Cirugía congénitos

J Endovasc Ther. 2025 Jun 21:15266028251344541. doi: 10.1177/15266028251344541. Online ahead of print.

ABSTRACT

INTRODUCTION: The use of intravascular catheters has become increasingly widespread in children, due to their use in diagnostic procedures (such as coronary, intracardiac, cerebral, and renal angiography, as well as pressure monitoring) and for therapeutic purposes (including angioplasties, valvuloplasties, congenital defect closure, chemotherapy, among other uses). However, they are not free from complications, which may include catheter fracture and migration within the cardiovascular system, potentially leading to vascular or cavity perforation, arrhythmias, and even death.

OBJECTIVE: To define the clinical and hemodynamic characteristics of pediatric patients undergoing catheterization for the retrieval of intravascular foreign bodies.

MATERIALS AND METHODS: A retrospective cohort study of all patients under 18 years of age who underwent endovascular extraction of foreign bodies at a cardiovascular reference center.

RESULTS: A high percentage of successful retrieval of intravascular foreign bodies was noted, with the most frequently retrieved catheter being the chemotherapy catheter, primarily located in the right atrium, between the pulmonary trunk and the right ventricle, and in the brachiocephalic vein. Few secondary complications were observed, occurring in only 2 patients.

CONCLUSION: Endovascular retrieval of foreign bodies is a highly effective procedure, and complications are relatively low in pediatrics, even during the neonatal period and in low-birth-weight cases, making it a preferable alternative to surgical extraction.Clinical ImpactBy documenting favorable outcomes across a diverse group, it encourages clinicians to adopt catheter-based approaches more confidently. The use of tools like snare loops and balloon-tipped guidewires in this population reflects procedural innovation and adaptability. Early, minimally invasive intervention may reduce morbidity and hospital stay. These findings can influence clinical decision-making and protocol development in pediatric cardiovascular care, particularly in centers equipped for interventional procedures.

PMID:40542822 | DOI:10.1177/15266028251344541

Sci Rep. 2025 Jun 20;15(1):20140. doi: 10.1038/s41598-025-04927-z.

ABSTRACT

Due to the increasing focus on neurodevelopment in children with congenital heart disease (CHD), early predictive markers are crucial for implementing interventions and improving neurodevelopmental outcomes. As postoperative delirium (PD) is known to have a long-term impact on neurocognitive function in adults, studies on the prevalence of and modifiable risk factors for PD offer new perspectives. We conducted a retrospective, single-centre study screening for PD using the Cornell Assessment of Pediatric Delirium (CAPD). We distinguished PD from iatrogenic withdrawal syndrome (IWS) by using the Withdrawal Assessment Tool 1 (WAT-1). A confirmatory, multivariate regression analysis was performed and included various pre-, intra-, and postoperative variables. The screening compliance rate was 95% among the 311 patients. The prevalence of PD was 40.2%, and 46.4% of the patients developed IWS. Infants were at the highest risk for PD (OR 2.9, p = 0.05). Prolonged mechanical ventilation > 100 h (OR 7.4, p = 0.003), infusion therapy with ketamine (OR 3.3, p = 0.009), IWS (mild: OR 7.7, p = < 0.001, severe: OR 17.0, p = < 0.001) and low cardiac output syndrome (LCOS) (OR 3.9, p = 0.02) were significant predictive risk factors for PD. Overall, PD and IWS are highly prevalent in paediatric cardiac intensive care units (pCICUs), especially in infants and children with prolonged ventilation durations who require multiple sedatives. This is one of the most extensive single-centre studies in the pCICU population, and the results revealed that IWS and lactatemia in the context of LCOS are novel predictors of PD.

PMID:40542017 | PMC:PMC12181358 | DOI:10.1038/s41598-025-04927-z

J Pediatr Surg. 2025 Jun 18:162420. doi: 10.1016/j.jpedsurg.2025.162420. Online ahead of print.

ABSTRACT

BACKGROUND: Currarino syndrome is a rare congenital condition characterized by a triad of anorectal malformation, sacral agenesis, and presacral mass, often a teratoma. Comparative outcomes of sacrococcygeal teratomas (SCTs) in Currarino versus non-syndromic cases are not well defined.

METHODS: A multicenter retrospective review of pediatric SCT resections from 2010-2020 was conducted across 11 institutions in the Midwest Pediatric Surgery Consortium. Patients were classified based on the presence or absence of Currarino syndrome. Demographic, surgical, pathologic, and long-term outcome data were analyzed. The primary outcome was tumor recurrence.

RESULTS: Of 203 patients, 25 (12.3%) had Currarino syndrome. Currarino patients were more often diagnosed postnatally (80% vs. 25%, p<0.001) and had predominantly Altman type IV tumors (87% vs. 17%, p<0.001). All tumors in the Currarino cohort were mature teratomas, while 27% of non-Currarino tumors were immature and 12% were malignant (p<0.001). Tumors in Currarino patients were significantly smaller in size (median 3.3 cm vs. 8.0 cm, p<0.001). Recurrence rates were low and comparable (4% Currarino vs. 10% non-Currarino, p=0.18). Currarino patients had higher rates of urinary incontinence (44% vs. 28%, p=0.048) and constipation (76% vs. 32%, p<0.001). Kaplan-Meier analysis showed a trend toward improved recurrence-free survival in Currarino patients, though not statistically significant.

CONCLUSIONS: Pediatric patients with Currarino-associated SCTs have excellent long-term outcomes, with low recurrence rates likely attributable to benign tumor histology and high rates of complete resection. These findings suggest that surveillance strategies may be tailored for Currarino patients to reduce unnecessary imaging and long-term follow-up burden.

STUDY DESIGN: Retrospective cohort study Level of Evidence: III.

PMID:40541662 | DOI:10.1016/j.jpedsurg.2025.162420

JACC Case Rep. 2025 Jun 18;30(15):103787. doi: 10.1016/j.jaccas.2025.103787.

ABSTRACT

Coronary artery fistulae (CAF) are rare aberrant connections between coronary arteries and the great vessels, coronary sinus, or cardiac chambers. Although most CAF are asymptomatic, patients with large, hemodynamically significant, or symptomatic fistulae might benefit from closure. Transcatheter occlusion is usually the preferred approach given that it avoids a sternotomy and cardiopulmonary bypass. However, there remain CAF with challenging features such as tortuous anatomy, large aneurysms, and concomitant cardiac disease that benefit from surgical ligation. We present 3 cases of CAF, all of which were treated by surgical ligation because of their hemodynamic and clinical significance, tortuosity, and massively dilated aneurysms. These cases illustrate the heterogeneous presentations of CAF and the varied open approaches to closure: epicardial versus intracardiac closure and with or without ligation of the involved coronary artery and distal revascularization. We present high-resolution 3-dimensional reconstructions of these impressively large fistulae and discuss the need for surgical consideration in similar cases.

PMID:40541346 | DOI:10.1016/j.jaccas.2025.103787

Pediatr Investig. 2025 Jan 3;9(2):181-184. doi: 10.1002/ped4.12459. eCollection 2025 Jun.

ABSTRACT

We recently came across an outbreak of parvovirus B19 related acute myocarditis in preschool children in a specific region in northern Greece. The children exhibited serious morbidity and mortality. All children had very poor left ventricular ejection fraction and required inotropic intensive care unit support.

PMID:40539004 | PMC:PMC12175628 | DOI:10.1002/ped4.12459

Ochsner J. 2025 Summer;25(2):116-118. doi: 10.31486/toj.24.0109.

ABSTRACT

BACKGROUND: The development of cardiac complications is common in patients with muscular dystrophy. However, advanced heart failure therapies such as implantation of durable ventricular assist devices and transplant are infrequently used in patients who develop cardiomyopathy, often because of comorbid impairments in mobility and respiratory function.

CASE REPORT: A 16-year-old male with limb-girdle muscular dystrophy type R4 presented with chronic decompensated heart failure. Recent worsening of his respiratory function and mobility were confounded by severe heart failure. In addition to our core advanced heart failure team, multidisciplinary assessment preoperatively included Neurology, Pulmonology, Genetics, and Physical Medicine and Rehabilitation. The patient underwent implantation of a HeartMate 3 left ventricular assist device and had an uneventful postoperative course. After intensive inpatient physical and occupational therapy, he was discharged home on postoperative day 16 with minimal residual heart failure symptoms and plans to continue robust outpatient physical therapy.

CONCLUSION: Patients with muscular dystrophy often have cardiac involvement; however, certain subtypes of muscular dystrophy are associated with an earlier presentation of severe life-limiting cardiomyopathy. Pediatric patients with muscular dystrophy should be considered for advanced heart failure therapies such as implantation of a durable left ventricular assist device at an appropriate center. Carefully selected patients may experience substantial improvements in their quality of life. Given the variable disease progression and life expectancy of patients with subtypes of muscular dystrophy, a thorough assessment by a multidisciplinary team is critical.

PMID:40538608 | PMC:PMC12175756 | DOI:10.31486/toj.24.0109

Innovations (Phila). 2025 Jun 19:15569845251337720. doi: 10.1177/15569845251337720. Online ahead of print.

ABSTRACT

OBJECTIVE: Partial heart transplantation (PHT) is a new procedure that delivers growing heart valve implants for children. However, awareness, attitudes, and perceptions of health care professionals regarding PHT remain unexplored.

METHODS: A national survey was conducted among members of the Congenital Heart Surgical Society, pediatric cardiac intensive care unit (ICU) directors, medical students, and organ procurement organization (OPO) representatives. The survey measured their awareness, perceptions, and attitudes toward PHT. Perceptions and attitudes were measured using a 5-point Likert scale. Statistical comparisons in ranked responses between survey questions were calculated using two-way analysis of variance, with multiple comparisons assessed by a Tukey post hoc test.

RESULTS: There were responses from 95 medical students (12.1%), 32 congenital cardiac surgeons (10.26%), 21 pediatric ICU directors (16.8%), and representatives from 8 OPOs (15%). Prior to survey distribution, 20% of students were aware of PHT. In contrast, almost all congenital heart surgeons (96.88%) and pediatric cardiologists (100%) were aware of PHT. Although surgeons and cardiologists understand the concepts of PHT, cardiologists were less likely to recommend and inform their patients about the procedure if they meet the criteria (Likert scale scores of 4.68 vs 3.14, P = 0.01 and 4.38 vs 3.69, P = 0.01, respectively). Surgeon and cardiologist perceptions regarding the use of PHT for different patient age groups were significantly different (P < 0.001).

CONCLUSIONS: Even though PHT is a relatively recent innovation, it is well known among pediatric cardiac surgeons and pediatric intensive care directors.

PMID:40538051 | DOI:10.1177/15569845251337720

Eur J Pediatr. 2025 Jun 20;184(7):430. doi: 10.1007/s00431-025-06274-7.

ABSTRACT

This systematic review and meta-analysis aimed to evaluate the clinical outcomes of cardiac surgery in neonates with trisomy 13 (T13) or trisomy 18 (T18) compared to those managed with palliative care. A literature search was conducted in PubMed® and EMBASE®, following PRISMA guidelines, and included five retrospective cohort studies (1627 patients). Outcomes analyzed included in-hospital mortality, survival at 12 months, length of stay (LOS), hospital discharge rates, and the need for mechanical ventilation. The meta-analysis showed that cardiac surgery significantly reduced the odds of in-hospital mortality (OR 0.12, CI 95% 0.03-0.42, p < 0.01), increased survival at 12 months (OR 19.77, CI 95% 5.12-76.36, p < 0.01), and improved discharge rates (OR 12.53, CI 95% 3.63-43.22, p < 0.01). However, limited data were available on quality of life and mechanical ventilation duration. Conclusion: Despite the positive impact of cardiac surgery on survival and discharge rates, the evidence remains low quality, as the included studies were primarily retrospective cohorts with moderate risk of bias. The findings highlight the importance of involving families in the decision-making process, given their differing perspectives on quality of life. Further high-quality studies, such as randomized controlled trials, are needed to provide stronger evidence on this topic.

PMID:40537699 | DOI:10.1007/s00431-025-06274-7

Cardiol Young. 2025 May;35(5):1082-1084. doi: 10.1017/S1047951125001751. Epub 2025 Jun 20.

ABSTRACT

Staged Fontan pathway treatment is a recognised surgical approach for managing congenital heart lesions with single ventricle physiology. Some patients necessitate communication between the tunnel and the atrium to maintain circulatory balance. During follow-up, adjustments to fenestration size may be required. While methods for enlarging or completely closing fenestrations are common, partially reducing flow through a fenestration remains challenging. We present an effective technique for partially reducing the size of a large, stented fenestration using a coronary stent and a vascular occluder.

PMID:40537442 | DOI:10.1017/S1047951125001751

BMC Anesthesiol. 2025 Jun 19;25(1):296. doi: 10.1186/s12871-025-03168-x.

ABSTRACT

BACKGROUND: The aim of this study was to develop a risk calculation model for peri-operative 30-day-mortality in preterm infants in non-cardiac surgery.

METHODS: Retrospective monocentric follow-up cohort-study of 27,453 pediatric anesthesias at a German university hospital and level one perinatal center between 2008 and 2021 for non-cardiac surgeries. Inclusion criteria were age < 37 post-menstrual weeks at the time of surgery. The primary endpoint was 30-day-mortality after surgery. For statistical analysis, stepwise backwards logistic regressions were performed to identify predictors for 30-day mortality after surgery.

RESULTS: Between 2007 and 2021, 278 preterm infants underwent surgery. The 30-day-mortality was 8.6% (24/278; CI95%:5.6–12.6). A preselection of potential risk factors was based primarily on prior knowledge available from the literature and the results of previously published studies. The final prediction model using a multivariable logistic regression revealed lower post-menstrual age (odds-ratio(OR): 0.67; CI95%: 0.54–0.83) and lower body weight at the time of surgery for extremely preterm infants (OR: 0.024; CI95%: 0.003–0.22), administration of dopamine or norepinephrine or epinephrine (OR: 11.6; CI95%: 3.58–37.7), and life-threatening emergencies between 10pm-7am (OR: 10.1; CI95%: 2.36–43.5) as significant independent risk factors for 30-day-mortality. The Area-Under-The-Receiver-Operating-Characteristic-Curve (0.90; CI95%: 0.85–0.96) showed a good discrimination of the final model. The investigation of the calibration curve (p = 0.99, Spiegelhalter test) and the goodness of fit test (p = 0.85, Hosmer-Lemeshow test) indicated no significant discrepancies between estimated and observed probabilities for the peri-operative 30-day mortality.

CONCLUSIONS: Peri-operative 30-day-mortality of preterm infants during non-cardiac surgery is high. The prediction model with easily ascertainable factors as described could be a valuable tool for estimating 30-day-mortality in preterm infants and should be validated in larger populations.

SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12871-025-03168-x.

PMID:40537757 | PMC:PMC12180206 | DOI:10.1186/s12871-025-03168-x

Am J Transl Res. 2025 May 15;17(5):3546-3553. doi: 10.62347/SMEE7102. eCollection 2025.

ABSTRACT

INTRODUCTION: Spinal meningoceles are congenital anomalies characterized by meningeal herniation through vertebral defects, most commonly occurring in the lumbosacral region. Intrathoracic meningoceles are rare and are typically associated with neurofibromatosis type 1 (NF-1). True anterior thoracic meningoceles unassociated with NF-1 represent exceptionally rare clinical entities.

CASE SUMMARY: This is a retrospective case report. We report a 15-year-old female with severe kyphoscoliosis and a non-NF-1-associated anterior thoracic meningocele who presented with progressive spastic paraparesis. Imaging examinations revealed thoracolumbar scoliosis with 100-degree kyphotic angulation, midline anterior spina bifida at T7, and a well-defined cystic structure exhibiting hypointense T1 and hyperintense T2 signals (measuring 5.7×4.5 cm) anterior to the T7-T8 vertebral bodies. Surgical intervention included posterior spinal laminar decompression, T7 vertebral osteotomy, microscopic dural sac reduction, and stabilization using a pedicle screw system with autologous bone grafting. The patient was discharged without any complications 12 days after surgery, and at the 12-month follow-up, the patient achieved ambulation with a walker and leg brace.

CONCLUSION: This case underscores the necessity of tailored surgical strategies for anterior thoracic meningoceles complicated by severe spinal deformities. The integration of microsurgical techniques and 3D-printed anatomical modeling may optimize procedural safety and functional outcomes.

PMID:40535678 | PMC:PMC12170365 | DOI:10.62347/SMEE7102

J Pediatr Surg Case Rep. 2025 May;116:102989. doi: 10.1016/j.epsc.2025.102989. Epub 2025 Mar 21.

ABSTRACT

INTRODUCTION: Lipomas are typically slow-growing tumors with the highest incidence in the fourth through sixth decades of life, less commonly occurring in pediatric patients, particularly extending across multiple body compartments. Excision is often reserved for cases that cause cosmetic or compressive symptoms and, due to the slow growth pattern, is less likely to be necessary in younger patients.

CASE PRESENTATION: A 2-year-old female with congenital albinism presented with a painless but visible 3 × 4 cm non-mobile left lateral neck mass that had been present for 2 months. MRI without contrast demonstrated a lobulated lesion in the left inferolateral supraclavicular region extending into the left thoracic inlet and thoracic apex. Due to concern for developing mass effect on carotid space structures, resection of the mass was performed. Complete excision was achieved through a single lower cervical incision, requiring dissection from the brachial plexus, carotid sheath, subclavian vessels, and extrapleural thoracic apex. Final pathology revealed an adipocytic neoplasm consistent with a lipoma with a total specimen size measuring 9 × 6.5 × 5 cm. The patient was discharged on postoperative day one and was healing well without complaint at the time of follow-up.

CONCLUSION: Despite their benign nature, cases such as the one presented here demonstrate the capability of lipomas to adhere to and involve surrounding critical structures, particularly when located in the cervicothoracic region and occurring in younger patients. However, with careful surgical planning involving cross-sectional imaging, such lesions can successfully be completely resected through a single cervical incision without morbidity.

PMID:40535308 | PMC:PMC12176381 | DOI:10.1016/j.epsc.2025.102989