Health Systems Strengthening to Tackle the Global Burden of Pediatric and Congenital Heart Disease: A Diagonal Approach

1 Background Congenital heart disease(CHD)is the most common major congenital anomaly,affecting approximately one in every 100 live births[1].Among congenital anomalies,66%of preventable deaths are due to CHD,and 58%of the avertable morbidity and mortality due to congenital anomalies would result from scaling congenital heart surgery services[2].Every year,nearly 300,000 children and adults die from CHD,the majority of whom live in low-and middle-income countries(LMICs)[3].Approximately 49%of all individuals with CHD will require surgical or interventional care at some point in their lifetime[4];as a result of advances in access to and the delivery of such services,over 95%of children born with CHD in high-income countries now live into adulthood[3].Here,adults have surpassed children in the number of CHD cases at a ratio of 2:1[5].

A New Three-Dimensional(3D)Printing Prepress Algorithm for Simulation of Planned Surgery for Congenital Heart Disease

Background:Three-dimensional printing technology may become a key factor in transforming clinical practice and in significant improvement of treatment outcomes.The introduction of this technique into pediatric cardiac surgery will allow us to study features of the anatomy and spatial relations of a defect and to simulate the optimal surgical repair on a printed model in every individual case.Methods:We performed the prospective cohort study which included 29 children with congenital heart defects.The hearts and the great vessels were modeled and printed out.Measurements of the same cardiac areas were taken in the same planes and points at multislice computed tomography images(group 1)and on printed 3D models of the hearts(group 2).Pre-printing treatment of the multislice computed tomography data and 3D model preparation were performed according to a newly developed algorithm.Results:The measurements taken on the 3D-printed cardiac models and the tomographic images did not differ significantly,which allowed us to conclude that the models were highly accurate and informative.The new algorithm greatly simplifies and speeds up the preparation of a 3D model for printing,while maintaining high accuracy and level of detail.Conclusions:The 3D-printed models provide an accurate preoperative assessment of the anatomy of a defect in each case.The new algorithm has several important advantages over other available programs.They enable the development of customized preliminary plans for surgical repair of each specific complex congenital heart disease,predict possible issues,determine the optimal surgical tactics,and significantly improve surgical outcomes.

Quality of Life in Congenital Heart Disease Patients according to Their Anatomical and Physiological Classification

Background:Living well is as important as living longer.The objective of this study is to assess quality of life(QoL)in congenital heart disease(CHD)according to current AHA/ACC anatomical and physiological classification.Methods:Cross-sectional study examining the World Health Organization QoL Bref questionnaire(WHOQoL-Bref)in consecutive outpatient CHD patients from a single unit.Results:191 CHD patients were studied.Median age was 28±13 years and 59%were male.44(23%),115(60%)and 33(17%)CHD patients showed mild,moderate and great anatomical defects respectively while 69(36%)patients were in physiological Stage A,27(14%)in Stage B,84(44%)in Stage C and 11(6%)in Stage D.No significant differences were seen in relation the anatomical classification and the different sections of the WHOQoL-Bref questionnaire.CHD patients in Stages C and D had significant lower physical domain scores than patients in the Stage A(p<0.05).However,no significant differences were seen in the psychological,social relationships and environmental domains.The binary logistic regression analysis showed that having a higher educational level was a protective factor[OR 0.32(95%CI,0.12-0.87),p=0.026]while being married or cohabit was a risk factor[OR 3.46(95%CI,1.13-10.63),p=0.030]for having a worse rated QoL.Meanwhile,having a worse functional class(NYHA≥2)[OR 3.44(95%CI,1.20-9.81),p=0.021]was associated with dissatisfaction with health.Conclusion:Patients with advanced physiological stages scored lower on the physiological domain.No statistical significance was seen,according to the anatomical and physiological classification,in the psychological,social relationship and environmental domains.

Psychological needs of parents of children with complicated congenital heart disease after admitting to pediatric intensive care unit: A questionnaire study

BACKGROUND Parents of children with complicated congenital heart disease(CHD)have different needs after surgery.Little literature reports the impact factors for psychological needs of parents of children with complicated CHD.AIM To investigate the status quo of the needs of parents of children after surgery for complex CHD,and analyze the influencing factors,in order to provide a theoretical basis for formulating corresponding nursing countermeasures.METHODS A modified Chinese version of the Critical Care Family Needs Inventory(MCCFNI)was used to select 200 parents of children with complex CHD after surgery within 72 h after admission to the intensive care unit in our hospital to conduct an online questionnaire survey.The aim was to understand the needs of parents in relation to the following five aspects:The support from medical staff,comfort of the parents themselves,the acquisition of information,their closeness to the children,and assurance of the child’s condition.RESULTS Parents of children with complex CHD had a higher degree of demand,especially in terms of condition assurance,acquisition of information,and closeness to the children.The age,education level,and residence of the parents were related to the five dimensions of the needs of parents of children with complex CHD who had undergone surgery.CONCLUSION In practice,nurses should formulate corresponding nursing strategies based on the different cultural and social backgrounds of parents of children after complex CHD surgery to meet their different needs,and improve satisfaction.These findings provide a theoretical basis for constructing a family participatory nursing model for children in the intensive care unit in the future.

Cardiac biomarkers in pediatric heart disease:A state of art review

Every year there are more than 11000 hospitalizations related to heart failure in children resulting in significant morbidity and mortality.Over the last two decades,our understanding,diagnosis and management of pediatric heart failure is evolving but our ability to prognosticate outcomes in pediatric heart acute heart failure is extremely limited due to lack of data.In adult heart failure patients,the role of cardiac biomarkers has exponentially increased over the last two decades.Current guidelines for management of heart failure emphasize the role of cardiac biomarkers in diagnosis,management and prognostication of heart failure.It is also noteworthy that these biomarkers reflect important biological processes that also open up the possibility of therapeutic targets.There is however,a significant gap present in the pediatric population with regards to biomarkers in pediatric heart failure.Here,we seek to review available data regarding cardiac biomarkers in the pediatric population and also explore some of the emerging biomarkers from adult literature that may be pertinent to pediatric heart failure.

Kabuki-Syndrome and Congenital Heart Disease-A Twenty-Year Institutional Experience

Background:Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality.Surgical outcomes and postoperative courses following congenital heart surgery in patients with Kabuki-Syndrome(KS)have not been well studied.Objectives:The purpose of this study was to describe the postoperative courses and associated outcomes in the largest set of KS patients undergoing congenital heart surgery to date.Methods:Patients with a confirmed molecular diagnosis of KS and a diagnosis of a CHD admitted to Texas Children’s Hospital between January 1,2000 and January 1,2020 were included(n=20).Demographics and medical histories were collected from the hospitals’electronic health records.Results:Of 20 patients identified with KS and a CHD,15 required surgical correction of their congenital cardiac malformation.Median age and weight at the time of surgery was 2 months and 4.1 kg,respectively.Median duration of hospital stay was 49 days for all surgeries and 151 days for the Norwood procedure.Postoperative infections and pleural effusions were detected and treated in 45.8%and 50%of patients,respectively.There was no in-hospital mortality for any surgery.Median follow up time was 5.6 years;survival at 6 years was 94%.Conclusions:Although KS patients seem to be at increased risk for a more complicated,prolonged postoperative course than that of patients without a genetic syndrome,patients with a diagnosis of a CHD and KS do not appear to be at increased risk of mortality following congenital heart surgery.

Effects of Palivizumab Guideline Changes on RSV Admissions in Patients with Congenital Heart Disease and Prematurity

Background: Respiratory syncytial virus (RSV) causes significant morbidity and mortality in patients with a history of prematurity and congenital heart disease (CHD). In 2014, the guidelines for Palivizumab became more restrictive for this population. We hypothesized the percentage of RSV+ admissions would increase overall and in this target group (TG) specifically. Methods: We conducted a retrospective review of patients under age 2 years admitted with bronchiolitis two seasons prior to the change (Pre) and two seasons after (Post). Our TG included patients who were eligible prior to the 2014 changes but currently no longer eligible. We used chi-square analysis to answer the two main hypotheses: 1) Percent RSV+/total bronchiolitis Pre vs Post and 2) Percent of TG/RSV+ Pre vs Post. Results: 1283 patients (546 pre, 737 post) were admitted with the diagnosis of RSV between 2012-2016, 866 actually tested positive for RSV (367 Pre, 499 Post). There was no significant difference in the number of total patients admitted with RSV (Pre = 67.2%, Post = 67.7%) or in our TG (Pre 7.1% vs Post 8.2%). TG overall had a more complicated course: longer length of stay, median 5 days, IQR 2 - 12 vs 3 days, IQR 1 - 5, (p < 0.001), intensive care unit admissions (36% vs 22.8%, p = 0.02), positive pressure ventilation (25.4% vs 15.4%, p = 0.03) and intubation (16.4% vs 6.8%, p = 0.004), but there was no difference Pre vs Post. Conclusion: The TG had an overall higher acuity, but there was no increase in the number of patients hospitalized with RSV or severity as a result of the Palivizumab guideline changes.

Perioperative extracorporeal membrane oxygenation in pediatric congenital heart disease:Chinese expert consensus

Background Congenital heart disease(CHD)is one of the main supportive diseases of extracorporeal membrane oxygena-tion in children.The management of extracorporeal membrane oxygenation(ECMO)for pediatric CHD faces more severe challenges due to the complex anatomical structure of the heart,special pathophysiology,perioperative complications and various concomitant malformations.The survival rate of ECMO for CHD was significantly lower than other classifica-tions of diseases according to the Extracorporeal Life Support Organization database.This expert consensus aims to improve the survival rate and reduce the morbidity of this patient population by standardizing the clinical strategy.Methods The editing group of this consensus gathered 11 well-known experts in pediatric cardiac surgery and ECMO field in China to develop clinical recommendations formulated on the basis of existing evidences and expert opinions.Results The primary concern of ECMO management in the perioperative period of CHD are patient selection,cannulation strategy,pump flow/ventilator parameters/vasoactive drug dosage setting,anticoagulation management,residual lesion screening,fluid and wound management and weaning or transition strategy.Prevention and treatment of complications of bleeding,thromboembolism and brain injury are emphatically discussed here.Special conditions of ECMO management related to the cardiovascular anatomy,haemodynamics and the surgical procedures of common complex CHD should be considered.Conclusions The consensus could provide a reference for patient selection,management and risk identification of periop-erative ECMO in children with CHD.

Management of Specific Complications after Congenital Heart Surgery(I)

In addition to the general consequences of surgery and cardiopulmonary by-pass, lesion-specific complications can occur after surgery for congenital heart disease. It is important for the pediatric intensive care specialist to fully understand the preoperative anatomy and the intraoperative details of these patients. This allows a timely and appropriate treatment of general and lesion-specific complications. In this article we provide a list of commonly-performed surgical procedures and possible associated problems to be anticipated in the early postoperative period. Then it follows a discussion about the diagnosis and management of these complications, based on their pathophysiological features.

家庭赋权方案对复杂先天性心脏病姑息术后患儿主要照顾者照顾能力的影响

目的:探讨家庭赋权方案对复杂先天性心脏病(CCHD)姑息术后患儿主要照顾者照顾能力的影响。方法:选取2022年6月1日~2024年4月1日首次接受CCHD姑息术后患儿主要照顾者60名,采取方便抽样法,将照顾者分为观察组和对照组各30名,对照组实施常规护理健康指导,观察组在常规健康指导基础上实施家庭赋权方案干预;比较两组主要照顾者照顾能力[采用照顾能力测量表(FCTI)]和照顾准备度。结果:出院时,两组FCTI评分和照顾准备度评分均高于入院时(P<0.05),且观察组高于对照组(P<0.01)。结论:对CCHD姑息术后的患儿主要照顾者实施家庭赋权方案,能有效提高照顾者的照顾准备度和照顾能力,帮助患儿家庭建立科学、有效的照顾方案,促进CCHD姑息术后患儿康复。

血池对比剂在儿童先心病CMR诊断中的应用研究

先天性心脏病(先心病)是目前全球最常见的出生缺陷。心脏磁共振成像对先心病患者的诊断、评估和随访起着至关重要的作用。儿童心脏磁共振扫描仍具有一定挑战性。血池对比剂具有高弛豫性和长血管半衰期的优点,在延长采集时间窗、减少对比剂剂量的同时还能够改善图像质量和提高诊断性能。本文主要对磁共振血池对比剂在儿童先心病诊断方面的应用进行综述。

先天性心脏病青少年过渡期准备度的横断面调查及影响因素分析

背景随着医疗水平提高,越来越多的先天性心脏病(CHD)患儿存活并进入成年。从青春期向成人的过渡期准备度体现了CHD青少年的过渡能力以及对疾病的自我管理能力,目前缺乏我国CHD青少年过渡期准备度的现况数据。目的 评估CHD青少年的过渡期准备度,了解目前国内CHD青少年向成人医疗过渡的准备现状及过渡能力,并分析过渡期准备度的影响因素,为形成个体化的过渡期护理策略提供依据。设计横断面调查。方法 纳入2018年1月1日至2023年8月31日于复旦大学附属儿科医院心胸外科随访、10~18岁的CHD青少年及其主要照护者,向其发放一般资料调查表、STARx自我管理和过渡准备情况评估量表(简称STARx量表)自评版及父母版、中文版鲁汶先天性心脏病知识问卷(LKQCHD)及一般自我效能感量表(GSES),评估CHD青少年过渡期准备度现状,并采用单因素和线性回归分析其影响因素。主要结局指标STARx量表得分。结果 共发放216份问卷,剔除无效问卷64份,用于分析的问卷152份。STARx量表自评版和父母版总体得分分别为(36.74±7.95)和(36.36±7.70)分,差异无统计学意义(P=0.557)。82.2%(125例)的CHD青少年过渡期准备度得分处于中低水平(<44分),仅17.8%(27例)的CHD青少年过渡期准备度处于高水平(≥44分)。63.2%(96例)的主要照护者表示更愿意留在儿童医院进行随访。对CHD青少年过渡期准备度得分的影响因素进行线性回归分析,结果显示,CHD青少年疾病知识正确率、年龄、是否独生子女及自我效能水平是影响CHD青少年过渡期准备度的主要因素。CHD青少年年龄越大、CHD疾病知识掌握程度越高、自我效能水平越高以及非独生子女的过渡期准备度水平越高。结论 国内CHD青少年过渡期准备度中等,主要受CHD青少年疾病知识水平、年龄和自我效能感影响,同时患儿家长对医院转移意愿不强烈。

前门控技术在复杂先天性心脏病的应用价值

目的探讨128层CT前门控技术在复杂先天性心脏病的临床应用价值。方法收集我院进行小儿先天性心脏病CTA检查并进行手术治疗的56例患者,随机分为2组,A组为前门控扫描模式28例,B组为非门控扫描模式28例。比较两种扫描方式在复杂先天性心脏病中显示心内结构和病变的能力、图像质量、辐射剂量的差异。结果A组有效辐射剂量为0.91±0.16msv,B组为1.05±0.25msv,A组患儿所受的有效辐射剂量低于B组;A组图像质量主观评分高于B组,A组术前诊断准确率为96.0%,漏诊3例,B组诊断准确率为86.8%,漏诊12例,误诊1例,A组术前CT诊断准确率大于B组。结论128层MSCT前门控技术在小儿复杂先心病中既可以降低患儿所受的有效辐射剂量,又能清楚显示心内外结构和病变,提倡推广使用。

以三尖瓣下移畸形为主的复杂先心病合并脑梗死1例

三尖瓣下移畸形是一种罕见复杂的先心病,常累及三尖瓣及右心室,可合并其他心内畸形。当合并房间隔缺损时,可引起反常性脑栓塞。本文报道1例59岁女性患者,因脑梗死症状就诊而首次诊断为复杂先心病(三尖瓣下移畸形合并房间隔缺损、室间隔缺损并膜部瘤)。临床上引起脑梗死的原因很多,筛查病因尤为重要。本文通过回顾患者的诊疗经过和相关文献,旨在提高临床医师对三尖瓣下移畸形的认识,有助于医师制定防治策略,改善患者预后。

肺动脉环缩术治疗小儿心力衰竭二例

本文报道2例于2021年12月至2022年1月期间在中国医学科学院阜外医院接受肺动脉环缩术治疗的小儿心力衰竭病例。1例患儿8个月大,术前超声心动图及心脏磁共振成像提示左心室心肌致密化不全、左心功能不全、室间隔缺损、房间隔缺损。另1例患儿4个月大,超声心动图考虑左心室心肌致密化不全、左心功能不全、主动脉弓缩窄。经肺动脉环缩术治疗后,两例患儿左心功能及形态均显著改善,未发生严重手术相关并发症。肺动脉环缩术在小儿心力衰竭治疗的个例经验中表现出比药物治疗更满意的疗效和安全性,尤其对于药物无法控制的小儿心力衰竭,具有一定的推广价值,但仍需更多临床数据支持。

儿童复杂性先天性心脏病超声心动图与多层螺旋CT检查及应用的临床效果观察

目的:观察儿童复杂性先天性心脏病超声心动图与多层螺旋CT检查及应用的临床效果。方法:回顾性分析开封市中心医院2011年1月-2022年1月收治的50例患有复杂性先天性心脏病儿童的临床资料。所有患儿入院时均进行超声心动图与多层螺旋CT检查,以手术病理为金标准,分析两种检查方式对复杂性先天性心脏病的诊断效果。结果:50名患儿中,共发现178种心脏畸形,包括71种心内畸形及107种心外畸形。多层螺旋CT对心外畸形的检出率为98.13%,高于超声心动图的84.11%,差异有统计学意义(χ^(2)=11.321,P<0.05)。多层螺旋CT检查对心内畸形的检出率为95.77%,超声心动图检出率为92.96%,差异无统计学意义(χ^(2)=0.133,P>0.05)。结论:多层螺旋CT和超声心动图对复杂性先天性心脏病的阳性检出率较高,但多层螺旋CT在诊断心外畸形方面优于超声心动图。

疼痛护理在小儿先天性心脏病重症监护室护理中的应用效果

目的探讨疼痛护理在小儿先天性心脏病重症监护室护理中的应用效果。方法选取2020年1月至2023年1月兰陵县人民医院重症监护室收治的60例先天性心脏病患儿为研究对象,按照随机数字表法将其分为对照组(30例)、观察组(30例)。对照组采取常规护理,观察组采取疼痛护理,比较两组的疼痛指标、心理状态和家长总满意率。结果观察组疼痛程度低于对照组,哭闹持续时间、疼痛面容持续时间、重症监护时间短于对照组(P<0.05);干预后,两组儿童抑郁障碍自评量表(DSRSC)、儿童焦虑性情绪障碍筛查表(SCARED)评分均降低,其中观察组DSRSC、SCARED评分低于对照组(P<0.05);观察组家长总满意率高于对照组(P<0.05)。结论疼痛护理在小儿先天性心脏病重症监护室护理中的应用效果显著,能够缓解疼痛,调节情绪,提高家长满意度,值得推广。

Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases

Background： Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. Methods： Clinical and hemodynamic data from 40 pediatric patients （24 boys and 16 girls） with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis （PVS）, valved conduit stenosis, pulmonary artery stenosis （PAS）, and supravalvular pulmonary stenosis （SVPS）. Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. Results： Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best （100%）, followed by the PVS group （90.9%） and the PAS group （85.7%）. There were a total of two transient complications （5.0%）. Conclusions： Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.

Predictive value of C-reactive protein and the Pediatric Risk of Mortality Ⅲ Score for occurrence of postoperative ventilator-associated pneumonia in pediatric patients with congenital heart disease

Importance:Ventilator-associated pneumonia (VAP) is one of the most common complications after cardiac surgery in children with congenital heart disease (CHD).Early prediction of the incidence of VAP is important for clinical prevention and treatment.Objective:To determine the value of serum C-reactive protein (CRP) levels and the Pediatric Risk of Mortality Ⅲ (PRISM Ⅲ) score in predicting the risk of postoperative VAP in pediatric patients with CHD.Methods:We performed a retrospective review of clinical data of 481 pediatric patients with CHD who were admitted to our pediatric intensive care unit.These patients received mechanical ventilation for 48 hours or longer after corrective Surgery.On the basis of their clinical manifestations and laboratory results,patients were separated into two groups of those with VAP and those without VAP.CRP levels were measured and PRISM Ⅲ scores were collected within 12 hours of admission to the pediatric intensive care unit.The Pearson correlation coefficient was used to evaluate the association of CRP levels and the PRISM score with the occurrence of postoperative VAP.A linear regression model was constructed to obtain a joint function and receiver operating curves were used to assess the predictive value.Results:CRP levels and the PRISM Ⅲ score in the VAP group were significantly higher than those in the non-VAP group (P < 0.05).Receiver operating curves suggested that using CRP + the PRISM Ⅲ score to predict the incidence of VAP after congenial heart surgery was more accurate than using either of them alone (CRP + the PRISM Ⅲ score:sensitivity:53.2%,specificity:85.7%).When CRP + the PRISM Ⅲ score was greater than 45.460,patients were more likely to have VAP.Interpretation:Although using CRP levels plus the PRISM Ⅲ score to predict the incidence of VAP after congenial heart surgery is more accurate than using either of them alone,its predictive value is still limited.

Prediction of Pulmonary Arterial Pressure Level after Repair of Congenital Cardiac Communications and Discharge from the Hospital: Role of Down Syndrome and Early Postoperative Hemodynamics

Background:Postoperative pulmonary hypertension limits the success of surgical treatment in some patients with unrestrictive congenital cardiac communications.Identifying patients at risk of developing postoperative pulmonary hypertension is important to individualize follow-up strategies.Methods:We analyzed a prospective cohort of 52 pediatric patients(age 3 to 35 months)looking for perioperative predictors of mildly elevated pulmonary arterial pressure 6 months after surgery,defined as a systolic pressure greater than 30 mmHg by transthoracic echocardiography.This corresponds to a mean pulmonary arterial pressure of>20 mmHg.Clinical,echocardiographic and hemodynamic parameters were investigated.Perioperative hemodynamics was assessed by directly measuring pulmonary and systemic arterial pressures using indwelling catheters.Early postoperative pulmonary hemodynamics was defined as the mean pulmonary/systemic mean arterial pressure ratio(PAP/SAP)obtained per patient during the first 6 h of postoperative care.Results:Among the factors that were investigated as possible predictors,perioperative hemodynamics and the presence of Down syndrome were initially selected using univariate analysis(p<0.030).Early postoperative PAP/SAP was correlated with PAP/SAP obtained in the operating room just after cardiopulmonary bypass(r=0.70,p<0.001),and it was higher in subjects with Down syndrome than in nonsyndromic individuals(p=0.003).Early postoperative PAP/SAP was the only predictor selected using multivariate analysis.It was characterized as an independent predictor after adjustments for possible confounders.An early postoperative PAP/SAP of>0.35 was 76%sensitive and 74%specific at predicting a systolic pulmonary arterial pressure of>30 mmHg 6 months after surgery(hazard ratio with 95%CI 8.972[2.428–33.158],p=0.002).Conclusion:The hypertensive early postoperative behavior of the pulmonary circulation was strongly but not exclusively associated with Down syndrome,and it was characterized as an independent predictor of altered pulmonary arterial pressure after discharge from the hospital.