World journal for pediatric & congenital heart surgery最新文献

Objective: Infants and children undergoing cardiac surgery are one of the highest-risk groups for thrombosis and its sequelae. We sought to define the current rate of and risk factors for postoperative central venous catheter (CVC)-associated deep vein thrombosis (CA-DVT) using the Pediatric Cardiac Critical Care Consortium (PC⁴) dataset. Design: Retrospective review of PC⁴ database from February 2019 to February 2022. Patients: Children <18 years of age admitted for a surgical encounter who had a CVC placed. Results: Included were 33,491 patient encounters, of whom 37.6% (12,582/33,491) were infants (<12 months of age). The overall CA-DVT rate was 2.5% (844/33,491), which varied widely among centers (0-11%). Multivariable analysis showed increased risk of CA-DVT with increasing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category (STAT 2 HR 1.8, CI [1.26-2.65]; STAT 3 HR 2.2, CI [1.56-3.39]; STAT 4 HR 2.1, CI [1.55-2.93]; STAT 5 HR 2.5, CI [1.69-3.82]), (P < .001 for all), low cardiac output syndrome (HR 1.5, CI [1.25-1.91]), P < .001, and postoperative arrhythmia (HR 1.23, CI [1.03-1.47]) P = .024). Patients with CA-DVT were less likely to have an internal jugular vein catheter or intracardiac line and more likely to have an umbilical venous catheter, femoral vein CVC, peripherally inserted CVC, and/or multiple CVCs. Conclusions: CA-DVT remains an important postoperative complication after pediatric cardiac surgery, with greatest risk of occurrence in the younger, smaller, more surgically complex by STAT category, and hemodynamically vulnerable patients. These risk factors must be considered when developing paradigms for CVC placement, thromboprophylaxis, and diagnosis/treatment of CA-DVT in the future.

Unilateral lung agenesis associated with congenital heart disease is very rare and the consequent malposition of the heart and mediastinal structures pose a technical challenge for definitive surgery. We describe a case of a three-month-old infant with right lung agenesis and a large ventricular septal defect who underwent successful surgical repair. The case highlights the importance of preoperative anatomic assessment and intraoperative adaption when presented with complex anatomy.

BackgroundDrawing on more than 30 years of valuable expertise in in-vitro, in-vivo, and clinical trials, we report the key findings of our institutional research on pulsatile cardiopulmonary bypass (CPB).MethodsA detailed search was performed in the National Institutes of Health National Library of Medicine, focusing on the PubMed database. This search combined key terms such as "Pulsatile flow," "Cardiopulmonary Bypass," "Undar A," "Vital Organ Injury," "Clinical outcomes," "Pediatrics," and "Neonates and infants" to find relevant peer-reviewed publications.ResultsA comprehensive review article was crafted by analyzing 88 peer-reviewed studies published between 1996 and 2025. This research provided substantial scientific evidence on optimizing neonatal and pediatric cardiopulmonary bypass (CPB) circuitry, focusing not only on pump performance but also on selecting appropriate oxygenators and arterial cannulas for effective pulsatile flow. While significant benefits regarding cerebral hemodynamics were observed in neonatal piglet models using pulsatile flow in conjunction with deep hypothermic circulatory arrest, the results from randomized clinical trials and retrospective studies raised questions regarding its efficacy. Despite some promising indications of improved Apolipoprotein E and plasminogen activator inhibitor-1/tissue plasminogen activator ratios, a rigorous randomized clinical trial involving 159 patients, along with a comprehensive retrospective study of 284 patients at Penn State Health Children's Hospital, demonstrated no significant clinical advantage of pulsatile flow compared with nonpulsatile flow. Importantly, while pulsatile flow did not provide a measurable benefit, it also did not result in any adverse outcomes in our clinical trials.ConclusionsThe existing evidence clearly indicates that pulsatile CPB does not provide significant short-term or long-term benefits for pediatric patients undergoing congenital heart surgery.

Background: Surgical repair of truncus arteriosus (TA) requires right ventricle-to-pulmonary artery continuity, achieved through either conduit-based (CB) reconstruction or a nonconduit-based (NCB) approach. As the optimal right ventricular outflow tract (RVOT) strategy is still debatable, we conducted a meta-analysis to compare outcomes. Methods: A systematic literature search was performed focusing on studies that compared CB and NCB RVOT reconstruction techniques for the primary correction of TA. Statistical analyses were conducted using RevMan 8.13.0, with effect estimates reported as odds ratios (ORs) and mean differences, along with 95% confidence intervals (CIs). Results: We included seven studies comprising 360 patients, with 174 neonates (48.3%) allocated to the CB group. No significant difference was observed in early mortality [OR 0.61; 95% CI 0.13-2.92; P = .43; I² = 43%] or long-term mortality [OR 1.22; 95% CI 0.09-17.23; P = .83; I² = 78%] between CB and NCB RVOT reconstruction techniques. However, the incidence of RVOT reoperation [OR 3.70; 95% CI 2.02-6.76; P = .003; I² = 0%] and the overall reintervention rate, including both surgical and catheter-based interventions, were higher in the CB cohort [OR 2.45; 95% CI 1.33-4.52; P = .01; I² = 0%]. Conclusion: While CB and NCB approaches yield similar early and long-term survival outcomes, CB reconstruction is associated with a significantly higher risk of RVOT reoperation and overall reintervention. These findings suggest that NCB approaches may offer superior long-term durability, reducing the need for subsequent interventions in neonates undergoing primary repair of TA.

Purpose: Some centers including ours have used glutaraldehyde-treated pericardium for reconstruction of coronary button defects at the arterial switch operation (ASO). This may impact the incidence of pulmonary stenosis and prevent primary closure of the pericardium. We report a series where 2-ply extracellular matrix (Tyke) was used to reconstruct the neopulmonary root. Methods: Since May 2016, all patients (n = 38) were repaired using Tyke for reconstruction of the coronary button defects. We measured the incidence of pulmonary stenosis and insufficiency at the main pulmonary artery (MPA) and branch pulmonary arteries by echocardiogram and compared these measurements with the historical cohort where pericardium (some treated with glutaraldehyde, some fresh) was used. Results: Mean follow-up was 3.0 ± 2.4 years (range, 1 month to 8 years). The mean gradient at the neopulmonary valve was 6.8 ± 7 mm Hg in the Tyke group versus 23.7 ± 16.1 in the pericardium group (P < .01). The mean gradient at the distal MPA was 8.9 ± 2.9 mm Hg for the Tyke group and 44.5 ± 40.1 in the historical group (P < .01). The mean gradient at the proximal left and right branch pulmonary arteries was 29 ± 19 and 32 ± 16 mm Hg in the Tyke group and 33 ± 16 and 36 ± 26 (NSD). At the last follow-up, six of 38 patients (18%) in the Tyke group had a pulmonary reintervention: five patients had a balloon dilation of branch pulmonary arteries, and one patient had dilation of the MPA. No patients underwent surgical intervention on the pulmonary valve or the reconstructed MPA. Conclusion: Tyke is a reasonable alternative material for reconstruction of the coronary button defects during the ASO within the follow-up period.

BackgroundAlthough recognized as common, the frequency of and reasons for cancellations of congenital heart disease surgery to our knowledge, has not been described previously.MethodsPatients with congenital heart disease scheduled for surgical repair from January of 2018 through December of 2023 were reviewed. For patients with multiple scheduled procedures, only the principal surgery was included. Subsequent noncardiac procedures were excluded. Patients' age at surgery and the Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) score (a measure of operative risk) were recorded. Cancellations were categorized into patient factors and institutional factors.ResultsA total of 729 cases were analyzed. Of these, 220 (30.2%) cases were canceled, including 146/220 (66.4%) cases for patient factors and 74/220 (33.6%) for institutional factors. The most common patient factor contributing to cancellation was a suspected or confirmed viral illness (69/146, 47.3%). The most common institutional factor was an urgent case taking priority (33/74, 44.6%). Cancellations were least common in neonates and STAT 5 procedures but were similar across all other age groups and STAT categories. Cancellations due to patient illness did not show a notable seasonal variation but increased after the COVID-19 pandemic. As surgical volume increased, cancellations related to institutional factors increased.ConclusionThe most common cause for cancellation of congenital heart disease surgeries is a viral illness. Although neonates and patients needing STAT 5 procedures were the least likely to be canceled, the rate of cancellation was similar across the other age and STAT categories. In a cohort including the COVID 19 pandemic, there was no obvious seasonal variation in cancellations.

The lysyl oxidase (LOX) gene encodes the extracellular enzyme lysyl oxidase, which is essential for cross-linking collagen and elastin, key structural proteins that maintain connective tissue stability. Pathogenic variants in the LOX gene are associated with familial thoracic aortic aneurysms. A five-year-old male presented to the emergency room with upper respiratory tract symptoms. A chest radiograph showed a markedly enlarged mediastinum. Further studies revealed a 9-cm thoracic aortic aneurysm confined to the ascending aorta. The child underwent successful surgical intervention.

ObjectivesTetralogy of Fallot (TOF) repair is associated with low mortality in high-performance centers, but outcomes are worse in low- and middle-income countries (LMICs). Prior studies have not demonstrated a strong link between Technical Performance Score (TPS) and mortality. The aim of this was to evaluate risk factors for mortality and complications after TOF repair in a high-volume LMIC heart center with a focus on surgical performance.MethodsWe retrospectively reviewed children undergoing TOF repair between 2015 and 2022. Patients over two years of age or with complex defects beyond atrial septal defect and persistent ductus arteriosus were excluded. Preoperative factors included age, weight, genetic syndromes, urgency, and pulmonary artery z-scores. Intraoperative variables included transannular patch use, cardiopulmonary bypass and cross-clamp times, surgeon, and TPS classification. Postoperative variables included delayed sternal closure (DSC), extracorporeal membrane oxygenation (ECMO), and Vasoactive Inotropic Score (VIS) during the first 24 h. Outcomes included mortality, complications, and length of stay (LOS). Analyses included logistic regression and Kaplan-Meier estimates.ResultsAmong 255 patients, in-hospital mortality was 7.5% (n = 19). Technical Performance Score class 3 was an independent predictor of mortality (P = .027), along with VIS 24 h and ECMO. Cardiopulmonary bypass time, ECMO, and DSC were associated with major complications. Technical Performance Score class 3 correlated with longer LOS, but multivariable analysis identified low weight and ECMO as independent LOS predictors. Five-year survival was worse in TPS class 3 (P = .007).ConclusionsInadequate TPS is an independent predictor of mortality after TOF repair in an LMIC context. Class 3 TPS also relates to longer hospitalization and worse medium-term survival. Surgical quality remains essential for better outcomes.

Congenital tricuspid valve regurgitation (TR) is rare, and chordae tendinae rupture is an even more uncommon finding in neonates. We describe a case with intrauterine tricuspid valve chordae tendinae rupture leading to severe TR and hydrops fetalis. Successful tricuspid valve repair using artificial chordae, leaflet approximation, and annular reduction were used as rescue therapy for the neonate.

The last 10 years have seen an exponential rise in the use of extracorporeal life support (ECLS), also frequently referred to as ECMO (extracorporeal membrane oxygenation) when a membrane lung is incorporated. One of the consequences of this rise in ECLS is the need for equipment to initiate and maintain the patient on support. This is especially true in pediatrics, as the majority of available systems are designed for adults. This review will outline changes that have occurred in the ECMO field and review current and upcoming systems which may advance the field even further. To assist in understanding the differences between devices, comparison tables are provided for ECMO systems and cannulas.