Cirugía congénitos

Clin Imaging. 2025 Sep 26;127:110627. doi: 10.1016/j.clinimag.2025.110627. Online ahead of print.

ABSTRACT

OBJECTIVE: To describe the lymphatic imaging findings in patients with both Trisomy 21 and congenital heart disease and to depict the most common central lymphatic abnormalities in these patients.

MATERIALS AND METHODS: We conducted a single-center retrospective review of patients with a confirmed history of Trisomy 21 and congenital heart disease who presented for lymphatic imaging over a 7-year period. Clinical history and outcomes were extracted from the medical records. Lymphatic distribution and flow were evaluated by two pediatric radiologists from dynamic contrast-enhanced MR lymphangiography studies, which included T2-weighted and dynamic contrast-enhanced T1-weighted images, conventional lymphangiography studies were also evaluated.

RESULTS: We identified 16 patients (12 male): 8 infants, 6 children, and 2 adults. 12 patients had cardiac surgery including Fontan (n = 5), hemi-Fontan (n = 1), Glenn (n = 1), and other cardiac surgeries (n = 5). Presenting symptoms included chylothorax, plastic bronchitis, chylous ascites, protein losing enteropathy, pericardial effusion, lymphedema, and anasarca. Two patients died (12 %) by the time of data collection. T2-weighted MR imaging demonstrated lymphatic edema in all patients. T1-weighted dynamic imaging revealed abnormal pulmonary and/or mesenteric lymphatic perfusion in 15 patients (88 %) across intranodal, intrahepatic, and intramesenteric access types. The thoracic duct was tortuous and/or dilated in most cases. Conventional lymphangiography confirmed thoracic duct obstruction.

CONCLUSION: This is a descriptive study of central lymphatic diseases in patients with Trisomy 21, congenital heart disease and clinical evidence of lymphatic dysfunction. Common findings in these patients include retrograde dermal lymphatic flow, pulmonary and mesenteric lymphatic perfusion as well as the presence of a dilated and tortuous duct.

PMID:41027150 | DOI:10.1016/j.clinimag.2025.110627

Dimens Crit Care Nurs. 2025 Nov-Dec 01;44(6):313-319. doi: 10.1097/DCC.0000000000000723.

ABSTRACT

BACKGROUND: The Complexity Assessment and Monitoring to Ensure Optimal Outcomes (CAMEO) acuity tool was developed to inform nurse staffing decisions and projections. We propose a novel use of the CAMEO data, ie, CAMEO Acuity Curves, to identify high-acuity periods after congenital heart surgery.

OBJECTIVES: The aims of this study were to describe CAMEO acuity scores for selected congenital heart surgeries and examine acuity trends during the cardiac intensive care unit (CICU) admissions.

METHODS: We performed a retrospective review of 3 common congenital heart procedures between January 2017 and January 2020: stage I palliation for single ventricle (stage I), Arterial Switch Operation (ASO) for dextro transposition of great arteries with or without ventricular septal defect repair (ASO), and tetralogy of Fallot repair. Postoperative acuity was assessed using CAMEO Acuity Curves stratified by severity of residual lesions in anatomic area of repair as assessed by the Residual Lesion Score (RLS).

RESULTS: Among 205 cases (stage I, n = 45; ASO, n = 60; tetralogy of Fallot repair, n = 100), the overall mean CAMEO scores varied in acuity by procedure, with the highest acuity during cardiac intensive care unit stay noted for stage I (76%), followed by ASO (69%) and tetralogy of Fallot repair (62%). When stratified by RLS, acuity increased for each procedure, with RLS 3 having a higher acuity than RLS 1 and 2.

DISCUSSION: The CAMEO Acuity Curves enabled construction of postoperative acuity timeline in cardiac intensive care unit, allowing description and comparison of acuity across various case complexities, which may guide clinical decision-making and optimize staffing to meet patient care resource needs.

PMID:41026053 | DOI:10.1097/DCC.0000000000000723

Turk Kardiyol Dern Ars. 2025 Sep 30. doi: 10.5543/tkda.2025.67916. Online ahead of print.

ABSTRACT

Double-inlet left ventricle (DILV) are rarely encountered congenital heart defects which are also known as single-ventricle defects and have a complex structure in which the blood exiting both atriums flows into a single ventricle, and they constitute approximately 1.5% of all congenital heart diseases. The 37-year-old female patient who did not have a history of cardiac disease visited our outpatient clinic for routine cardiological assessments. Transthoracic echocardiography (TTE) was performed upon hearing a 3/6 pansystolic murmur at the mesocardiac focus and a 3/6 systolic ejection murmur at the pulmonary focus on cardiac auscultation. The TTE of the patient revealed that the atrioventricular valves opened into one ventricular chamber in four-chamber apical imaging. There was no noticeable interventricular septum, while a rudimentary right ventricle and a ventricular septal defect (VSD) were observed. There was a gradient of 38 mmHg in the pulmonary valve. Mild-to-moderate pulmonary stenosis was present. In the transesophageal echocardiography (TEE), a rudimentary interventricular septum and the right ventricle were observed. Because the patient did not have any symptoms or cyanosis, the patient was given information about a potential need for occasional infective endocarditis prophylaxis and phlebotomy, and close medical follow-up was decided. A double-inlet left ventricle is known as a severe congenital heart anomaly that is typically symptomatically diagnosed in childhood and requires surgical intervention. However, in the literature, cases with an asymptomatic course reaching adulthood without surgical intervention, albeit very rare, have been reported.

PMID:41025493 | DOI:10.5543/tkda.2025.67916

Trials. 2025 Sep 29;26(1):374. doi: 10.1186/s13063-025-09067-3.

ABSTRACT

BACKGROUND: Opioids were considered the main analgesics for pain management during and after cardiac surgery. There are many complications associated with the use of opioids. Paravertebral block (PVB) is injecting Anaesthetics into the paravertebral space. We designed a randomised controlled trial to investigate whether PVB-based opioid-free general Anaesthesia, as compared to traditional low-dose opioid-based fast-track anaesthesia, can reduce opioid consumption within 24 h after thoracotomy incision cardiac surgery with cardiopulmonary bypass (CPB) in paediatric patients.

METHODS: This is a single-centre, single-blinded, randomised controlled trial with a 1:1 allocation ratio. Patients will be randomised into two groups (control group and PVB group); 20 children will be enrolled in this trial, with 10 subjects in each group. Block randomisation will be performed. Patients aged 1-6 years, with the diagnosis of atrial and/or ventricular septal deficient And scheduled for cardiac surgery via a right thoracotomic incision, will be eligible for enrolment. The primary outcome is opioid consumption during the first 24 h after surgery. The main secondary outcomes include the perioperative stress response, inflammatory level, and intraoperative haemodynamics.

DISCUSSION: This is the first randomised clinical study investigating opioid-free anaesthesia based on PVB for paediatric congenital thoracotomy surgery with CPB. If the OPTION trial proves that opioid-free anaesthesia based on PVB is safe for children undergoing thoracotomic cardiac surgery, we would be glad to provide an OPTION for the perioperative management of these children, especially in the era of ERAS.

TRIAL REGISTRATION: Chinese Clinical Trial Registry: ChiCTR2200066517 ( www.chictr.org.cn ), Registered on December 7, 2022.

PMID:41024258 | PMC:PMC12482201 | DOI:10.1186/s13063-025-09067-3

Respir Res. 2025 Sep 29;26(1):276. doi: 10.1186/s12931-025-03361-z.

ABSTRACT

BACKGROUND: The mechanism of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) remains poorly understood. Fibulin 5, a multifunctional extracellular matrix protein, was reinduced in balloon-injured vessels and may contribute to vascular remodelling. This study aimed to investigate the role and molecular mechanism of action of fibulin 5 in the pathogenesis of CHD-PAH.

METHODS & RESULTS: We found elevated fibulin 5 expression in pulmonary artery smooth muscle cells (PASMCs) from CHD-PAH patients and shunt-induced PAH rats. In vivo, downregulation of fibulin-5 expression by intratracheally delivered adeno-associated viral vectors prevents shunt-associated PAH and associated pulmonary artery remodeling. In vitro, fibulin 5 expression in human PASMCs (hPASMCs) was changed through transduction with lentiviruses. We found fibulin 5 overexpression enhanced TGF-β1-induced proliferation and migration, as assessed by EdU, cell count, and transwell assays. Western blotting showed altered expression of contractile and synthetic phenotype markers. These effects were reversed by the PI3K/AKT inhibitor LY294002, suggesting fibulin 5 acts through the TGF-β1/PI3K/AKT pathway.

CONCLUSIONS: Overall, our data provide new insights into the influence of fibulin 5 on the modification of hPASMC dysfunction and pulmonary artery remodelling in shunt-associated PAH.

PMID:41024156 | PMC:PMC12482218 | DOI:10.1186/s12931-025-03361-z

World J Pediatr Congenit Heart Surg. 2025 Sep 29:21501351251369435. doi: 10.1177/21501351251369435. Online ahead of print.

ABSTRACT

Artificial neochordae crafted from expanded polytetrafluoroethylene are commonly utilized in mitral valve repair. Yet their application in tricuspid valve repair, particularly in the pediatric demographic, remains relatively unexplored. Upon reviewing the available literature, we have identified pediatric patients with tricuspid valve regurgitation, primarily of congenital origin and diagnosed at birth, who underwent repair procedures involving artificial neochordal implantation. Postoperatively, trivial-to-mild tricuspid regurgitation was predominantly observed in most cases. Operative mortality appears to be low, but a reliable estimate of operative mortality rates, early failure, and reoperation was not possible due to incomplete data reporting in the studies reviewed. Moreover, long-term data are not available since in most reports, postoperative evaluation was obtained early after repair. Our review suggests that artificial neochordal repair of the tricuspid valve may be a feasible surgical option to prosthetic valve replacement in pediatric patients. However, comprehensive data and further late follow-up are needed to analyze long-term effectiveness and clinical outcomes.

PMID:41021658 | DOI:10.1177/21501351251369435

Eur Heart J Case Rep. 2025 Sep 2;9(9):ytaf424. doi: 10.1093/ehjcr/ytaf424. eCollection 2025 Sep.

ABSTRACT

BACKGROUND: It was in 1866 when Wilhelm Ebstein first described a rare case of a 19-year-old cyanotic patient with tricuspid valve regurgitation caused by a congenital malformation. Since then, Ebstein's anomaly (EA) has been recognized as a condition that exhibits a diverse clinical course with a formidable challenge for clinicians in terms of diagnosis, management, and prognosis.

CASE SUMMARY: We present a case series of four patients with EA, shedding light on the varied clinical manifestations, treatment approaches, and outcomes that underscore the multifaceted nature of this condition.

DISCUSSION: The management of EA requires a comprehensive and individualized approach in specialized centres. The cardiologists responsible for the long-term care of these patients are faced with various challenging 'dilemmas' in the clinical course of the disease at different stages. The evolving landscape of cardiology offers a promising avenue for improving the outcomes and quality of life of patients with EA.

PMID:41019305 | PMC:PMC12461249 | DOI:10.1093/ehjcr/ytaf424

J Vasc Surg Cases Innov Tech. 2025 Aug 21;11(6):101957. doi: 10.1016/j.jvscit.2025.101957. eCollection 2025 Dec.

ABSTRACT

Isolated unilateral absence of pulmonary artery is a rare congenital malformation and often asymptomatic in adults. However, aneurysm formation in the aortopulmonary collateral vessels carries a potential risk of life-threatening hemorrhage. This case report describes a 79-year-old woman who presented with rupture of a mediastinal collateral aneurysm associated with isolated unilateral absence of pulmonary artery. We discuss the clinical course, including vascular morphology changes, and subsequent endovascular treatment. Notably, this case demonstrates the rupture of a de novo collateral aneurysm rather than a preexisting one.

PMID:41018207 | PMC:PMC12475422 | DOI:10.1016/j.jvscit.2025.101957

Cardiol Young. 2025 Sep 29:1-6. doi: 10.1017/S1047951125109499. Online ahead of print.

ABSTRACT

BACKGROUND: CHD is a major risk factor for acute ischaemic stroke in paediatric patients due to endothelial changes from surgically manipulated vessels, prosthetic material, flow stasis in variable circulations, and hypercoagulability from chronic cyanosis. Stroke recognition in critically or chronically ill patients is challenging, yet rapid identification allows for mechanical thrombectomy to restore cerebral blood flow, particularly in those ineligible for thrombolysis or beyond its therapeutic window. We present a case series highlighting the importance of prompt stroke diagnosis and the role of mechanical thrombectomy in paediatric CHD patients, including children as young as four.

METHODS: We conducted a single-centre retrospective chart review of paediatric CHD patients who experienced thromboembolic stroke and underwent mechanical thrombectomy from July 2018 to March 2024. Data collected included age, stroke territory, maximum Paediatric NIH Stroke Scale (PedNIHSS) score, pre-thrombectomy neurological deficits, and post-thrombectomy outcomes using thrombolysis in cerebral infarction (TICI) scores.

RESULTS: Four CHD patients underwent mechanical thrombectomy for thromboembolic stroke (Table ). They exhibited diverse cardiac anatomies, including two-ventricle and single-ventricle physiology, with a wide age range at presentation.

CONCLUSION: Stroke presentation in CHD patients is variable, necessitating a high index of suspicion. Mechanical thrombectomy is safe and effective in patients as young as four, with no haemorrhagic complications in this series. Further research is needed to develop tailored stroke management guidelines for paediatric CHD patients, particularly younger children and those ineligible for thrombolysis.

PMID:41017363 | DOI:10.1017/S1047951125109499

Eur J Intern Med. 2025 Sep 26:106514. doi: 10.1016/j.ejim.2025.106514. Online ahead of print.

ABSTRACT

BACKGROUND: To provide information on adults with congenital heart disease (ACHD) undergoing non-cardiac surgeries (NCS), specific risk compared non-ACHD, independent risk factors for adverse outcome and mortality.

METHODS: Based on non-selective data including all in-hospital admissions in Germany from 2009 to 2021, all ACHD cases that underwent NCS were selected. NCS was categorized in low, medium and high-risk procedures. As primary endpoints, major adverse cardiovascular events (MACE), major infection (MIE), major bleeding (MBE), major thromboembolism (MTE), and in-hospital death were defined. Outcomes of ACHD were compared to a propensity score matched cohort of non-ACHD.

RESULTS: Overall, 15,349 inpatient ACHD cases were selected for analysis. Of those 72.3 % (n=11,094) were simple, 20.1 % (n=3,086) were moderate and 7.6 % (n=1,169) were complex ACHD. Patients with more than moderate ACHD faced a substantially higher risk for adverse outcome regarding all predefined endpoints compared to non-ACHD. Specifically, risk for MACE was increased with an Odds ratio (OR) of 1.29 (95 % CI 1.11-1.51) for moderate ACHD and OR 1.58 (95 % CI 1.23-2.02) for complex ACHD. In-hospital mortality was OR 1.39 (95 % CI 1.13-1.71) for moderate and OR 2.22 (95 % CI 1.62-3.03) for complex ACHD compared to non-ACHD.

CONCLUSIONS: Patients with more than moderate complexity ACHD are at specific risk for adverse outcomes when undergoing non-cardiac surgery. Further analyses are needed to give precise recommendations on the choice of appropriate surgical site as well as how to improve care and outcome of ACHD undergoing NCS.

PMID:41015714 | DOI:10.1016/j.ejim.2025.106514

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2025 Sep 26;60:1030-1039. doi: 10.3760/cma.j.cn115330-20250205-00081. Online ahead of print.

ABSTRACT

根据先天性耳畸形伴发的多发畸形发生率从高到低涉及的主要专科：耳鼻咽喉头颈外科、整形外科、颌面外科、心外科、骨科与神经外科、泌尿外科、眼科、口腔正畸科、普通外科、胸外科、超声科、心理科以及产前诊断等（14个专科），来自中国25家单位（医院）的资深专家（均为学科带头人、25年以上专科工作经验），通过反复研讨，结合各专科诊治经验及本领域的最新进展，提出各专科诊疗的最佳时机，再通过综合分析多发畸形对功能、外形、心理的影响程度进行序列安排，达成共识，形成最佳诊疗时机日历表及联合诊疗专家团队。从备孕期开始到出生后的不同时段，分别列出每个时段需要进行处理的常见畸形及就诊科室，并做相关说明，指导医生和患者及家属进行选择和规划；同时参与研讨的专家组成联合诊疗团队，实现内部直接推送患者，使其获得“一站式”高水平诊疗。.

PMID:41015439 | DOI:10.3760/cma.j.cn115330-20250205-00081

Am Heart J. 2025 Sep 25:S0002-8703(25)00336-9. doi: 10.1016/j.ahj.2025.09.014. Online ahead of print.

ABSTRACT

BACKGROUND: Prophylactic steroids are often used to reduce the systemic inflammatory response to cardiopulmonary bypass in infants undergoing heart surgery. The STRESS trial found that the odds of a worse outcome did not differ between infants randomized to methylprednisolone (n=599) versus placebo (n=601) (adjusted odds ratio [OR], 0.86; P=0.14). However, secondary analyses showed possible benefits with methylprednisolone. To investigate further using a different probabilistic approach, we re-analyzed the STRESS trial using Bayesian analytics.

METHODS: We used a covariate-adjusted proportional odds model using the original STRESS trial primary endpoint, a ranked composite of death, transplant, major complication and post-op length of stay. We performed Markov Chain Monte Carlo simulations to assess the probability of benefit (OR <1) versus harm (OR >1). Primary analysis assumed a neutral probability of benefit versus harm with weak prior belief strength (nearly non-informative prior distribution). To illustrate magnitude of effect, we calculated predicted risk of death, transplant or major complications for methylprednisolone and placebo. Sensitivity analyses evaluated pessimistic (5%-30% prior likelihood of benefit), neutral and optimistic (70%-95%) prior beliefs, and controlled strength of prior belief as weak (30% variance), moderate (15%) and strong (5%). A secondary analysis derived empirical priors using data from four previous steroid trials.

RESULTS: The posterior probability of any benefit from methylprednisolone was 92% and probability of harm was 8%. Composite death or major complication occurred in 18.8% of subjects with an absolute risk difference of -2% (95% CI -3%, +1%) for methylprednisolone. Each of 9 sensitivity analyses demonstrated greater probability of benefit than harm in the methylprednisolone group with 8 of 9 demonstrating >80% probability of benefit and ≥1% absolute difference in risk of death, transplant or major complications. In secondary analysis deriving priors from previous steroid trials, results were consistent with a 95% posterior probability of benefit.

CONCLUSION: Our Bayesian re-analysis of the STRESS trial, using a range of prior beliefs, demonstrated a high probability that perioperative methylprednisolone reduces the risk of death or major complications in infants undergoing cardiopulmonary bypass compared with placebo. This more in-depth analysis expands the initial clinical evaluation of methylprednisolone provided by the STRESS trial.

TRIAL REGISTRATION: Clinicaltrials.gov: NCT03229538 (https://clinicaltrials.gov/study/NCT03229538).

PMID:41015071 | DOI:10.1016/j.ahj.2025.09.014

Life (Basel). 2025 Sep 21;15(9):1482. doi: 10.3390/life15091482.

ABSTRACT

Anomalous origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare congenital coronary anomaly with an estimated incidence of 1 in 300,000 live births. While commonly diagnosed in infancy, adult presentations are exceedingly rare and pose significant diagnostic challenges. Delayed diagnosis may result in progressive myocardial ischemia, heart failure, arrhythmias, or sudden cardiac death. Surgical correction is the definitive treatment, with the goal of restoring a dual coronary artery system and preventing irreversible myocardial damage. We present the case of a 30-year-old male with a prior history of non-ST-elevation myocardial infarction who was referred for evaluation of exertional angina and symptoms of heart failure. Transthoracic echocardiography revealed a dilated left ventricle with an ejection fraction (LVEF) of 35%. Coronary angiography and cardiac MDCT identified an anomalous origin of the left circumflex artery (LCx) from the right pulmonary artery (RPA) and a coronary-pulmonary artery fistula involving the LAD. The patient underwent successful surgical correction with reimplantation of the LCx into the ascending aorta. Postoperative recovery was uneventful. At 3-month follow-up the patient was symptom-free, though echocardiography revealed persistent LV dilation and reduced LVEF, necessitating continued pharmacologic therapy and monitoring. This case highlights the importance of maintaining a high index of suspicion for ALCAPA in adult patients with unexplained cardiomyopathy or ischemic symptoms. Early diagnosis and surgical intervention remain crucial for improving long-term outcomes and preventing life-threatening complications.

PMID:41010424 | PMC:PMC12471381 | DOI:10.3390/life15091482

Animals (Basel). 2025 Sep 16;15(18):2717. doi: 10.3390/ani15182717.

ABSTRACT

A 10-month-old Poodle was presented with intermittent non-weight-bearing lameness of the left thoracic limb. Orthopedic and radiographic examinations revealed medial shoulder luxation and markedly reduced elbow extension. A two-stage surgical approach was performed. In the first stage, selective myotomy of periarticular structures, including the biceps brachii-brachialis complex and the extensor carpi radialis muscle, was conducted via medial and lateral approaches. A trans-articular external skeletal fixator was applied to maintain elbow extension. Elbow extension improved from 105° preoperatively to 142°. After confirming functional recovery of the elbow joint, the second stage involved shoulder arthrodesis using a circular osteotomy technique with a radial saw, which enabled fine-tuned intraoperative adjustment of limb alignment based on the contralateral limb posture. At nine months postoperatively, the patient exhibited a symmetrical gait, full weight-bearing, and no evidence of discomfort on range of motion assessment. This case highlights the clinical relevance of secondary elbow contracture associated with congenital shoulder instability and suggests that a combination of targeted muscle release and adjustable arthrodesis may offer favorable outcomes in managing complex joint dysfunction.

PMID:41007962 | PMC:PMC12466446 | DOI:10.3390/ani15182717

Children (Basel). 2025 Sep 16;12(9):1244. doi: 10.3390/children12091244.

ABSTRACT

Background/Objectives: Complex cases in pediatric surgery involving multiple congenital anomalies pose significant diagnostic and therapeutic challenges. These conditions require coordinated interdisciplinary care tailored to the individual patient. We present a case of syndromic congenital anomalies in a neonate, later diagnosed with CHARGE syndrome, to illustrate the importance of staged, multidisciplinary management. Methods: A 34-year-old woman in her third pregnancy developed significant polyhydramnios at 31 weeks of gestation, followed by preterm labor. The neonate presented with esophageal atresia with tracheoesophageal fistula (EA/TEF), intrathoracic stomach, aortic coarctation, patent ductus arteriosus, atrial septal defect, and bilateral choanal atresia. A structured treatment protocol was developed and implemented at Klinikum Stuttgart by an interdisciplinary team comprising gynecology, pediatric surgery, cardiology, ENT, neonatology, and genetics. Results: Initial pediatric surgical procedures included ligation of the tracheoesophageal fistula, repositioning of the intrathoracic stomach, and primary esophageal anastomosis. Cardiovascular anomalies were managed through staged interventions. Bilateral choanal atresia was surgically corrected. Genetic testing confirmed CHARGE syndrome. Postoperative care included respiratory support, enteral nutrition, and regular esophageal dilations. Due to persistent reflux esophagitis, antireflux surgery is planned. Conclusions: This case underscores the importance of a highly individualized and interdisciplinary approach in the management of syndromic congenital anomalies. The presence of CHARGE syndrome with multiple system involvement required careful staging of surgical interventions and long-term coordination of follow-up care. Early genetic diagnosis and integrated team planning were critical in optimizing outcomes in this complex neonatal case.

PMID:41007109 | PMC:PMC12468033 | DOI:10.3390/children12091244

Children (Basel). 2025 Sep 9;12(9):1202. doi: 10.3390/children12091202.

ABSTRACT

Three-dimensional (3D) modeling and printing technologies are increasingly used in pediatric surgery, offering improved anatomical visualization, surgical planning, and personalized approaches to complex conditions. Compared to standard imaging, patient-specific 3D models-virtual or printed-provide a more intuitive spatial understanding of congenital anomalies, tumors, and vascular anomalies. This review compiles evidence from pediatric surgical fields including oncology, abdominal, and thoracic surgery, highlighting the clinical relevance of 3D applications. The technological workflow-from image segmentation to computer-aided design (CAD) modeling and multimaterial printing-is described, emphasizing accuracy, reproducibility, and integration into hospital systems. Several clinical cases are presented: neuroblastoma, cloacal malformation, conjoined twins, and two cases of congenital diaphragmatic hernia (one with congenital pulmonary airway malformation, CPAM). In each, 3D modeling enhanced anatomical clarity, increased surgeon confidence, and supported safer intraoperative decision-making. Models also improved communication with families and enabled effective multidisciplinary planning. Despite these advantages, challenges remain, such as production time, cost variability, and lack of standardization. Future directions include artificial intelligence-based automation, expanded use of virtual and mixed reality, and prospective validation studies in pediatric cohorts. Overall, 3D modeling represents a significant advance in pediatric precision surgery, with growing evidence supporting its safety, clinical utility, and educational value.

PMID:41007067 | PMC:PMC12469229 | DOI:10.3390/children12091202

Children (Basel). 2025 Aug 27;12(9):1131. doi: 10.3390/children12091131.

ABSTRACT

BACKGROUND/OBJECTIVES: This study aimed to investigate the prevalence of liver damage and its associated non-invasive markers and echocardiographic risk factors in patients who underwent surgery for congenital heart disease.

METHODS: This retrospective observational study was conducted at a single tertiary-care university hospital in Niigata, Japan. Of 142 patients (ventricular septal defect [VSD] n = 47, tetralogy of Fallot [TOF] n = 67, Fontan n = 28), 52.8% were male [median age: 22.7 years; VSD (24.3 years), TOF (24.0 years), and Fontan (12.5 years)]. Pediatric patients with liver diseases unrelated to congestive liver disease, such as viral hepatitis and alcoholic liver disease, were excluded. We compared non-invasive liver fibrosis age-invariant biomarkers, such as the aspartate aminotransferase-to-platelet ratio index (APRI), and various serum markers and echocardiographic parameters to assess the prevalence and predictors of hepatic fibrosis.

RESULTS: The Fontan circulation group had the highest APRI, followed by the TOF group, while the VSD group had a low risk of APRI elevation. Postoperative TOF patients required monitoring for cirrhosis progression. Inferior vena cava mobility was associated with echocardiographic parameters and fibrosis severity, along with a loss of respiratory variability. The limitations of other cardiac assessments were highlighted by poor anatomical measurements. Gamma-glutamyl transpeptidase (γ-GTP) demonstrated strong discriminatory ability. The optimal cutoff value was 53.0 U/L, suggesting its use as a clinical marker.

CONCLUSIONS: Assessing fibrosis is crucial in CHD patients, especially those with late post-TOF repair findings. Non-invasive markers (APRI, γ-GTP, and B-type natriuretic peptide), along with echocardiographic findings, may help detect fibrosis early, enabling timely intervention and improving long-term outcomes.

CLINICAL TRIAL REGISTRATION: 2020-0199.

PMID:41006995 | PMC:PMC12468072 | DOI:10.3390/children12091131

Pediatr Cardiol. 2025 Sep 26. doi: 10.1007/s00246-025-04037-7. Online ahead of print.

ABSTRACT

Infective endocarditis (IE) is uncommon in children with congenital heart disease (CHD), and limited data exist regarding in-hospital outcomes of those requiring cardiac surgery. We retrospectively reviewed hospitalizations from the pediatric health information system from January 2016 to November 2024. Among 357,031 unique pediatric patients with CHD (≤ 21 years), 2108 patients (0.5%) were hospitalized for IE. The index hospitalizations during which cardiac surgery was performed were included. We aimed to characterize in-hospital outcomes among CHD children undergoing cardiac surgery for IE, with sub-analysis of those who required surgery versus medical therapy. The incidence of IE in CHD was 5.6 cases per 1000. Microorganism coding was unavailable in 41.3%; Staphylococcus (24.1%) and Streptococcus (22.3%) were the most common. Stroke occurred in 10.7%. Prosthetic valve endocarditis was present in 9.4%. Overall, 30% of patients required cardiac surgery, most commonly pulmonary valve or conduit procedures (36.6%) and tricuspid valve surgery (16.7%). Two-valve surgery was performed in 13.3%. In-hospital mortality was comparable between surgical and medical patients (10.1% vs 9.6%, p = 0.7). Permanent pacemaker implantation was more common after surgery (6.6% vs 2.2%, p < 0.01). Length of hospital stay and hospitalization costs were higher in the surgical group. On multivariable analysis, older age, heart failure, stroke, and extracorporeal membrane oxygenation (ECMO) were associated with in-hospital mortality. IE in children with CHD remains associated with substantial morbidity and mortality. In-hospital mortality remains high despite cardiac surgery. Older age, heart failure, stroke, and ECMO requirement are independent factors of in-hospital mortality.

PMID:41006584 | DOI:10.1007/s00246-025-04037-7

JACC Case Rep. 2025 Sep 24;30(29):105142. doi: 10.1016/j.jaccas.2025.105142.

ABSTRACT

BACKGROUND: Bioprosthetic valve failure is a significant and debilitating sequela in cardiac patients caused primarily by structural changes in valve leaflets due to endocarditis, calcification, and thrombosis.

CASE SUMMARY: We describe a rare case of bioprosthetic pulmonary valve failure due to an allergic reaction to molybdenum within the valve cage. We describe the clinical presentation, clinical methods leading to the diagnosis, and treatment progression until we could resolve symptoms and a significant return of valvular competency.

DISCUSSION: After excluding infectious etiologies as a cause, we used skin allergy testing to identify molybdenum as the allergic agent. The patient's symptoms and valve function improved and stabilized with immunosuppression and antihistamines.

TAKE-HOME MESSAGES: Allergy to a valve metal component was diagnosed with skin allergy and treated with immunosuppression and antihistamines.

PMID:41005850 | DOI:10.1016/j.jaccas.2025.105142

Int J Surg Case Rep. 2025 Sep 23;136:111980. doi: 10.1016/j.ijscr.2025.111980. Online ahead of print.

ABSTRACT

BACKGROUND: Right ventricle-to-pulmonary artery (RV-PA) reconstruction often requires a valved conduit; however, no single option is ideal across ages and anatomical variations. We report early outcomes of a bovine pericardial xenograft conduit (NeoCor) as a practical solution in a resource-limited setting.

METHODS: In this single-center retrospective series, eight pediatric patients (1-14 years) underwent RV-PA reconstruction (2019-2024) using a bovine pericardial xenograft conduit sized by nomogram (10-21 mm). A single surgical team performed all procedures under standardized perioperative protocols with intraoperative echocardiography. Complications were graded using standardized criteria. The primary outcome was clean follow-up (no mortality, no reintervention on the conduit/RVOT, no ≥moderate conduit regurgitation, peak Doppler gradient ≤30 mmHg, and no echogenic calcification).

RESULTS: Indications included TGA/VSD/PS (n = 4), tetralogy of Fallot with absent pulmonary valve (n = 2), VSD-PA with confluent PAs/PDA (n = 1), and truncus arteriosus redo (n = 1). Follow-up ranged 6 months-5 years (mean 2 years). All patients met the clean follow-up definition. Conduit Doppler gradients were 5-8 mmHg perioperatively and 8-12 mmHg at follow-up; no calcification or ≥ moderate regurgitation was observed.

CONCLUSIONS: A bovine pericardial xenograft conduit is a feasible, accessible option for RV-PA reconstruction with promising short-term hemodynamics in this context. Larger prospective studies and/or registry participation are warranted to evaluate durability, reintervention rates, and cost-effectiveness.

PMID:41005129 | DOI:10.1016/j.ijscr.2025.111980