World journal for pediatric & congenital heart surgery最新文献

Primary cardiac leiomyosarcoma is exceedingly rare in children. We describe a four-year-old girl presenting with recurrent syncope due to a mass in the right ventricular outflow tract causing severe obstruction. She underwent surgical resection of the tumor, presumed as benign on cardiac imaging. The histopathology, however, confirmed it to be leiomyosarcoma. Post-surgery there was rapid tumor regrowth despite first-line chemotherapy, which prompted treatment with pazopanib, a multitargeted tyrosine kinase inhibitor, which led to complete remission within six months, obviating the need for a high-risk reoperation. This case highlights the utility of pazopanib in pediatric sarcomas unresponsive to conventional therapy.

BackgroundHospital-acquired infections (HAIs) are associated with morbidity and mortality in children following congenital cardiac surgery. Our center performs approximately 200 congenital surgeries a year, but infection rates and risk factors remain unknown. This study aims to identify HAI prevalence, risk factors, and outcomes.MethodsRetrospective cross-sectional study of children who underwent congenital cardiac surgery between 2018 and 2022. The infection risk factors were identified using univariate and multivariate analyses. Assessed outcomes are length of stay, duration of mechanical ventilation, and mortality.ResultsOut of 653 patients, 102 (15.6%) developed an infection. Independent risk factors for infection included preoperative critical care admission (odds ratio [OR], 2.7; 95% confidence interval [CI], 1.01-7), delayed sternal closure (OR, 3.3; 95% CI, 1.37-8.0), peritoneal drain (OR, 12.7; 95% CI, 1.65-97), heterotaxy syndrome (OR, 5.6; 95% CI, 1-31), and extracorporeal membrane oxygenation (ECMO) placement in the critical care unit (OR, 10.6; 95% CI, 1.8-64.2). The pediatric critical care and hospital stays of patients with infections were significantly longer with 12.79 (12.15) days versus 4.07(5.47) days and 25.53 (16.86) days versus 10.21 (7.5) days, respectively. Infected patients had a longer mechanical ventilation duration, 169.5 (264.8) versus 28.16 (53.6) hours. Infections were associated with a higher mortality rate, with 7 (6.9%) versus 12 (2.2%).ConclusionsThe prevalence of HAIs following cardiac surgery was 15.6%. Preoperative critical care unit admission, delayed sternal closure, heterotaxy syndrome, peritoneal drain, and ECMO placement in the critical care unit were independent risk factors. Infected patients have worse outcomes. Preventive strategies are greatly needed.

We report the case of a four-month-old child with complex congenital heart disease, including tricuspid atresia, absent pulmonary valve, hypoplastic right ventricle, right ventricular coronary artery fistula, and Uhl anomaly, and to describe the challenges and management decisions involved. A Blalock-Taussig-Thomas (BTT) shunt was initially planned. However, severe ST-segment depression occurred upon oral feeding, indicating coronary insufficiency. Cardiac catheterization revealed a single coronary artery with a right ventricular coronary artery fistula, which increased the risk of myocardial ischemia with the BTT shunt. Consequently, a bidirectional Glenn (BDG) procedure with left pulmonary artery reconstruction was performed after maintaining ductal patency with prostaglandin for four months. The patient had a favorable postoperative course without ischemic events. In patients with complex congenital heart defects and coronary anomalies, the presence of a right ventricular coronary artery fistula may increase the risk of ischemia with a BTT shunt. A BDG procedure can offer a safer alternative, leading to a positive clinical outcome and avoiding ischemic complications.

ObjectiveReports on the use of the Impella device in children undergoing heart transplantation have been limited. We sought to provide what is to our knowledge the first multi-institutional national report of pediatric Impella device utilization as a bridge-to-heart transplant strategy.MethodsAll patients (<18 years) who underwent Impella placement in the United Network for Organ Sharing (UNOS) from the first use in 2013 to June 2024 were identified. Descriptive analysis was performed, and posttransplant survival analyzed using Kaplan-Meier survival analysis.ResultsFifty children who underwent Impella placement were identified. All UNOS regions utilized the Impella device in pediatric patients. The earliest use was in 2013. Starting in 2022, use as a bridge-to-transplantation experienced a more than two-fold increase, with a consistent rise to 11 devices in 2023 and 10 devices as of June 2024. The median age at Impella implantation was 15 years [13-17]. The median weight was 61 kg [48-82], and the median height was 167 cm [153-172]. The most frequent listing diagnosis was cardiomyopathy (N = 35/50, 70%), followed by congenital heart disease (N = 10/50, 20%). The median device duration was 12 days [6-21], and among all 50 children, 84% (N = 42) underwent heart transplantation the one-year survival was 94.45% [91.23%-97.59%].ConclusionThis report demonstrates the diverse and increasing use of the Impella device as a bridge-to-heart transplantation strategy in children. While early outcomes are promising, investigation is warranted to understand how this less invasive and versatile device can maximize outcomes for children.

BackgroundThere is geographic disparity in the provision of Pediatric and Congenital Heart Disease (PCHD) services; Africa accounts for only 1% of global cardiothoracic surgical capacity.MethodsWe conducted a survey of PCHD services in Africa, to investigate institution and national-level resources for pediatric cardiology and cardiothoracic surgery. Results were compared with international guidelines for PCHD services and institutions were ranked by a composite score for low- and middle-income PCHD services.ResultsThere were 124 respondents from 96 institutions in 45 countries. Eighteen (40%) countries provided a full PCHD service including interventional cardiology and cardiopulmonary bypass (CPB) cardiac surgery. Ten countries (22%) provided cardiac surgery services but no interventional cardiology service, 4 of which did not have CPB facilities. One provided interventional cardiology services but no cardiac surgery service. Ten countries (22%) had no PCHD service. There were 0.04 (interquartile range [IQR]: 0.00-0.13) pediatric cardiothoracic surgeons and 0.17 (IQR: 0.02-0.35) pediatric cardiologists per million population. No institution met all criteria for level 5 PCHD national referral centers, and 8/87 (9.2%) met the criteria for level 4 regional referral centers. Thirteen (29%) countries report both pediatric cardiology and cardiothoracic surgery fellowship training programs.ConclusionsOnly 18 (40%) countries provided full PCHD services. The number of pediatric cardiologists and cardiothoracic surgeons is below international recommendations. Only Libya and Mauritius have the recommended 2 pediatric cardiologists per million population, and no country meets the recommended 1.25 cardiothoracic surgeons per million. There is a significant shortage of fellowship training programs which must be addressed if PCHD capacity is to be increased.

Background: Down syndrome is the most common chromosomal abnormality in live-born infants, and approximately 40% to 60% of children with Down syndrome are diagnosed with congenital heart defects. Corrective cardiac surgery can be performed in these individuals with good outcomes. In this study, we aimed to determine the incidence and outcomes of postoperative arrhythmias in patients with Down syndrome who underwent cardiac surgery. Methods: We conducted a retrospective analysis of patients < 18 years of age who were diagnosed with Down syndrome and had cardiac surgery between 2008 and 2023 at two tertiary cardiac centers. Results: Of 328 total patients, the most frequently encountered lesions were complete atrioventricular septal defect (145/328, 44%), ventricular septal defect (85/328, 26%), and partial atrioventricular septal defect (38/328, 11%). The incidence of postoperative arrhythmias was 24% (87 events in 80 patients). Of these arrhythmias, 27/87 (31%) were tachyarrhythmias (junctional ectopic, atrial ectopic, and reentrant paroxysmal supraventricular tachycardia) and 60/87 (69%) were bradyarrhythmias. These included third-degree atrioventricular [AV] block (n = 56) and sinus node dysfunction (n = 4). Twenty-four patients required permanent pacemaker implantation for persistent postoperative AV block (7% of all the study group cohorts). This incidence was higher than the reported incidence of 1% to 3% in the literature and higher than that in infants without Down syndrome who underwent cardiac repair at our two centers (2.4%). Conclusion: Children with Down syndrome who undergo cardiac surgery have a higher incidence of complete AV block requiring permanent pacemaker implantation when compared to children without Down Syndrome. This information is important for the preoperative counseling of families of these patients and postoperative planning.

BackgroundAspirin has the potential of offering coronary arterial protection from thromboembolism for patients following the arterial switch operation (ASO) but is used inconsistently and the benefits are unknown. We sought to discern trends and outcomes associated with the use of aspirin following the ASO.MethodsPatients undergoing the ASO were identified from the Pediatric Health Information System and divided into two groups: those who received aspirin post-ASO versus the nonaspirin group to evaluate trends, variations, and outcomes.ResultsA total of 6,350 ASO cases were identified (44 centers) of which 1,751 (27.6%) were in the aspirin group. Aspirin was started after a median postoperative duration of four days (interquartile range: 1-7). The aspirin group was more likely to have coronary anomalies (221/1,751[12.6%] vs 358/4,599 [7.8%], P < .001) and transposition of the great arteries + double outlet right ventricle (109/1,751 [6.2%] vs 313/4,599 [6.8%], P = .01). Aspirin use increased from 17% of case in 2004 to 39% in 2022. The number of centers using aspirin for ≥50% of their ASO patients increased from 2004 to 2008: 4 centers to 2019 to 2022: 15 centers (out of 32 centers with consistent participation) with a greater proportion of high-volume centers using aspirin. Overall, the aspirin group had lower in-hospital mortality (16/1,751 [0.9%] vs 155/4,599 [3.4%], P < .001) and similar rates of reoperation for bleeding (59/1,751 [3.4%] vs 203/4,599 [4.4%], P = .06) and postoperative extracorporeal membrane oxygenation (70/1,755 [4%] vs 195/4,599 [4.2%], P = .67). After adjusting for confounders, the mortality difference remained significant: odds ratio: 0.22 (95%CI: 0.10-0.47), P < .001.ConclusionConsiderable variation exists in the use of aspirin post-ASO with an overall increasing trend in aspirin use. In this study, the use of aspirin did not result in an increase in postoperative bleeding or other major adverse events. Prospective studies are needed to confirm the safety and benefits of aspirin.

BackgroundPulmonary artery (PA) stenosis is a known complication of the arterial switch operation (ASO) for the repair of transposition of the great arteries. This has previously been described in small, single-center reports. This study aimed to examine the incidence of PA intervention and the impact of center volume in a large national sample.MethodsA multicenter, retrospective cohort study of infants undergoing neonatal ASO from January 2005 to December 2021 at hospitals in the Pediatric Health Information System database was performed. Primary outcomes were the overall prevalence of PA intervention and cumulative incidence rate by center volume tertile. A Cox proportional hazard model clustered by center and partitioned at 1.5 years, assessed the impact of volume on intervention rates.Results7411 infants underwent ASO; 1262 (17%) had a PA intervention. The median time to intervention was 0.8 years. Those who underwent intervention had longer initial hospital length of stay (22 vs 18 days, p < .01), lower birth weight (3000 gm vs 3150 g, p < .01), and were more likely to have a ventricular septal defect (59.3% vs 43.6%, p < .01). The incidence of PA intervention differed significantly by center volume over time with hazard ratios of 2.14 (95% CI, 1.71-2.69) and 1.32 (95% CI, 1.04-1.68) for the lowest and middle volume tertile compared with the highest volume tertile, respectively.ConclusionsPA intervention is common after ASO with the majority occurring in the first year. Larger volume centers had lower intervention rates. Studies are needed to identify modifiable factors to decrease PA intervention rates.

PurposeTransposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction (TGA + IVS + LVOTO) is a rare congenital cardiac malformation. This study aims to describe the operations performed for patients with TGA + IVS + LVOTO in the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and review their short-term outcomes.MethodsA total of 112 patients with the diagnosis of TGA + IVS + LVOTO in the STS-CHSD who underwent cardiac surgery between January 1999 and June 2021 were included. Patients' characteristics, operative data, and postoperative outcomes were analyzed.ResultsA total of 120 index cardiac operations were performed. The most common operations were the arterial switch procedure (n = 33), Glenn/HemiFontan (n = 10), Fontan (n = 9), Rastelli with ventricular septal defect (VSD) creation (n = 6), Damus-Kaye-Stansel procedure (n = 5), heart transplant (n = 5), and atrial switch (n = 4). Concomitant repair of subvalvar aortic stenosis was coded in 7 of 33 patients undergoing the arterial switch operation. The overall operative mortality was 4.5% (5 deaths in 112 patients undergoing 120 index cardiac operations). The overall major complication rate was 19% (23 of 120 cases). Major complications occurred in 9 of 33 (27%) of the arterial switch operations. The most common complications were unplanned reoperation in 14 of 120 cases (12%) arrhythmias requiring pacemaker implantation in 5 of 120 (4.2%), and postoperative mechanical circulatory support in 4 of 120 (3.3%).ConclusionThe diagnosis of TGA + IVS + LVOTO is rare in the STS-CHSD. The most common procedures performed for this disease were the arterial switch operation (often with concomitant repair of subvalvar aortic stenosis), Rastelli with VSD creation, and palliative functionally univentricular operations.