World journal for pediatric & congenital heart surgery最新文献

Nonbacterial thrombotic endocarditis (NBTE) is an extremely rare and usually fatal condition in neonates, characterized by sterile fibrin-platelet vegetations on cardiac valves. We report a term male neonate with severe left ventricular outflow tract and aortic valve obstruction due to a 7 × 8 mm mass. Urgent surgical resection preserved valve anatomy, and recovery was uneventful; histopathology confirmed NBTE. Differential diagnosis of neonatal cardiac masses, including rhabdomyomas, fibroelastomas, and thrombi, is crucial. Early echocardiographic recognition, prostaglandin support, and timely surgery can enable survival despite an otherwise poor prognosis.

An anastomosis between the superior vena cava and the right pulmonary artery was devised to increase pulmonary blood flow in congenital heart malformations characterized by pulmonary hypoperfusion. The problem of oxygenation of the pulmonary blood under venous pressure and without the contribution of the right cardiac output was first studied and applied experimentally by Carlon, Mondini, and De Marchi, at the University of Padova, by creating a connection between the superior vena cava and the right pulmonary artery. This operation has been replicated both experimentally and clinically by many others but the original Italian contribution has been neglected for many years. The cavopulmonary anastomosis to the right pulmonary artery, as initially designed, has essentially been abandoned and replaced by the bidirectional cavopulmonary anastomosis. Nevertheless, the ingenuity of those who first devised it must be adequately appreciated, respected, and remembered.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly typically diagnosed in infancy. Adult presentation is uncommon and often discovered incidentally. Management becomes more complex in the setting of prior cardiac surgeries, particularly mechanical valve replacement. We report a case of a 26-year-old male with a history of mitral valve repair in 2003 and mechanical mitral valve replacement in 2016, who presented with progressive fatigue and exertional dyspnea. Transthoracic echocardiography revealed severe patient-prosthesis mismatch. Preoperative computed tomography incidentally diagnosed ALCAPA, which had not been detected in previous surgeries. The patient underwent successful redo mitral valve replacement and ALCAPA revascularization using a 5-mm polytetrafluoroethylene vascular prosthesis tunneled through the pulmonary artery. Postoperative recovery was uneventful, and follow-up echocardiography demonstrated satisfactory mitral valve function, patent graft flow, and no significant pulmonary valve gradient. This case highlights the importance of preoperative multimodality imaging in redo cardiac surgery and demonstrates the feasibility of coronary artery revascularization using an intrapulmonary vascular prosthesis for an adult with ALCAPA in a surgically complex setting.

Background: Deferral of the Stage 1 Norwood Palliation (S1P) for infants with hypoplastic left heart syndrome and high-risk features is sometimes accomplished using a hybrid approach involving external pulmonary artery (PA) banding. Recently, percutaneous restriction of pulmonary blood flow has been accomplished with off-label modification of microvascular plugs to delay surgery. Methods: This single-center, retrospective case series examines outcomes associated with PA flow restrictors (PAFR) in S1P candidates. Primary outcomes included death, extracorporeal membrane oxygenation, PA reintervention, and variation from expected pathway post bidirectional Glenn (BDG), which includes prolonged intubation (>24 h), persistent hypoxia (saturation <75%), or prolonged hospital length of stay (>14 days). Results: From December 2022 to March 2025, bilateral PAFRs were placed in 12 S1P-eligible neonates. Three patients died with flow restrictors before S1P. One patient developed progressive heart failure and underwent a heart transplant. The remaining eight patients underwent S1P with removal of flow restrictors and pulmonary arterioplasty at a median of 21 days postinsertion. Three patients required PA stents between S1P and BDG. Six patients have progressed to BDG, all with courses that significantly varied from the expected pathway, including three with early PA stenting and one patient who died due to sequelae of failed BDG circulation. Conclusion: Flow restrictors are an adjunct to the management of high-risk patients prior to S1P, allowing for evaluation of additional comorbidities. However, their use appears to be associated with an important rate of PA complications, resulting in significant variation from the expected clinical course and may impact quality of BDG circulation.

Introduction: Right ventricular outflow tract (RVOT) reconstruction in congenital heart diseases such as tetralogy of Fallot (TOF) often requires a transannular patch, which can lead to postoperative pulmonary regurgitation (PR). Persistent PR may result in significant complications, including right ventricular (RV) dysfunction, arrhythmias, and the need for reintervention. The use of autologous right atrial appendage (RAA) tissue to create a functional neovalve has emerged as a promising alternative. Methods: Twelve pediatric patients who underwent RVOT reconstruction with an RAA neovalve between July 2023 and March 2025 were retrospectively evaluated. Demographic, surgical, and echocardiographic data were analyzed to assess early clinical outcomes. Results: The median age at surgery was eight months (IQR: 6-11), with a median weight of 7.1 kg (range: 5.7-16). Primary diagnoses included TOF (n = 9), TOF with pulmonary atresia (n = 2), and absent pulmonary valve (n = 1). No in-hospital mortality occurred. Predischarge echocardiography showed no or trivial PR in eight patients and mild PR in four patients. At a mean follow-up of 12.8 months, all patients exhibited either no or mild PR, and RV function remained normal. No patient required reintervention. Conclusion: Neovalve reconstruction using autologous RAA tissue is a safe, feasible, and hemodynamically effective technique for RVOT repair in selected pediatric patients. Due to its autologous nature, growth potential, and favorable early outcomes, it offers a meaningful alternative to synthetic or allogenic materials. Further follow-up studies are necessary to evaluate its long-term durability.

Objective: Children undergoing surgery for congenital heart disease (CHD) are at risk for catheter-associated deep vein thrombosis (CA-DVT). We sought to understand the practice variations which may impact the risk for CA-DVT by conducting a comprehensive survey. Methods: Analysis of electronic survey of Pediatric Cardiac Critical Care Consortium (PC⁴) hospitals caring for children undergoing surgery for CHD. Results: Responses from 45 respondent PC4 centers was analyzed; 71% of centers (n = 32) had a prophylactic anticoagulation protocol. Two of the 45 respondent centers utilized a protocol for proactive screening for CA-DVT; 64% of centers (29/45) treated CA-DVT for a duration of 6 to 12 weeks. Internal jugular central vein catheters (CVC) were the most common primary access in children who were 1 to 18 years of age undergoing surgery (89% [40/45] of centers) and in infants 1 to 12 months of age (73% [33/45] of centers). Significant variability CVC-type selection was reported in neonates (<30 days of age). More than half of centers reported avoiding upper extremity peripherally inserted central catheter placement in patients both prior to and after stage 2 palliation for single ventricle CHD (58% [26/45] and 53% [24/45], respectively). Conclusions: Significant variability in prevention and management of CA-DVT is reported among PC⁴ centers. Only half of respondent PC4 centers reported having an established treatment protocol for CA-DVT. Consensus and evidence-based guidelines for the treatment of CA-DVT are not consistently followed with only 62% (28/45) of centers reported treating for the recommended 6 to 12 weeks with anticoagulation. There is high variability in CVC-type and location selection in neonates undergoing CHD surgery.

We present a simple technique to decrease Gore-Tex needle hole bleeding during pediatric cardiac surgery. Use of this technique will potentially decrease blood loss, and blood product transfusion, along with shortening operating room time.

We report a four-year-old female with anomalous left coronary artery from the pulmonary artery who developed a large left ventricular (LV) aneurysm following coronary reimplantation in early infancy and subsequent mitral valve repair and replacement for progressive mitral valve regurgitation. While the patient was asymptomatic, echocardiogram demonstrated an enlarged LV aneurysm, which was confirmed by cardiac computed tomography angiography. Surgical exclusion of the aneurysm with a patch and replacement of the prosthetic mitral valve were performed. The etiology of late development of an LV aneurysm is inconclusive but could be associated with prior ischemic injury and additional local myocardial impairment following the mitral valve replacement. Routine postoperative surveillance is recommended.

Background: ABO-Incompatible (ABO-I) heart transplantation (HT) is a promising strategy to extend the hope of HT to many infants and children facing donor organ shortages and waitlist mortality. Despite increasing experience with ABO-I HT and comparable outcomes, universal adoption of this strategy has yet to be achieved. We aimed to provide a contemporary analysis of ABO-I HT with emphasis on the impact of the 2016 United Network for Organ Sharing policy change. Methods: This is a retrospective study of the Organ Procurement and Transplantation Network database examining patients listed for HT at ≤2 years of age in two eras: July 7, 2008, to July 7, 2016, and July 8, 2016, to July 8, 2024, to account for allocation policy changes. The primary outcome was posttransplant survival. Additional outcomes included waitlist mortality, center volume-survival relationship, and incidence of postoperative complications. Results: A total of 2,770 children underwent HT; 493 (18%) were ABO-I recipients. Frequency of ABO-I HT increased across the study period; there was no difference in waitlist mortality for ABO-I eligible candidates across eras (hazard ratio [HR] 0.70, confidence interval [CI 0.48-1.02, P = .07). No differences were observed in one- or five-year survival between ABO-I and ABO-compatible (ABO-C) cohorts. There was no difference in survival among ABO-I recipients between high-volume centers (HVCs) and non-HVCs (HR 0.87, CI 0.43-1.76, P = .70). Conclusions: ABO-Incompatible transplantation offers comparable posttransplant outcomes with ABO-C transplantation, regardless of center volume. The 2016 United Network for Organ Sharing policy has not impacted waitlist mortality for this population. These data provide support for universal adoption of ABO-I HT in appropriately selected patients as the standard of care for infants and children awaiting HT.

PurposeWe reviewed all 181 patients with pediatric and/or congenital heart disease (CHD) who underwent cardiac transplantation at the University of Florida from January 1, 2011, to March 1, 2022, and compared those with CHD with those who had acquired heart disease (AHD).MethodsPatient characteristics were assessed, stratified by CHD versus AHD. Continuous variables are presented as mean (SD); median[interquartile range](range). Categorical variables are presented as N (%). Univariable associations with long-term mortality were assessed with a Cox proportional-hazards model. The impact of CHD versus AHD on mortality was estimated with multivariable models. The Kaplan-Meier method was applied to estimate survival stratified by CHD versus AHD status.ResultsCongenital heart disease was present in 112/181 patients = 61.9% of the total cohort. Acquired heart disease was present in 69/181 = 38.1%. More patients with CHD had prior cardiac surgery (95/112 = 84.8% vs 35/69 = 50.7%, P < .0001) and pretransplant liver dysfunction (19/112 = 17.0% vs 4/69 = 5.8%, P = .037). Congenital heart disease patients had longer bypass times (minutes): 148 (55.2); 137 [113-174] (72-526) versus 108 (40.3);101 [81-125] (59-300), P < .0001; longer cross clamp time (minutes): 85.9 (21.2); 85 [73-100] (49-152) versus 66.3 (14.7); 65 [55-75] (44-106), P < .0001; and lower minimum temperature on bypass (°C): 27.4 (4.3); 28 [24-31] (19-34) versus 31.6 (3.1); 32 [31-34] (20-36), P < .0001. Posttransplant length of stay (days) was longer in CHD patients: 34.5 (26.8); 27 [19-40] (3-158) versus 29.7 (48.2); 18 [15-26] (0-343), P = .0001. In multivariable analysis, CHD remained a risk factor for mortality after controlling for all variables significant in univariable modeling. Overall Kaplan-Meier 5-year survival in all 181 patients (95% confidence interval) = 85.8% (80.0%-92.1%). Kaplan-Meier 5-year survival = 77.4% (68.4%-87.5%) in CHD and 98.5% (95.6%-99.9%) in AHD, P < .0001.ConclusionCongenital heart disease is a risk factor for mortality after cardiac transplantation. Efforts must be made to understand and mitigate this risk.