The findings support further validation in multicenter settings and integration into clinical workflows to enhance postoperative critical cardiac care neonates.

PMID:41058756 | PMC:PMC12499747 | DOI:10.1097/CCE.0000000000001327

Cardiol Young. 2025 Oct 8:1-4. doi: 10.1017/S1047951125109761. Online ahead of print.

ABSTRACT

BACKGROUND: Clinical outcomes of a standardised enhanced recovery after surgery protocol, including thoracic epidural analgesia, were studied in children undergoing trans-atrial cardiac surgery via the right mid-axillary thoracotomy approach.

METHODS: This single-centre retrospective study examined 42 paediatric patients who underwent trans-atrial cardiac surgeries via the mid-axillary approach (2018 to 2020), of whom 30 received epidural catheters. The standardised enhanced recovery after surgery protocol included transesophageal echo-guided thoracic epidural catheter placement, multimodal analgesia, reduced opioid use, and planned early extubation and discharge. Clinical outcomes assessed included extubation times, postoperative analgesic requirements, ICU pain scores, hospital length of stay, and any complications related to regional anaesthesia or surgery.

RESULTS: Thirty patients received an epidural placed between the third and sixth thoracic interspace levels under transesophageal ultrasonography guidance. The median age was 42 months (range 3-156), and the median weight was 15.7 kg (range 4.9-61 kg). Epidural infusions were continued for a median of 52 hours postoperatively. The intraoperative fentanyl usage was a median of 4.5 mcg/kg (interquartile range (IQR) 2-9). Intraoperative extubation was achieved in 28 of the 30 patients. Median post-extubation pain scores in the first 6 and 12 hours were 0 (IQR 0), and postoperative rescue opioid requirements were low in epidural patients. There were no instances of tracheal reintubation, neuraxial blockade-related complications, or other anaesthesia-related adverse events.

CONCLUSION: Through our standardised and reproducible anaesthetic protocol, we achieved excellent and nearly pain-free recovery in paediatric patients undergoing trans-atrial cardiac surgeries via the mid-axillary approach.

PMID:41058510 | DOI:10.1017/S1047951125109761

Semin Pediatr Surg. 2025 Sep 11:151539. doi: 10.1016/j.sempedsurg.2025.151539. Online ahead of print.

ABSTRACT

OBJECTIVE: To investigate the risk factors of postoperative poor incision healing in children with congenital pectus excavatum who underwent thoracoscopic-assisted Nuss procedure.

METHODS: In this study, a retrospective cohort study method was adopted to statistically analyze the clinical data of children with congenital pectus excavatum who underwent thoracoscopic-assisted Nuss procedure from 2017 to 2024. The children were categorized into groups with good incision healing and groups with poor healing according to the criteria, and the risk factors that might affect the incision healing were collected, including gender, age, height, weight, BMI, HI, type of pectus excavatum, and previous allergy history, number of the Nuss bars, whether fixation piece was applied, fixation method, suture method, surgical time and bleeding amount.

RESULTS: Based on the inclusion and exclusion criteria, a total of 272 children with Nuss procedure were collected, and during the follow-up period, 25 children experienced poor incision healing. Univariate and multivariate logistic regression analyses were performed in both groups, and the results showed that low BMI, asymmetric pectus excavatum, previous allergy history, wire-fixed bars, and intradermal sutures were independent risk factors for poor incisional healing after the Nuss procedure for pectus excavatum (all P < 0.05).

CONCLUSION: Poor postoperative incision healing is more likely to occur in children with low BMI, asymmetric pectus excavatum, previous allergy history, and children with intraoperative wire fixation of bars and intradermal sutures.

PMID:41058358 | DOI:10.1016/j.sempedsurg.2025.151539

Innovations (Phila). 2025 Oct 7:15569845251375442. doi: 10.1177/15569845251375442. Online ahead of print.

ABSTRACT

Despite efforts to improve donor heart allocation policies, geographic disparities, logistical challenges and patient criteria limit organ availability. However, recent surgical innovations offer potential solutions. These include partial heart transplantation and xenotransplantation. Partial heart transplantation is a new procedure with the potential to address several clinical challenges in treating congenital heart defects. Cardiac xenotransplantation involves the transplantation of genetically modified porcine hearts into humans, offering a potential solution to the shortage of donor organs. Although immunologic barriers and ethical concerns remain, ongoing research aims to mitigate the risks and optimize outcomes, providing hope for patients in need. These innovative surgical approaches offer promising avenues for addressing the critical shortage of donor hearts and with ongoing research, may hold the potential to revolutionize heart transplantation, and improve outcomes for patients facing terminal heart failure.

PMID:41058154 | DOI:10.1177/15569845251375442

J Clin Invest. 2025 Oct 7:e186287. doi: 10.1172/JCI186287. Online ahead of print.

ABSTRACT

Genetic factors are fundamental in the etiology of thoracic aortic aneurysm and dissection (TAAD), but the genetic cause is detected in only about 30% of cases. To define unreported TAAD-associated sequence variants, exome and gene panel sequencing was performed in 323 patients. We identified heterozygous CDKL1 variants [c.427T>C p.(Cys143Arg), c.617C>T p.(Ser206Leu), and c.404C>T p.(Thr135Met)] in 6 patients from 3 families with TAAD-spectrum disorders. CDKL1 encodes a protein kinase involved in ciliary biology. Amino acid substitutions were predicted to affect CDKL1 catalytic activity or protein binding properties. CDKL1 was expressed in vascular smooth muscle cells in normal and diseased human aortic wall tissue. Cdkl1 knockdown and transient knockout in zebrafish resulted in intersomitic vessel (ISV) malformations and aortic dilation. Co-injection of human CDKL1wildtype, but not CDKL1Cys143Arg and CDKL1Ser206Leu RNA, rescued ISV malformations. All variants affected CDKL1 kinase function and profiling data, and altered protein-protein binding properties, particularily with ciliary transport molecules. Expression of CDKL1 variants in heterologeous cells interfered with cilia formation and length, CDKL1 localization, and p38-MAPK and Vegf signaling. Our data suggest a role of CDKL1 variants in the pathogenesis of TAAD-spectrum disorders. The association between primary cilia dysregulation and TAAD expands our knowledge of the underlying molecular pathophysiology.

PMID:41056017 | DOI:10.1172/JCI186287

Crit Care Explor. 2025 Oct 3;7(10):e1327. doi: 10.1097/CCE.0000000000001327. eCollection 2025 Oct 1.

ABSTRACT

OBJECTIVE: To train and test supervised machine learning (ML) models to predict low cardiac output syndrome (LCOS) within the first 48 postoperative hours in neonates undergoing cardiothoracic surgery.

DESIGN: Retrospective observational study. An efficient tree-based gradient-boosting algorithm (LightGBM) ML models were developed to predict LCOS occurrence at 2-, 4-, 6-, and 12-hour forecasting horizons, incorporating data from the prediction time and the two preceding hours. SHapley Additive exPlanations (SHAP) analyses were used for feature importance analyses.

SETTING: Single center, January 2012 to April 2023.

PATIENTS: Neonates 28 days old or younger who underwent cardiothoracic surgery.

INTERVENTIONS: None.

MEASUREMENTS AND MAIN RESULTS: A total of 181 neonates were included, with 14.9% experiencing LCOS. A multivariate time-series dataset was constructed using hourly clinical and laboratory variables recorded during the first 48 postoperative hours. The LightGBM ML models achieved area under the receiver operating characteristic curve values ranging from 0.91 to 0.98 and area under the precision-recall curve values ranging from 0.60 to 0.80 for LCOS prediction across 2-, 4-, 6-, and 12-hour forecasting horizons. SHAP analyses identified higher vasoactive inotrope score, lower urine output, and higher serum lactate as the most influential predictors.

CONCLUSIONS: This study demonstrates that the supervised machine learning models can accurately predict LCOS in neonates, offering high interpretability. The findings support further validation in multicenter settings and integration into clinical workflows to enhance postoperative critical cardiac care neonates.

PMID:41056014 | DOI:10.1097/CCE.0000000000001327

Cardiol Young. 2025 Oct 7:1-10. doi: 10.1017/S1047951125109773. Online ahead of print.

ABSTRACT

BACKGROUND: Double-outlet right ventricle is a complex congenital cardiac anomaly in which both great arteries arise predominantly from the right ventricle. Accurate anatomical evaluation is critical for surgical planning. While transthoracic echocardiography is commonly used for intracardiac assessment, its limitations in visualising extracardiac structures highlight the need for additional imaging modalities like CT angiography.

OBJECTIVE: To compare the diagnostic accuracy and clinical utility of transthoracic echocardiography and CT angiography in the pre-operative evaluation and surgical planning of infants with double-outlet right ventricle.

METHODS: This retrospective, single-centre study included 78 infants diagnosed with double-outlet right ventricle. All patients underwent both transthoracic echocardiography and CT angiography before surgical intervention. Imaging findings were compared with intraoperative surgical results to assess sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy for each modality.

RESULTS: CT angiography was significantly more effective than transthoracic echocardiography in identifying extracardiac anomalies such as coronary artery anomalies (p = 0.014), aortic arch hypoplasia (p = 0.024), aortopulmonary collateral vessels (p = 0.039), anomalous pulmonary venous connections (p = 0.001), and persistent left superior vena cava (p = 0.001). In contrast, transthoracic echocardiography was more sensitive in detecting small ventricular septal defects (p = 0.035).

CONCLUSION: Transthoracic echocardiography remains the first-line modality for intracardiac evaluation in double-outlet right ventricle, but CT angiography provides superior visualisation of extracardiac structures. The combined use of both imaging techniques improves diagnostic accuracy and surgical planning, supporting better outcomes in the management of CHD.

PMID:41054313 | DOI:10.1017/S1047951125109773

Ann Thorac Surg. 2025 Oct 4:S0003-4975(25)00948-8. doi: 10.1016/j.athoracsur.2025.08.070. Online ahead of print.

ABSTRACT

BACKGROUND: We intended to identify whether the lateral tunnel (LT) and extra-cardiac conduit (ECC) Fontan provide similar short-term outcomes in contemporary data.

METHODS: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database who underwent a primary LT or ECC from 2012 to 2022 were included. Hospital mortality, 30 days readmission, and complications were investigated.

RESULTS: There were 11,429 Fontan procedures performed in 133 centers: 2,012 LT (18%) and 9,417 ECC (82%). Only 3% of surgeons (9) were exclusively performing LT while 57% (190) surgeons were exclusively performing ECC and 40% (132) were using both techniques. Similarly, only one hospital performed LT exclusively, while 37% (50) performed the ECC and 62% (82) used both techniques. The age at the time of the Fontan surgery was younger in the LT group (p<0.001). Fenestration was made in 85% of patients with LT and 57% of patients with ECC (p<0.001). The operative mortality (1.1% for LT vs 1.3% for ECC; p= 0.49) was similar. Patients undergoing ECC had higher rate of 30-day readmission (1445 (18%) vs 220 (14%), p< 0.001), prolonged length of stay >30days (583 (6%) vs 78 (4%), p< 0.001), pleural effusion requiring drainage (1147 (17%) vs 140 (13%), p< 0.001), chylothorax (864 (13%) vs 98 (9%), p< 0.001), and stroke (117 (1.8%) vs 9 (0.9%)), p=0.03).

CONCLUSIONS: In conclusion, the Lateral Tunnel Fontan has similar mortality and lower short-term complication rates than the extra-cardiac conduit Fontan, but the latter has become the most frequently performed technique.

PMID:41052666 | DOI:10.1016/j.athoracsur.2025.08.070

BJA Educ. 2025 Oct;25(10):400-410. doi: 10.1016/j.bjae.2025.07.003. Epub 2025 Aug 12.

NO ABSTRACT

PMID:41050565 | PMC:PMC12491798 | DOI:10.1016/j.bjae.2025.07.003

CASE (Phila). 2025 Jul 18;9(9):301-304. doi: 10.1016/j.case.2025.05.003. eCollection 2025 Sep.

ABSTRACT

PMID:41049621 | PMC:PMC12490570 | DOI:10.1016/j.case.2025.05.003

Respirol Case Rep. 2025 Oct 2;13(10):e70369. doi: 10.1002/rcr2.70369. eCollection 2025 Oct.

ABSTRACT

Phrenic nerve injury is a recognised complication of congenital cardiac surgery in children, potentially leading to diaphragmatic paralysis and long-term respiratory dysfunction. Diaphragmatic plication is performed to improve lung mechanics, but its mid-term outcomes remain underexplored. The objective was to assess mid-term pulmonary function in children who underwent diaphragmatic plication for phrenic nerve palsy following congenital heart surgery. This retrospective case series included five children who received left diaphragmatic plication between 2011 and 2021 at a tertiary paediatric centre. Pulmonary function was evaluated using spirometry, plethysmography, and the 6-min walk test (6MWT), and compared to predicted normative values. At a mean follow-up of 5 years, forced volume capacity (FVC), forced expiratory volume (FEV1), vital capacity (VC), and total lung capacity (TLC) were significantly lower than predicted (p < 0.05). FEV1/FVC, residual volume (RV), and forced residual capacity (FRC) were not significantly different. All patients completed the 6MWT (> 300 m), though post-exercise oxygen saturation declined significantly (p = 0.011). Diaphragmatic plication leads to a restrictive pattern and exercise-induced desaturation despite preserved walking capacity.

PMID:41049032 | PMC:PMC12490969 | DOI:10.1002/rcr2.70369

Circ Genom Precis Med. 2025 Oct 6:e005192. doi: 10.1161/CIRCGEN.125.005192. Online ahead of print.

ABSTRACT

BACKGROUND: Hypertrophic cardiomyopathy (HCM) is a relatively rare but debilitating diagnosis in the pediatric population, and patients with end-stage HCM require heart transplantation. Here, we have examined the transcriptome in ventricular tissue from this patient group to identify cell states and underlying cellular processes unique to pediatric HCM.

METHODS: We performed single-nucleus RNA sequencing on explanted hearts at transplant in 3 pediatric patients with end-stage HCM and compared findings to pediatric control and adult HCM.

RESULTS: We identified distinct underlying cellular processes in cardiomyocytes, fibroblasts, endothelial cells, and myeloid cells compared with controls. Pediatric HCM was enriched in cardiomyocytes exhibiting stressed myocardium gene signatures and underlying pathways associated with cardiac hypertrophy; cardiac fibroblasts exhibited activation signatures and compared with adult patients, exhibited heightened downstream processes associated with fibrosis and a unique, myofibroblast-like cluster with increased metabolic stress and antiapoptotic properties. We noted depletion of tissue-resident macrophages and increased vascular remodeling in endothelial cells in pediatric HCM.

CONCLUSIONS: Our analysis provides the first single-nucleus analysis focused on end-stage pediatric HCM. Fibroblast-mediated cellular processes were the most prominent in pediatric HCM, which had more downstream processes associated with fibrosis than did adult HCM.

PMID:41048033 | DOI:10.1161/CIRCGEN.125.005192

Ann Thorac Surg. 2025 Oct 2:S0003-4975(25)00907-5. doi: 10.1016/j.athoracsur.2025.08.061. Online ahead of print.

ABSTRACT

BACKGROUND: Surgical outcomes in neonates with complex congenital heart disease (CHD) and pulmonary over-circulation (POC) initially palliated with pulmonary flow reducers (PFR) were evaluated.

METHODS: Retrospective study between Jan 2020 - 2024. Eleven neonates underwent PFR palliation. Fenestrated Medtronic microvascular plugs™ (PFR) were delivered into branch pulmonary artery (PA). Target surgical procedure (TSP) involved the removal of PFR and biventricular (BiV) repair (9) or single ventricle (SV) palliation (2).

RESULTS: Patients were deemed high risk based on prematurity (5), early gestational age (5), heterotaxy (1), hypoxic-ischemic encephalopathy (1), chromosomal abnormality (4), or complex intracardiac anatomy (4). Median age and weight at PFR implantation (PFRI): 48 days (IQR 16-85) and 2.8 kg (IQR 1.7-3.4), respectively. Median PFRI time: 84 days (IQR 33-202). Weight gain following PFRI, 20.31 versus 3.75 g/day prior. Complications of PFRI included POC in 2 patients [bilateral PFR migration (1) and embolization (1)], excessive flow restriction (1), PFR release failure (1), and malposition (1). All patients were bridged to TSP. Devices were surgically removed without need for PA reconstruction needed. There was 1 in-hospital mortality following targeted surgery with 9/11 patients achieving 1-year survival. One-year echocardiographic follow-up showed 3/22 PAs with mild stenosis.

CONCLUSIONS: Outcomes of high-risk neonates with complex CHD and POC are good following PFR palliation. Removal of PFR is feasible without PA stenosis.

PMID:41046011 | DOI:10.1016/j.athoracsur.2025.08.061

J Cardiol. 2025 Oct 2:S0914-5087(25)00249-7. doi: 10.1016/j.jjcc.2025.09.019. Online ahead of print.

ABSTRACT

BACKGROUND: The comparative clinical outcomes of homograft (HG) and handmade polytetrafluoroethylene (PTFE) tri-leaflet valved conduit for right ventricular outflow tract reconstruction (RVOTR) in children with cyanotic congenital heart diseases remain unclear.

METHODS: We reviewed 265 consecutive patients (aged <14 years old) with cyanotic congenital heart diseases who underwent surgical intervention from 2015 to 2025 in our center (HG n = 141, PTFE n = 124). Competing risk analysis was performed for perioperative mortality, early reintervention, and re-operative conduit replacement during follow-up.

RESULTS: There were five early deaths (1.9 %). Multivariate analysis revealed a significant relationship between early mortality and preoperative left ventricular ejection fraction [hazard ratio (HR), 0.879; 95 % confidence interval (CI), 0.777-0.993; p = 0.039]. During a median follow-up of 45 months (range, 1-96), six patients (2.3 %) had reintervention on pulmonary artery by percutaneous balloon pulmonary dilation in the early postoperative period (average 7.8 months). Compared with HG conduit, patients in the PTFE group demonstrated longer conduit durability (p = 0.013) and lower reoperation rate (p = 0.049) during follow-up. Overall, freedom from reoperation for conduit dysfunction was no different for both conduits (p = 0.037). Freedom from re-operative conduit replacement at 3 and 5 years in the PTFE group was 95.2 % and 91.8 %; 90.3 % and 81.2 % in the HG group, respectively without significant difference (p = 0.37). Multivariate analysis revealed a significant relationship between reoperation and cardiopulmonary bypass time (HR, 0.989; 95 % CI, 0.979-0.999; p = 0.036), conduit size/body surface area > 25.77 mm/m2 (HR, 0.152; 95 % CI, 0.038-0.614; p = 0.008) and PTFE conduit (HR, 0.350; 95 % CI, 0.127-0.964; p = 0.042). Patients aged >34 months were associated with longer freedom from reoperation (p = 0.013).

CONCLUSION: We demonstrate excellent outcomes for RVOTR in pediatric patients with cyanotic congenital heart diseases. The handmade PTFE tri-leaflet valved conduit showed better results for reoperation and conduit durability. Younger age showed worse survival without reoperation for both conduits.

PMID:41045961 | DOI:10.1016/j.jjcc.2025.09.019

Int J Surg Case Rep. 2025 Oct 2;136:111999. doi: 10.1016/j.ijscr.2025.111999. Online ahead of print.

ABSTRACT

INTRODUCTION AND SIGNIFICANCE: Congenital Infantile Fibrosarcoma (CIFS) is an exceptionally rare pediatric malignancy, representing approximately 10 % of all soft tissue cancers in young children. Its clinical manifestations vary according to tumor location. When tumors occur in unusual anatomical sites, symptoms may be misleading and delay proper diagnosis and treatment.

CASE PRESENTATION: We report an 11-month-old male with a month-long persistent dry cough and high fever unresponsive to antibiotics. Chest CT revealed a pleural mass with mild effusion. Thoracotomy achieved complete excision (R0). Histopathology showed low-grade fibrosarcoma; immunohistochemistry was positive for Vimentin and SMA, focally CD34, and negative for S100, Desmin, and Myogenin, with low Ki-67 (<3 %). ETV6-NTRK3 fusion testing was unavailable. The patient received six cycles of adjuvant VAC chemotherapy and tolerated treatment well, with only mild transient neutropenia.

CLINICAL DISCUSSION: Diagnosis depends on histopathological and immunohistochemical analysis, as CIFS mimics several other soft tissue sarcomas. Our review of medical literature found no prior cases of CIFS originating in the pleura, underlining the uniqueness of this case. Management followed a standard multidisciplinary approach consisting of complete surgical excision and adjuvant chemotherapy (VAC protocol). At 12-month follow-up, the patient remained recurrence-free.

CONCLUSION: Persistent pneumonia-like symptoms unresponsive to standard treatment in infants should prompt consideration of underlying malignancy. Early recognition and prompt management are essential for improving outcomes in such rare presentations.

PMID:41045685 | DOI:10.1016/j.ijscr.2025.111999

Am J Hematol. 2025 Oct 4. doi: 10.1002/ajh.70074. Online ahead of print.

ABSTRACT

Viscoelastic testing (VET) has evolved significantly since its inception in the mid-20th century, when it was first developed to guide transfusion strategies in trauma and surgical patients. Initially, VET technologies such as TEG and ROTEM assessed clot formation by measuring the mechanical resistance of a pin or piston within a blood sample. Recent advances have introduced automated, cartridge-based systems and novel detection methods-including resonance frequency and ultrasound-based sonorheometry-these new systems allow for more precise, rapid, and user-friendly assessment of clot dynamics at the point of care. VET is now indicated for a wide range of clinical scenarios where complex coagulopathy is anticipated, including trauma, cardiac surgery, liver transplantation, obstetric hemorrhage, and hematologic disorders such as DIC. Its use is expanding into new populations, including pediatric cardiac surgery, patients with inflammatory bowel disease, and those with COVID-19. However, VET remains limited in its ability to reliably detect therapeutic anticoagulants and certain congenital bleeding disorders, such as von Willebrand disease and deficiencies of protein C, S, and antithrombin. Technical limitations, including potential discrepancies between in vitro and in vivo clot formation, and lack of FDA approval for pediatric use have imposed implementation barriers to centers interested in pediatric VET. Looking forward, the integration of VET data with electronic medical records, the development of predictive models, artificial intelligence, and continued innovation in platelet function assessment and detection technologies are poised to enhance the clinical utility of VET. As guidelines and evidence continue to evolve, VET is positioned to become an increasingly important tool for real-time, individualized management of coagulopathy in diverse patient populations.

PMID:41045051 | DOI:10.1002/ajh.70074

Perfusion. 2025 Oct 3:2676591251386601. doi: 10.1177/02676591251386601. Online ahead of print.

ABSTRACT

BackgroundMaintaining optimal anesthetic depth during cardiopulmonary bypass (CPB) in pediatric patients is challenging due to altered physiology and unreliable conventional monitoring. Entropy, a processed electroencephalogram metric, offers a potential solution. This study aimed to evaluate the relationship between end oxygenator sevoflurane concentration and entropy values during pediatric CPB using fixed-dose versus entropy-guided sevoflurane administration.MethodologyA prospective, randomized study was conducted on 62 pediatric patients undergoing congenital heart surgery with CPB. Patients were allocated into two groups: Group A received fixed-dose sevoflurane (1% v/v), and Group B received sevoflurane titrated to maintain entropy values between 40 and 60. Parameters such as end oxygenator sevoflurane concentration, entropy (Response and State entropy, RE and SE), and sevoflurane consumption were recorded intraoperatively. Postoperative hemodynamic data, length of stay, and complication rates were assessed.ResultsEntropy-guided patients showed significantly higher end oxygenator sevoflurane concentrations [1.64 (1.51-1.85)% versus 1.0%, p = .001] and sevoflurane consumption (1.26 ± 0.12 vs 0.645 ± 0.03 mL/min, p = .001). RE and SE values were significantly lower in the entropy group (p = .001), indicating better anesthetic depth control. A negative correlation was found between entropy and sevoflurane concentration (r = -0.6987, p = .02). Despite higher postoperative inotropic scores in the entropy group (p = .001), no significant differences were found in length of stay, mechanical ventilation duration, or morbidity and mortality rates between groups.ConclusionEntropy-guided sevoflurane administration during pediatric CPB provides improved anesthetic depth control at the cost of higher anesthetic and inotropic requirements. However, it does not adversely affect clinical outcomes, supporting its safety and potential utility in refining pediatric anesthesia practices.

PMID:41043805 | DOI:10.1177/02676591251386601

Anesthesiology. 2025 Oct 3. doi: 10.1097/ALN.0000000000005783. Online ahead of print.

ABSTRACT

INTRODUCTION: Patients with congenital heart disease (CHD) remain at high risk for morbidity and mortality when undergoing noncardiac procedures. Existing studies have utilized national databases focusing on mortality or are limited by small data sets from single institutions. Through a multi-institutional registry study, the primary aim is to describe the incidence of intraoperative cardiac events in patients with CHD undergoing noncardiac procedures. The secondary aim is to describe the risk factors associated with the events.

METHODS: Patients with CHD from birth to 21 years undergoing noncardiac procedures between January and December 2021, were identified at all participating centers. The primary outcome was occurrence of an intraoperative cardiac event at each encounter, defined as the composite of intraoperative hemodynamic instability, cardiac arrest, and pulmonary hypertensive crisis.

RESULTS: The final analysis involved 4,343 unique patients at 7 centers undergoing 6455 procedures. Among the cohort, 335 of 6455 procedures involved an intraoperative cardiac event (5.2%) in 296 unique patients. The most common event was hypotension (n=315, 4.9%); there were 12 occurrences of cardiac arrest (0.2%). Univariate analysis showed multiple factors associated with the increased likelihood of an intraoperative cardiac events including patient and procedure characteristics, cardiac disease, and anesthetic management. Examples of patient characteristics include prematurity (OR1.34,95%CI 1.02-1.76, P=0.038), chronic medical conditions (gastrointestinal (OR1.51, 95%CI 1.15-1.99,P=0.003), respiratory (OR2.12, 95%CI 1.62,2.76,P<0.001)), preoperative ventilatory support (OR3.88, 95%CI2.8,5.38, P<0.001), concurrent respiratory illness (OR 2.18, 95% CI 1.47,3.2, P<0.001), major cardiac disease (OR2.09, 95%CI 1.54,2.83, P<0.001), and severe CHD (OR3.48, 95%CI 2.47,4.91, P<0.001).

DISCUSSION: Pediatric patients with severe CHD, those undergoing emergency procedures, and those undergoing surgical interventions were at higher risk of not only mortality as shown previously, but also hemodynamic instability. These are established risk factors for intraoperative cardiac events and should be considered when planning risk mitigation strategies.

PMID:41043169 | DOI:10.1097/ALN.0000000000005783