Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease

Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.     

基于回归分析和机器学习的先天性心脏病患儿术后住院死亡预测模型的建立—单中心12年大数据汇总分析

目的探索建立先天性心脏病患儿术后住院病死率的预测模型。方法收集上海儿童医学中心2006年1月1日至2017年12月31日所有行先天性心脏病手术的病例资料,根据亚里士多德评分系统对不同手术评分,分别应用logistic回归分析和机器学习建立包含手术复杂性评分和患儿术前危险因素的术后住院病死率预测模型并比较两种模型的C指数。结果共纳入24693例行先天性心脏病手术病例,595例(2.4%)术后住院死亡。所有手术术后住院病死率为0~77.8%,其中32种手术术后住院病死率为0。除手术复杂性评分,其他危险因素包括年龄、身高、手术史、超声心动图检查及实验室检查结果(主要为凝血因子)与术后住院死亡显著相关。仅用手术复杂性评分预测术后住院死亡的ROC曲线下面积为0.654(95%CI:0.628~0.681,P<0.001),综合分析手术复杂性评分和术前危险因素建立预测模型的ROC曲线下面积为0.886(95%CI:0.868~0.904,P<0.001),应用机器学习建立预测模型的ROC曲线下面积为0.889(敏感度为0.817)。应用logistic回归和机器学习建立模型所参考的危险因素略有差异。结论应用logistic回归分析和机器学习建立的包含手术复杂性评分和术前危险因素的预测模型能更准确预测术后住院死亡,这对于先天性心脏病患儿手术风险评估及术后管理具有重要临床意义。     

Heart–lung and lung transplantation in grown-up congenital heart disease: long-term single centre experience

Objective: Because of considerable progress in paediatric cardiac surgery life expectancy of patients with congenital heart disease (CHD) has improved significantly over the years. There are a growing number of adults with CHD presenting with progressive decline of cardiopulmonary function and Eisenmenger's syndrome. We analysed our experience with heart–lung and lung transplantation in this patient group. Methods: Since 1988, a total of 46 heart–lung transplantations and 5 double lung transplantations have been performed in adults with CHD at our institution. Underlying diagnoses were: ventricular septal defect, atrial septal defect, persistent ductus arteriosus and others. Pulmonary hypertension was present in all patients. Twelve patients had undergone previous cardiac procedures. All patients were included in this retrospective analysis. Mean follow up was 5.1 ± 4.7 years. Patient survival was estimated with the Kaplan–Meier method and analysed using the log-rank test. Results: Thirty-day mortality was 11.8% (n = 6). Survival was 80% at 1 year, 69% at 5 years and 53% at 10 years. Major causes of death were infection and sepsis, chronic rejection, initial graft failure and acute rejection. Compared to the overall mortality after lung and heart–lung transplantation for other indications at our institution there was no significant difference (1 year, 5 years, 10 years: 76%; 60%; 45%), but a tendency towards a better long-time survival of the CHD patients. Conclusions: Lung and heart–lung transplantation can be performed with an acceptable risk and a favourable long-term outcome in patients with grown-up CHD. Careful patient selection and planning of the surgical strategy is essential in this high-risk patient population.     

A case of congenital midline cervical cleft with congenital heart disease

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.     

Heart transplantation after congenital heart surgery: improving results and future goals

With growing numbers of children with complex congenital heart disease surviving initial surgical procedures, more patients are presenting in later childhood or early adulthood in cardiac failure. This presents an obvious increased burden on transplant centres, and a further strain on a limited donor pool. Historically, results for heart transplant following congenital heart disease (CHD) have been worse than those following cardiomyopathy. With increased surgical experience and intensive care expertise, the gap between the two aetiologies in our practice is decreasing. This article reviews the current protocols for transplantation in this setting, presenting a large single-centre experience over 20 years, and speculates on possible future advancements in this very challenging field.     

Infective endocarditis with congenital heart disease

From 1988 to 2005, seven patients were operated at our hospital because of infectious endocarditis (IE) with congenital heart disease (CHD). Underlying CHD included ventricular septal defect (VSD) in 4 (2 previous operations with residual region), atrial septal defect (ASD) in 2 and bicuspid aortic stenosis (AS) in 1. No cases had preventive antibiotic prophylaxis for dental procedures. We could confirm bacteria origin from blood culture in all cases, but two patients had operations without gaining control of the infection. VSD or ASD closure and valve surgery were performed in four patients. One patient had a VSD closure, two patients had valve surgery. There were no operative or hospital deaths and there were no recurrences of IE during the study period. We successfully treated IE with CHD by enough debridement of the infective focus of IE, and valve surgery. It is important for patients with CHD to have preventive antibiotic prophylaxis for dental procedures.     

Donor and recipient age matching in heart transplantation: analysis of the UNOS Registry

The association of donor and recipient age with survival following adult heart transplantation has not been well characterized. The purpose of this study was to examine the impact of the relationship between donor and recipient age on post‐transplant survival. We retrospectively reviewed the 2005–2018 UNOS heart transplant database for all adult recipients undergoing first‐time isolated heart transplantation. The impact of donor and recipient age on survival was analyzed with Cox proportional hazards modeling using restricted cubic splines. A total of 25 480 heart transplant donor and recipient pairs met inclusion criteria. Unadjusted and adjusted Cox proportional hazards modeling demonstrated a near‐linear association between increasing donor age and decreased survival; in addition, older and younger recipient age was associated with decreased survival. After adjustment, there was no significant interaction between donor and recipient age. Older donors decreased survival similarly in both older and younger recipients. Increasing donor age and both younger and older recipient age are independently associated with worsened post‐heart transplant survival. The relationship between donor and recipient age does not significantly affect survival following heart transplant.