World journal for pediatric & congenital heart surgery最新文献

BackgroundAs survival rates improve, adults with congenital heart disease (ACHD) increasingly present with aortopathies ranging from aortic root dilatation and aneurysm formation to life-threatening dissection and rupture. These vascular complications are associated with various congenital cardiac lesions, including bicuspid aortic valve, coarctation of the aorta, Tetralogy of Fallot, and transposition of the great arteries. Understanding the incidence, progression, and outcomes of aortopathy in this population is essential for guiding surveillance and surgical strategies.ObjectiveTo summarize current evidence on the clinical characteristics, progression, surgical decision-making and outcomes of aortopathies in ACHD.MethodsA systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Databases searched included PubMed, SCOPUS, Cochrane Library, and Medline, with articles screened for relevance using predefined criteria.ResultsOf 3382 records identified, 46 studies met the inclusion criteria following a full-text review, and these studies combined included 24 241 patients. Bicuspid aortic valve and repaired coarctation were the most studied conditions, with aortic dilatation commonly observed even in the absence of valve dysfunction. Neo-aortic root enlargement postarterial switch operation and root dilation in Tetralogy of Fallot were also prevalent. The decision for surgical intervention is individualized; it is based on overall balance of procedural versus observation risk (ie, aortic diameter thresholds), patient risk factors, suitability for valve preservation, and the presence of concurrent cardiac lesions. However, there remains heterogeneity in imaging protocols, surgical thresholds, and long-term outcome data.ConclusionsAortopathies in ACHD patients represent a significant long-term complication requiring lifelong imaging surveillance and individualized surgical management. Despite growing recognition, evidence remains limited regarding optimal thresholds for surgical intervention and personalized risk stratification.

BackgroundCardiac surgery requiring cardiopulmonary bypass began at Federal Medical Centre (FMC), Abuja, in February 2022 through visiting mission teams. Continuous collaboration between subsequent missions and local staff has led to progressive skills acquisition and increasing independence in both adult and pediatric cardiac surgery. This report outlines our path to independence and the challenges encountered.MethodsFrom February 2022 to December 2024, we reviewed 45 patients who underwent cardiac surgery with cardiopulmonary bypass. Data included the operating team (visiting vs local), case type, and outcomes.ResultsOf 45 surgeries, visiting teams performed 27 (11 adult, 16 pediatric) and local surgeons performed 18 (5 adult, 13 pediatric). Six surgical missions were conducted. The proportion of cases done independently by local surgeons increased from 14.3% in 2022 (n = 7) to 26% in 2023 (n = 23), and 73% in 2024 (n = 15). Competence was achieved first by cardiologists, followed by scrub nurses, intensive care unit nurses, anesthetists, and surgeons. The local perfusionist has yet to attain independence, representing a major program limitation. Of 45 operations, nine mortalities (20%) occurred-five in visiting-team cases and four in local-team cases. All but one were pediatric, and all occurred postoperatively.ConclusionStructured collaboration with visiting missions, deliberate skills transfer, and perseverance have enabled rapid development of cardiac surgical competence at the Federal Medical Centre, Abuja. While adult and selected pediatric cases are now managed independently, further progress requires dedicated perfusionist training and improved pediatric postoperative care.

Transcatheter ballon dilation has emerged as a valuable strategy to treat residual or recurrent right ventricular outflow tract (RVOT) obstruction following valve-sparing repair of tetralogy of Fallot (TOF). When balloon dilation is unsuccessful, stent placement in the RVOT may be effective. While uncommon, stent-related complications can occur and contribute to unintended morbidity. We report a case of RVOT stent malposition leading to severe tricuspid regurgitation and ventricular dysrhythmias in a child with recurrent RVOT obstruction following valve-sparing repair of TOF. Surgical management was required and included complete stent removal and bioprosthetic pulmonary valve replacement.

Objective: To evaluate long-term outcomes following surgical repair of coarctation of the aorta (CoA) in neonates and infants. Materials and Methods: We assessed the operative results in 308 infants with CoA who underwent primary repair at our institute between 2008 and 2023. All of the patients were <12 months of age when they had their operation. Results: The overall mortality rate was 3.9% (12/308), with critical CoA identified as the only independent predictor of death. Recoarctation occurred in 8.1% (25/308) of patients, and the significant predictors of recoarctation included low body weight at the time of surgery, use of the lateral thoracotomy approach, aortic arch remodeling, and the use of absorbable sutures. Postoperative arterial hypertension was observed in 20.4% (63/308) of cases. Independent predictors of hypertension included gothic arch morphology and a left ventricular mass index (LVMI) greater than 59 g/m². Conclusions: The choice of surgical strategy significantly affects the risk for development of recoarctation. Median sternotomy with the use of native tissue for anastomosis reduces the risk of recoarctation. The shape of the aortic arch is a predictor of long-term complications. Lowering the risk of recoarctation is the Romanesque-type aortic arch and increasing the risk of arterial hypertension is the Gothic-type arch. The strongest predictor for arterial hypertension was a LVMI greater than 59 g/m².

Purpose of ReviewPulmonary autograft autotransplantation represents a popular surgical approach for pediatric patients requiring aortic valve replacement due to the potential for autograft enlargement to accommodate somatic growth. Nevertheless, autograft dilatation and the subsequent need for reintervention are quite common. Published data suggest that autograft enlargement may result from pathological passive remodeling rather than active somatic growth and vice versa. The present review serves to comprehensively evaluate available evidence related to the fate of the pulmonary autograft after the Ross procedure as it relates to the etiology, risk factors and patterns of autograft failure.ResultsStudies present conflicting results supporting both pathological dilation and active somatic growth. Primary factors impacting the successful remodeling of the autograft include native aortic and pulmonary valve anatomy, medical history, concomitant procedures, perioperative management, age at the time of surgery, and the surgical technique used for the Ross procedure.ConclusionAutograft enlargement after the pediatric Ross operation may result from either somatic growth or passive dilation or a combination of both factors. Distinguishing between the two primary etiologies of autograft enlargement depends upon meticulous patient selection and a surgical strategy precisely tailored to individual anatomy and risk.

Abnormalities of the left ventricular papillary muscles, albeit rare, can be clinically significant. We report two pediatric cases with thickened, fused papillary muscles obstructing left ventricle inflow with abnormal attachments to the ventricular septum leading to midcavitary outflow obstruction. Surgical management, consisting of septal myectomy, splitting of hypertrophied muscles, and resection of fibrous adhesions, successfully relieved obstruction. These cases underscore the importance of considering papillary muscle abnormalities in the differential diagnosis of midcavitary obstruction.

Objective: To report our center's experience with hepatic fibrosis scores derived from transvenous hepatic biopsy and ultrasound shear-wave elastography (SWE) values in post-Fontan patients. Methods: We identified patients who underwent a lateral tunnel Fontan (LTF) or an extracardiac Fontan (ECF) Fontan between 1990 and 2020. We further identified those who underwent transvenous hepatic biopsy between February 2013 and February 2025 and SWE between January 2017 and February 2025. We defined a total hepatic fibrosis score (TFS 0-8) as the sum of the portal fibrosis (0-4) and sinusoidal fibrosis (0-4) scores. Results: We identified 318 patients. Of the 318, 204 (64.1%) were male, 266 (83.6%) had ECF, and 52 (16.4%) had LTF, 173 (54.4%) ≥5 years of age underwent 265 transvenous liver biopsies. Of the 265 biopsies, 220 were performed in 143 ECF and 45 in 30 LTF patients. Of the 173 biopsied patients, 76 underwent 115 SWE measurements. Analysis of correlation (TFS vs kPa) between 160 unique biopsy and elastography pairs, showed a correlation coefficient of R = 0.6, P ≤ .00001. Of the 160 comparisons, 67 (41.8%) had TFS 0-2 (mild), average kPa: 10.7 ± 2.3, 76 (47.6%) had TFS 3-5 (moderate), average kPa: 12.6 ± 3.3 and 17 (10.6%) had TFS 6-8 (severe), average kPa: 15.6 ± 3.7, P = .00001. We found no significant correlation between Fontan mean pressures and elastography values, R = 0.1, P = .29. Conclusions: In post-Fontan patients, we found a moderately strong correlation between SWE and TFS. Further, although data overlap, SWE may be clinically useful in differentiating the degree of hepatic fibrosis, especially mild versus severe.

Background: The Fontan operation is typically performed between two and five years-of-age in the United States. In this study, we analyzed the immediate outcomes of the Fontan operation performed beyond the first decade of life in the United States using a large administrative database. Methods: Kids' Inpatient Database (2003-2019) and Nationwide Inpatient Sample (2016-2021) datasets were used; 10,245 pediatric patients undergoing the Fontan operation were identified. The cohort was divided into: Traditional Fontan (TF, 2-5 years-of-age, n = 9,900) and Late Fontan (LF, ≥10 years-of-age, n = 345); Survivor and non-survivor status were based on discharge mortality. Demographic and clinical characteristics were assessed using standard statistical tests. Results: Only 3% of the Fontan procedures (n = 345/10,245) belonged to the LF group. LF was comprised predominantly of non-Caucasian ethnicity, higher socioeconomic class, and had a greater comorbidity burden. Heterotaxy syndrome and total anomalous pulmonary venous return were more common in the LF group as compared with hypoplastic left heart syndrome in the TF group. Patients in the LF group experienced higher postoperative morbidity but similar mortality and often required specialized healthcare post-discharge. Multivariate regression analysis revealed inferior survival among Fontan patients with ECMO use, atrioventricular septal defect, coagulopathy, acute kidney injury, infection, prolonged mechanical ventilation, but not age at Fontan. Conclusion: The proportion of patients undergoing LF compared with TF has decreased over time signalling a move toward earlier timing of the Fontan procedure. However, within the LF group, the number of LF patients has increased over time signifying an extended application of the Fontan operation. The patients in the LF group experienced greater postoperative morbidity with an associated higher baseline comorbidity but not short-term mortality after the Fontan procedure.