World journal for pediatric & congenital heart surgery最新文献

There are fewer than 100 reported cases of congenital left main coronary artery atresia. In this report, we present an adolescent male presenting with exertional syncope in the setting of this rare coronary defect, and review important diagnostic and therapeutic considerations imperative to obtain a favorable outcome.

Hypoplastic left heart complex presents a diverse spectrum of variants. Traditional management has been dichotomous, involving either single ventricle palliation or high-risk biventricular repair. Surgical approaches to achieve biventricular repair in children with borderline left ventricle are continually evolving. One such method is the staged left ventricular recruitment (SLVR) strategy. Here, we illustrate a unique surgical approach to SLVR, achieving biventricular repair over 22 months in a neonate with hypoplastic left heart variant.

Long-term use of transvenous pacemakers, a common modality used for symptomatic bradyarrhythmias, can lead to rare but significant complications, especially in patients with congenital heart disease. We present a complex and challenging surgical lead extraction for a patient with long-standing superior vena cava syndrome resulting from prolonged transvenous leads.

Background: Previous reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported.

Methods: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined.

Results: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort.

Conclusions: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit.

We present a very rare case of right atrial isomerism, double-outlet right ventricle, and incomplete atrioventricular septal defect (intact ventricular septum). In the neonatal period, the right ventricle was compressed by a "blind-ended" left ventricle with mild-to-moderate left atrioventricular valve regurgitation. The regurgitation gradually decreased from mild-to-moderate to mild with body weight gain. The patient underwent systemic-to-pulmonary shunt at three months of age and bilateral bidirectional Glenn at eight months of age. Although the echocardiogram demonstrated that the right ventricle was still compressed by the left ventricle, over time the size of the left ventricle reduced significantly and the left atrioventricular valve regurgitation became trivial.

Background: This study describes the 20-year experience of managing common arterial trunk (CAT) in a low-and-middle-income country and compares the early and medium-term outcomes following the transition from conduit to nonconduit repair at the Red Cross War Memorial Children's Hospital.

Methods: Single-center retrospective study of consecutive patients aged less than 18 years who underwent repair of CAT from January 1999 to December 2018 at the Red Cross War Memorial Children's Hospital. Patients with interrupted aortic arch or previous pulmonary artery banding were excluded.

Results: Fifty-four patients had CAT repair during the study period. Thirty-four (63.0%) patients had a conduit repair, and 20 (37.0%) patients had a nonconduit repair. There were two intraoperative deaths. Thirty-day in-hospital mortality was 22.2% (12/54). Overall, in-hospital mortality was 29.6% (16/54). Eight (21.1%) late mortalities were observed. The actuarial survival for the conduit group was 77.5%, 53.4%, and 44.5% at 6, 12, and 27 months, respectively, and the nonconduit group was 58.6% at six months. The overall freedom from reoperation between the conduit group and nonconduit group was 66.2% versus 86.5%, 66.2% versus 76.9%, and 29.8% versus 64.1% at 1, 2, and 8 years, respectively.

Conclusions: The outcomes following the transition to nonconduit repair for CAT in a low- and middle-income setting appear to be encouraging. There was no difference in mortality between conduit and nonconduit repairs, and importantly the results suggest a trend toward lower reintervention rates.

Cardiopulmonary bypass (CPB) is essential for the conduct of open-heart procedures. While lifesaving, CPB can be associated with significant end-organ injuries believed to result from inflammatory responses triggered by the extracorporeal surfaces encountering cellular elements in the blood stream. In this review, we discuss the role of interleukin-6 (IL-6) and the potential for Tocilizumab, an anti-IL-6 receptor antibody, in mitigating these effects. We compare the inflammatory responses in CPB and cytokine storm, a clinical condition in which Tocilizumab has been effectively implemented. Finally, we examine why corticosteroids, used to reduce the morbidity of CPB, may not effectively reduce IL-6 levels.

Background: Despite surgical advances, neonatal truncus arteriosus repair remains high risk and approximately 10% of patients receive perioperative extracorporeal membrane oxygenation (ECMO). We aimed to assess factors and outcomes associated with the use of perioperative ECMO in infants undergoing truncus arteriosus repair. Methods: We conducted a retrospective cohort study of patients who underwent truncus arteriosus repair between 2004 and 2019, using administrative data from the Pediatric Health Information System database. Results: We identified 1,645 neonates and infants who underwent truncus arteriosus repair at 49 centers, of which 141 (8.6%) received ECMO. Prematurity (adjusted odds ratio [aOR], 2.06; 95% CI, 1.38-3.06; P < .001), truncal valve intervention (aOR, 4.69; 95% CI, 2.56-8.59; P < .001), and interrupted aortic arch repair (aOR, 1.80; 95% CI, 0.96-3.38; P = .07) were associated with perioperative ECMO. Hospital mortality occurred in 87 of 141 (62%) patients who received ECMO compared with 77/1504 (5.1%) who did not require ECMO(aOR, 13.39; 95% CI, 8.70-20.61; P < .001). In the 1,481 patients who survived to hospital discharge, ECMO was associated with higher rates of postoperative length of stay >30 days (63% [34/54] vs 28% [400/1427]; aOR 2.65; 95% CI, 1.24-5.64, P = .012) and hospital readmission within 90 days (61% [33/54 [ vs 33% [474/1427] [; aOR, 2.66; 95% CI, 1.47-4.82; P = .001). Conclusions: Prematurity, truncal valve intervention, and interrupted aortic arch repair are important risk factors that could help predict the use of perioperative ECMO. Extracorporeal membrane oxygenation utilization is strongly associated with greater odds of hospital mortality, prolonged postoperative length of stay, and higher rates of hospital readmission in surviving patients.

Background: In newly emerging economy countries, the shortage of pediatric donor hearts and poor healthcare infrastructure poses a significant challenge. Although mechanical circulatory support (MCS) has been proven effective in enhancing heart transplant waiting list outcomes, economic barriers hinder its widespread adoption. Methods: A single-center retrospective study reviewed children under 10 kg on the heart transplant (HTx) list from 2012 to 2023. Elective or priority status was assigned based on their clinical condition at the time of transplant. In cases of clinical decompensation, centrifugal pump and extracorporeal membrane oxygenation, transitioned to the Berlin Heart EXCOR (BHE) pediatric ventricular assist device (VAD) was employed. Pre- and post-HTx outcomes were analyzed with descriptive statistics, Cox regression, and competing survival risks. Results: Out of 81 infants on the HTx list, 61.7% (50/81) were in critical condition. The median wait time was 224 days, and 34% (28/81) died while waiting. Out of 37 transplanted patients, 6 (16%) had graft dysfunction, and 10 (27%) had acute renal injury. Survival to discharge was 84% (31/37). Patients who received the BHE exhibited higher chances of receiving a transplant (hazard ratio: 2.3; 95% confidence interval: 1.2-4.6; P = .01). Priority status or MCS use did not significantly impact mortality post-transplant. Conclusion: Advanced MCS technologies can potentially reduce the mortality risk on the pediatric HTx waitlist. The findings highlight the significant waiting time for HTx and the critical role of the BHE in improving outcomes in children, particularly those under 10 kg. The results advocate for the adoption of ventricular assist devices as a viable interim solution to bridge critically ill children to HTx, ultimately enhancing their chances of survival despite limited donor heart availability.

Background: Patients with congenital heart disease (CHD) are increasingly surviving to adulthood. Given their age- and condition-specific needs, the optimal postoperative setting for these patients is not yet determined. This study aims to evaluate the utility of an adult-specific order set in improving clinical outcomes for these patients in a pediatric cardiovascular intensive care unit (ICU). Methods: Adults with CHD (ACHD) admitted to the cardiovascular ICU following cardiac surgery at a pediatric tertiary care center were identified for a retrospective cohort study. Health care delivery metrics and clinical outcomes of participants who received the adult-specific order set were compared with control patients admitted prior to the intervention. Categorical outcomes were compared using the χ² test of independence or Fisher exact test, and continuous outcomes were assessed using the t test. Results: A total of 130 ACHD patients received the intervention, with no significant differences observed in clinical outcomes when compared with 89 controls. While there was additionally no reduction in hospital-related mortality (2.3% vs 7.8%, RR 0.3; P = .1), clinician ordering behaviors were better aligned with best-practices following the intervention. Among patients with moderate or greater anatomic complexity, a post hoc analysis demonstrated reduced in-hospital mortality from those who received the adult order set. Conclusions: The implementation of an adult-specific order set in a pediatric care setting did not improve clinical outcomes for all ACHD patients. Patients with moderate and greater anatomic complexity did see a mortality benefit, suggestive that targeted electronic tools may most benefit those who have the highest risk.