World journal for pediatric & congenital heart surgery最新文献

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.

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.

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.

ObjectivesWe set out to determine the characteristics and outcomes of symptomatic infants with tetralogy of Fallot who due to insufficient pulmonary blood flow required transcatheter or surgical intervention in early infancy.MethodsFive centers in the United Kingdom contributed data for infants born with tetralogy of Fallot who underwent an initial procedure between January 2015 and January 2022. The studied group were infants requiring palliative or reparative procedure at less than three months of age. The primary outcome was survival to one year; secondary outcomes were periprocedural complications, cumulative mechanical ventilation time, length of hospital stay, and need for reintervention.ResultsA total of 535 infants with tetralogy of Fallot were identified during the study period. 96 infants underwent initial palliation (58 right ventricular outflow tract stent, 7 ductal stent, 17 balloon pulmonary valvuloplasty, 9 modified Blalock-Taussig-Thomas shunt, and 5 right ventricular outflow tract patch augmentation). 37 infants underwent primary repair at less than three months of age, 402 infants had primary repair at 3 to 12 months of age. Median age and weight were 26 days and 3.1 kg for initial palliation; 68 days and 4.7 kg for primary repair under three months (P < .001). One year survival post-procedure was 95.7% for initial palliation, and 97.3% for primary repair under three months (P = .55, hazard ratio [HR] 1.97, 95% confidence interval [CI] 0.33-11.67). Five-year freedom from reintervention on the right ventricle-to-pulmonary artery segment was 76% after staged repair and 88% after primary repair under three months (P = .23, HR 1.90, 95% CI 0.75-4.80).ConclusionsInitial palliation with staged repair remains the predominantly employed approach for the highest risk young infants with tetralogy of Fallot in the United Kingdom. Survival outcomes are excellent; concerns remain regarding increased cumulative costs and associated reintervention rates. Primary repair under three months of age has also proven to be feasible with excellent results, although with individual candidate selection bias.

Temporary mechanical circulatory support (tMCS) has been utilized as a bridge to heart transplantation with increasing duration of support. We describe the clinical course of a 14-year-old patient, with TNNT2 and KCNQ1 mutations, requiring Impella 5.5 support for 76 days. We discuss the patient's underlying genetic etiology, potential complications of prolonged tMCS, and the importance of multidisciplinary support. To our knowledge, this is the first published case in which a patient has this specific combination of genetic mutations and the longest published support duration of an Impella 5.5 in a pediatric patient.

Background: The arterial switch operation (ASO) is the preferred surgical treatment for transposition of the great arteries with intact ventricular septum (TGA/IVS). Delays beyond the first month of life may lead to left ventricular deconditioning. This study evaluated the perioperative outcomes of the neonatal ASO within the first month versus later in life in a middle-income country where late presentations are common, aiming to assess the safety of the ASO beyond early intervention. Method: This retrospective study included 295 TGA/IVS patients who underwent the ASO from January 2010 to December 2023. We excluded those with other significant cardiac anomalies or deconditioned left ventricle (LV). Preoperatively, we evaluated the left ventricular ejection fraction and the posterior LV wall thickness to ascertain the adequacy of the LV. Study outcomes included in-hospital mortality, postoperative chest reexploration, extracorporeal membrane oxygenation use, and the duration of ventilation and hospitalization. Results: One hundred and fifty of 295 patients (51%) had the ASO within 30 days of life (early group) whereas 145 patients (49%) had the ASO beyond 30 days of life (late group). The mean age was 20.3 ± 5.7 days for the early group and 80.0 ± 60.0 days for the late group (P < .001). The late group had significantly lower in-hospital mortality (4.8% vs 14.0%, P = .007) and a lower incidence of chest reexploration (8.3% vs 17.3%, P = .020), with odds ratio of 0.312 and 0.430, respectively. Conclusion: Our findings indicate that the ASO performed after 30 days of life for those with a preserved LV is associated with satisfactory perioperative outcomes, underscoring the importance of carefully selecting patients who present late for the ASO.

BackgroundCarotid artery ligation has been a common practice after extracorporeal membrane oxygenation (ECMO) decannulation due to the risks of embolization, cerebral infarction, aneurysm, and stenosis over time. Carotid artery reconstruction (CAR) after ECMO decannulation is increasingly used, though studies report variable stenosis rates. Data on CAR in children with heart disease, who may require carotid artery patency for future surgery or repeat ECMO, are lacking.ObjectiveTo evaluate carotid artery patency and the incidence of cerebral infarction in pediatric cardiac patients after CAR and ECMO decannulation.MethodsA retrospective review of children (0-21 years of age) who required venoarterial extracorporeal membrane oxygenation (VA-ECMO) via neck cannulation in the cardiovascular intensive care unit at a quaternary children's hospital (2015-2022) was conducted. Children who underwent CAR and survived to discharge were included.ResultsFifty-three children (ages 2 days to 21 years, median, 2 years of age) met the study criteria. Carotid artery imaging was performed in 25 children (47%), with 84% (21/25) showing unobstructed arteries. Imaging follow-up ranged from two days to four years, with a median of three months. Of 33 children who had post-ECMO head imaging, 11 showed new cerebral infarcts, with 4 of those infarcts diagnosed following transition to ventricular assist device (VAD). Given the variability of timing and modality of head imaging and the significant proportion of patients on a VAD, we are unable to determine the true incidence of new infarcts following CAR.ConclusionCarotid artery reconstruction after VA-ECMO decannulation in children with congenital heart disease shows early favorable vessel patency, but the long-term neurological benefits compared with ligation remain unclear. Further prospective studies are needed to evaluate long-term patency and neurological outcomes.

Patients who have undergone congenital heart surgery often require evaluation for potential complications in the immediate postoperative period, as well as ongoing imaging surveillance to monitor for long-term complications. Computed tomography is an excellent modality for evaluating potentional complications after congenital heart surgery. The purpose of this article is to provide a pictorial review of the potential complications of a wide spectrum of congenital heart surgical procedures.