Depression and anxiety levels of the mothers of children and adolescents with left ventricular assist devices

Ozbaran B, Kose S, Yagdi T, Engin C, Erermis S, Yazici KU, Noyan A, Ozbaran M. Depression and anxiety levels of the mothers of children and adolescents with left ventricular assist devices. Abstract: VADs have been used to provide treatment for end‐stage heart failure. Parents may feel overwhelmed with the VAD regimes responsibility and be affected from this process beside children. In this study, we aimed to evaluate the depressive and anxiety symptoms of mothers of the first eight children equipped with a VAD in Turkey. The mothers of eight pediatric patients living with VADs were filled BDI and STAI at first month of VAD implantation (E.I) and secondly six months after their first evaluation (E.II). In E.I, the BDI mean score of mothers was 20.87, in E.II 14.37. STAI‐S mean score was 53.37 in E.I and 43.62 in E.II. The Wilcoxon nonparametric‐paired t‐test revealed significant difference between baseline and end‐point STAI‐S scores (Z: ?2.035; p: 0.042), and for BDI scores (Z, ?1.965; p, 0.049). Prolonged usage of VAD may increase distress in parents. Psychiatric evaluation and support of the primary caregiver is important for the well‐being of the pediatric patients.     

Biventricular support with HeartWare ventricular assist device in a pediatric patient

Use of ventricular assist devices is increasing in the pediatric population. This has included the extended use of adult continuous‐flow devices in the pediatric population. In a minority of cases, biventricular support may be needed. In these situations, biventricular support with continuous‐flow devices can be surgically challenging, and therefore, only few cases have been reported. Here, we present a case of implantation of two HeartWare HVAD devices for biventricular support for a decompensating patient with acute myocarditis as well as present an alternative implantation surgical strategy.     

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目的探讨组织多普勒(TDI)、二维斑点追踪技术(STI)和血清脑利钠肽(BNP)评估室间隔缺损(VSD)合并不同程度肺动脉高压(PH)患儿右心室功能的价值。方法 2008年6月至2010年6月在苏州大学附属儿童医院住院的VSD合并PH患儿68例,根据三尖瓣反流压差测得肺动脉压力程度分为轻度组(23例)、中度组(21例)、重度组(24例)。同时选择24名健康体检者作为正常对照组。采用GEVIVID7仪器测量右室长轴整体收缩期应变(GLS)和应变率(GLSR),三尖瓣环脉冲组织多普勒图测量三尖瓣环收缩期峰值速度(Sa)、等容收缩加速度(IVA)、Tei指数,用M型超声测量三尖瓣环收缩期位移(TAPSE)。采用ELIASA法测血清脑钠钛(BNP)浓度。结果 (1)IVA和TAPSE随着PAH升高而降低,而Tei指数随着PAH增加而增大,3个指标除正常对照组和轻度组间差异无统计学意义外(P>0.05),其他各组间差异均有统计学意义(P<0.05)。Sa在中、重度组间差异无统计学意义(P>0.05),在正常对照、轻度组间差异无统计学意义(P>0.05),其余组间有统计学意义(P<0.05)。(2)右室GLS、GLSR随着PAH升高而减小,GLS在4组间两两比较差异有统计学意义(P<0.05);GLSR中度和重度组与正常对照组和轻度组差异有统计学意义(P<0.05),中重度间及正常对照组和轻度间差异无统计学意义(P>0.05)。(3)血清BNP浓度在VSD组中随着PAH程度的加重而升高,在4组间两两比较差异有统计学意义(P<0.05)。(4)BNP与Tei指数及心功能分级相关性最好。结论 TDI和STI各个参数均能反映VSD合并PH患儿右心室功能,其中BNP和Tei指数与心功能相关性最好,而BNP和GLS可以反映轻度PAH时右室功能受损。     

儿童先天性心脏病合并心肌致密化不全的介入治疗及随访分析

目的探讨儿童先天性心脏病合并心肌致密化不全介入治疗的安全性及预后。方法回顾分析2016年1月至2017年10月收治的先天性心脏病合并心肌致密化不全患儿的临床资料。比较患儿在心脏介入治疗前后,以彩色多普勒超声心动图测量的非致密心肌/致密心肌(N/C)比值及心功能参数的变化;记录并发症发生情况。结果共纳入患儿25例,男9例、女16例,中位年龄为1岁(0.14～8岁);动脉导管未闭22例、房间隔缺损2例、室间隔缺损1例。术前、术后1、6、12个月之间左心室舒张末期内径(LVEDD)-Z值和N/C比值的差异均有统计学意义(P0.05),均以术后12月时为最低;其中6例患儿在随访过程中心肌致密化不全基本消失。但各时间点之间左室射血分数(LVEF)的差异无统计学意义(P0.05)。术前及术后1、6、12个月,患儿N/C比值、LVEDD-Z值与LVEF值均无相关性(P0.05)。术后随访中位时间19个月(12 ～27个月),随访过程中未出现心血管不良事件及心律失常发生。结论儿童先天性心脏病合并心肌致密化不全如满足介入治疗指征,应积极介入治疗以减少左向右分流,减轻心脏负荷,有利于左室心肌发育及心室逆重构。     

Auditory brainstem responses in children with congenital heart disease

BACKGROUND: Cyanotic congenital heart diseases usually lead to growth and developmental delay in children due to chronic hypoxemia and undernourishment that may affect the central nervous system. The auditory brainstem responses are determined to assess the maturation and function of the brainstem. Therefore, we used the auditory brainstem responses to investigate the effect of cyanotic congenital heart diseases on brainstem maturation. METHODS: The auditory brainstem responses were investigated in 45 children (23 cyanotic, 22 acyanotic) with congenital heart diseases and compared with the results of 30 healthy counterparts (all children were aged between 2 months and 15 years). RESULTS: The results of auditory brainstem responses were similar in acyanotic patients and in normal children. The cyanotic patients under 1 year of age had more prolonged I-V interpeak latencies than those of control and acyanotic patients (P < 0.05). There was no difference between all groups older than 1 year of age. In cyanotic children, I-V interpeak latencies showed significant negative correlation with arterial oxygen saturation and partial oxygen pressure (P < 0.05). CONCLUSIONS: Cyanotic congenital heart diseases may cause significant retardation on brainstem maturation due to chronic hypoxemia, especially in infants under 1 year of age, whereas acyanotic congenital heart diseases have no effect on auditory brainstem responses.     

父母亲高同型半胱氨酸血症与子女先天性心脏病的相关性研究

为研究父母亲同型半胱胺酸血症（Hhe）与子女先天性心脏病（CHD）的相关性，选择33例正常儿童父母亲和49例CHD患儿父母亲，应用高压液相色谱检测血浆同型半胱氨酸（HCY）。结果显示，如以15μmol／L为正常上限，CHD母亲组发生Hhe／11／32例（34．38％），对照组未见异常，χ^2检验差异有高度显著性（P＝0．0014）；CHD父亲组发生HHE9／17例（52．94％），对照组仅1例，差异有显著性（P＝0．04）。病例组中有CHD家庭史的父母亲发生Hhe者占83．3％（5／6例），高于无CHD家族史的Hhe发生率36．9％（15／43例），P＝0．035。结果表明，父母亲Hhe与子女CHD有相关性，Hhe可能是子女发生CHD的病因之一；具有CHD家族史的父母亲更易发生Hhe。     

先天性心脏病肺高压患儿血浆肾上腺髓质素浓度的测定及其临床意义:附33例报告

用特异性放射免疫非平衡法测定左向右分流型先天性心脏病 (CHD)患儿血浆肾上腺髓质素 (AM )水平 ,并观察心脏手术后AM的动态变化。结果显示 :CHD肺高压组AM明显高于无肺高压组及正常对照组 (P<0.05 ,P<0.01)。且AM随着肺高压病情加重而升高 ;中、重度肺高压组AM高于轻度肺高压组 ,差异有显著性 (P<0.01)。肺高压组术后AM明显下降 ,与术前比较差异有显著性 (P<0.05 ,P<0.01)。CHD组患儿AM与肺动脉收缩压 (PASP)呈显著正相关 (r=0.77,P<0.05)。本资料表明 ,AM参与了左向右分流型CHD合并肺高压的病理生理过程 ,AM的升高是机体的一种防御性反应 ,对维持肺循环稳定 ,减缓肺高压的发展有积极作用     

先天性心脏病并心力衰竭患儿血浆肾上腺髓质素和内皮素水平变化的意义

目的探讨左向右分流先天性心脏病(CHD)患儿并充血性心力衰竭(CHF)血浆肾上腺髓质素(ADM)和内皮素(ET-1)变化的意义。方法左向右分流CHD并CHF患儿18例。正常对照组20例,检测CHD患儿心力衰竭期和症状、心衰体征消失的恢复期及对照组儿童血浆ADM和ET-1。结果CHF急性期ADM、ET-1明显高于恢复期和正常对照,恢复期ADM或ET-1仍高于正常对照组(P<0.01或0.02)。结论ADM和ET-1均参与了左向右分流CHD并CHF的病理生理过程,观察其血浆水平变化,可能对判断心衰患者病情有一定意义。     

先天性心脏病并心力衰竭患儿血浆氨基末端脑利钠肽前体水平变化的临床意义

目的 观察先天性心脏病(CHD)并心力衰竭(HF)患儿血浆氨基末端脑利钠肽前体(NT-proBNP)水平的变化,分析NT-proBNP与HF严重程度的关系及在心功能评估中的价值.方法 选择本院2008年6月-2011年6月住院CHD患儿46例,并根据纽约大学儿童HF指数(NYU PHFI)的评价标准将其分成3组:无HF组(15例)、轻度HF组(16例)、中重度HF组(15例).选取同期25例健康儿童作为健康对照组.分别留取各组儿童空腹静脉血2 mL,应用ELISA法测定其血浆NT-proBNP水平,同时测定其左心室射血分数(LVEF)、二尖瓣舒张早期流速峰值/二尖瓣舒张晚期流速峰值(E/A值).分析其血浆NT-proBNP水平与HF严重程度及LVEF、E/A值的相关性.结果 血浆NT-proBNP水平与HF严重程度呈明显正相关(r=0.82,P＜0.01),HF程度越重,血浆NT-proBNP水平升高越显著;中重度HF组NT-proBNP水平显著高于轻度HF组(P＜0.01),轻度HF组NT-proBNP水平显著高于无HF组(P＜0.01),无HF组NT-proBNP水平显著高于健康对照组(P＜0.05).血浆NT-proBNP水平与LVEF呈负相关(r=-1.20,P＜0.01).结论 血浆NT-proBNP水平随着HF的严重程度增加而升高,检测血浆NT-proBNP对评估CHD并HF患儿病情严重程度及判断预后具有重要临床意义.     

Ventricular assist devices in children: current achievements and future perspectives

Mechanical circulatory support systems for the treatment of acute and chronic heart failure are now available for use in several clinical situations and are designed for different indications and support times. In children, particularly in small infants, extracorporeal membrane oxygenation and centrifugal pumps have been most widely used in the past. These systems are preferred for support after cardiac operations and for use in patients who have concomitant respiratory failure, but they are suitable for short-term application only and intensive care is obligatory. VADs are designed for long-term application and allow patients to be discharged home. Pneumatic pulsatile VADs have been available in pediatric sizes since 1992. Currently at our institution, 74 children have been supported with pediatric extracorporeal VADs for up to 14 months. In the past five yr, a notable rise in survival has been achieved by improvements in pump design and pre- and post-operative management. We have been able to discharge 78% of the infants under one yr old. In this review, our current VAD experience in children will be presented in the light of improvements in decision-making, device technology, and implantation techniques, and in coagulation monitoring and anticoagulation. Additionally, new developments in the field of pediatric assist devices will be presented.     

Long-term growth of children with congenital heart disease: a retrospective study

This retrospective study evaluates long-term growth of children with congenital heart disease (CHD) and looks for possible relationships between postsurgical catch-up growth and both severity of preoperative growth failure and operation age. Growth data of 123 children with isolated CHD were available. Mean z-scores and 95% confidence intervals for weight, height and weight-for-height were plotted for age-periods as well as for pre- and postoperative periods. Growth of children with a large VSD or a Tetralogy of Fallot was most abnormal and improved but did not normalize after operation. Catch-up growth for length was strongly correlated with severity of the preoperative growth failure ( r = 0.92, p < 0.05) but not with operation age ( r = 0.20, NS). We conclude that surgical correction results in catch-up growth for most individuals. Catchup growth is positively correlated with the severity of the initial growth disturbance and not with age at the moment of surgical correction.     

IgG subclass deficiency in children with congenital heart disease

This study of 66 children with congenital heart disease found 26 (39%) with IgG subclass deficiency, the majority being of the IgG₄ isotype. Conventional immunoiogical assessment (IgG, IgA, IgM, T cell) revealed 21 (32%) with immunodeficiency, while inclusion of IgG subclass assessment revealed a total of 35 (53%) of the 66 children had immuno-deficiency. Children with conotruncal lesions appeared to be predisposed to immunodeficiency affecting T cells and IgG subclasses (especially IgG₄) while those with shunt and stenotic lesions had a broad spectrum of immunoglobulin deficiencies. There was significant correlation between immunodeficiency and proneness to infection in these children (p < 0.01). These results suggest that immunodeficiency is a frequent occurrence in children with congenital heart disease, and that IgG subclass measurements should be added to the diagnostic work-up.     

Evaluation of growth and neurodevelopment in children with congenital heart disease

Background: Children with congenital heart disease are under risk of delayed growth and development. We evaluated physical growth parameters and neurodevelopment in these patients in comparison with normal children and examined the effect of hemodynamic status. Methods: Patients with congenital heart disease (n= 76) and healthy children (n= 51) aged 1–72 months applied to Mersin University Hospital, Mersin, Turkey were included. Patients with heart failure and those requiring intervention or surgery were classified as hemodynamically impaired (HI group, n= 30), and the others, hemodynamically normal (HN group, n= 46). Growth parameters including weight, height, body mass index (BMI), mid‐arm circumference (MAC), and triceps skin fold thickness (TSF) were measured and standard deviations (SD) were determined. Functional development was assessed by Denver Developmental Screening Test‐II (DDST II). Results: MAC and BMI values of the group with impaired hemodynamic status were significantly lower than the hemodynamically normal and control groups (MAC P < 0.05 and BMI P < 0.01). In the DDST II, the group with hemodynamic abnormality had more failures in gross motor and fine motor skills than HN group and controls (gross motor P= 0.011, P < 0.001 and fine motor P= 0.028, P= 0.001, respectively) and more failures in language development than the control group (P= 0.001). Conclusion: The results showed the importance of hemodynamic status in growth and neurodevelopment of children with congenital heart disease. Besides routine growth parameters, more detailed examinations such as BMI, MAC, TSF, and developmental screening tests appear useful in identifying children with cardiac disease who are under risk for delayed growth and development.     

先天性食管闭锁合并简单先天性心脏病新生儿预后危险因素分析

目的探讨先天性食管闭锁(CEA)合并简单先天性心脏病(CHD)新生儿预后不良的危险因素。方法回顾性分析1998年—2013年收治并手术的CEA合并简单CHD患儿的临床资料,包括一般情况、实验室检查、术后并发症等,并比较存活和死亡患儿的临床资料。结果 75例患儿纳入最终研究,存活67例,死亡8例,病死率10.67%。死亡及存活患儿的出生胎龄,出生体质量,入院日龄,手术日龄,手术持续时间,手术前、手术当天、痊愈出院/死亡前最近的一次血常规、肝肾功能、电解质、血气分析的差异均无统计学意义(P均0.05);死亡患儿的呼吸衰竭和心力衰竭的发生比例均高于存活患儿,差异有统计学意义(75%、9.0%,P=0.000;50%、1.5%,P=0.000)。结论 CEA合并简单CDH患儿死亡可能与并发呼吸衰竭和心力衰竭有关。     

Brain abscess in children

Objectives: Brain abscess is a serious life-threatening complication of several diseases. The objective of this study was to look at the clinical profile of patients, predisposing conditions, microbiology and outcome of children suffering from brain abscess.Methods: Thirty children aged less than 15 years were reviewed. There were 15 males and 15 females. The mean age of presentation was 5.6±4.4 years.Results: The duration of illness at the time of admission was 17.6±24.6 days. Typically patients presented with fever, vomiting, headache and seizures. The predisposing conditions found were cyanotic congenital heart disease in 11 (37%) of children, meningitis in 6 (20%), septicemia in 7 (23%), and no underlying cause was found in 5 (17%) children. The most common microbe in children with cyanotic congenital heart disease was of theStreptococcus milleri group (52%). Computerized tomography confirmed the diagnosis and the most common location of the abscess was the parietal lobe of the cerebral hemisphere. All abscesses were large, more than 2 cm in diameter and were aspirated surgically. Excision was performed in 6 children. Five children expired, one due to a intracranial bleeding and the others due to severe cerebral edema and tentorial herniation. Complications were seen in 20 children and 16 had sequelae, hemiparesis in 11 and seizure disorder in 5.Conclusion: Brain abscess is a serious infection with poor outcome if diagnosed late. Delayed surgical drainage has high morbidity and mortality. The threshold for diagnosis should be low, particularly in children with a predisposing condition like cyanotic congenital heart disease.     

Excess of congenital abnormalities in French-Canadian children with neuroblastoma: A case series study from Montreal

Neuroblastoma is one of the most common cancers of childhood. Some studies have shown an excess of congenital abnormalities in children who have been diagnosed with neuroblastoma. In this study we examined the medical records of all children with neuroblastoma seen at St. Justine Children's Hospital between the years 1977 and 1993. A total of 141 children (131 of French-Canadian ancestry) were included in this study. Twelve children (8.5%) had 21 defined congenital abnormalities (1,490 per 10,000 children). This compared with a rate of 444.3 children with abnormalities per 10,000 live births (4.44%) for all congenital abnormalities in the British Columbia Health Surveillance Registry, 1979–1988 (relative risk 1.91, P = 0.03). Six of the 12 children had cardiovascular malformations. These and previous results suggest that there may be a common developmental origin to neuroblastoma and to some congenital malformations. Genes that control development may be worthy of further study in these children. Med. Pediatr. Oncol. 29:272–279, 1997. © 1997 Wiley-Liss, Inc.     

先天性心脏病儿童神经发育异常的预测

相当一部分先天性心脏病患儿存在神经系统发育异常。多种生化及物理学检查指标可用于预测不良的神经发育预后,有助于早期干预和改善先天性心脏病所致的神经发育障碍。近年来,关于先天性心脏病神经发育异常的研究在实验室检测、神经物理学检查、围手术期处理等方面均取得了新进展。文章就预测先天性心脏病神经发育异常的相关因素进行综述。     

Fibrinogen half-life in children with cyanotic congenital heart disease

Fibrinogen half-life was determined in 3 healthy individuals and in 17 children with cyanotic congenital heart disease. Fibrinogen half-life was normal in the healthy individuals and shortened in 9 of the 17 patients. The simultaneously performed coagulation tests were often pathological but did not lead to a clear diagnosis of the haemostatic defect and did not correlate with the fibrinogen half-life. Significant correlations were found between fibrinogen half-life and red cell count (P<0.001), packed cell volume (P<0.001), mean corpuscular volume of the red cell (P<0.001), platelet count (P<0.01), aortic oxygen saturation (P<0.001), base excess (P<0.01), and maximal amplitude of the thrombelastography (P<0.001). The results indicate a definite relationship between the hypoxaemia and the frequently observed disturbance of haemostasis in cyanotic heart disease. The chronic hypoxaemia causes a partly compensated intravascular coagulation with increased fibrinogen metabolism.Supported by Land Nordrhein-Westfalen, Ministerium für Wissenschaft und Forschung