40例先天性心脏病患儿外周血免疫功能的变化

本文对４０例不同类型先心病患儿和１５例正常儿童之外周血免疫球蛋白及Ｔ细胞亚群进行检测，并计算Ｔ４／Ｔ８的比值，以观察先心病患儿免疫调节功能的变化。结果肺充血性先心病儿较正常儿Ｔ。明显降低Ｔ８明显升高，提示肺充血性先心病感染可能与免疫调节功能紊乱有关。     

体外循环对先天性心脏病患儿免疫功能的影响

目的探讨体外循环对先天性心脏病患儿免疫功能的影响。方法测定体外循环组与非体外循环组术前、术后d1、d4、d7、d14 IgA、IgM、IgG及NK活性、IL-2变化。结果体外循环术后上述指标降低,降低程度与体外循环时间有关。结论体外循环降低机体免疫力,时间越长,损害越重。     

泛影葡胺对先天性心脏病患儿肾功能的影响

本文观察了造影剂泛影葡胺对先天性心脏病（ＣＨＤ）患儿肾脏功能的影响。结果发现，虽然造影后血清肌酐，尿素氮、β２—微球蛋白（β２—Ｍ）无明显改变，但尿β２—Ｍ、／血β２—Ｍ比值明显升高（Ｐ＜０．０１），特别是当造影剂总量超过４．５ｍｌ／ｋｇ时，二者的升高更为明显，对紫绀型ＣＨＤ患儿尤其如此。本文讨论了泛影葡胺对ＣＨＤ患儿肾脏功能的损伤作用及其与剂量的关系。     

先天性心脏病患儿血清甲状旁腺素水平观察

目的探讨先天性心脏病(CHD)患儿甲状旁腺功能。方法用放射免疫法(IRMA)测定18例学龄前期CHD患儿血清甲状旁腺素(parathyrin,PTH)水平,并测定其血清Ca2 、Mg2 浓度;17例门诊体检的健康儿童作为对照。结果CHD患儿血清PTH水平(14.87±6.02ng/L)较对照组(19.68±5.76ng/L)明显降低,两者比较差异有显著性(P<0.05),CHD组中有8例(44.4%)患儿血清PTH<10ng/L,而对照组均高于10ng/L;CHD患儿血清Ca2 和Mg2 较对照组略低,但无统计学意义(P>0.05)。结论CHD患儿甲状旁腺素分泌不足,存在亚临床甲状旁腺功能低下。     

儿童先天性心脏病脑功能损害评估

相当一部分先天性心脏病患儿存在不同程度的脑功能损害,其病因包括先天性因素和后天获得性因素.早期准确地对先天性心脏病患儿的脑损伤进行评估相当重要.近年来在神经影像学、神经病理学及精神运动发育和智能评估等方面均取得了新进展,能更好地指导临床医师及时干预,使先天性心脏病患儿的脑功能损害程度减少到最小,提高先天性心脏病患儿的生存质量.     

先天性心脏病患儿弓形体感染的初步研究

为探讨先心病（ＣＨＤ）与弓形体（ＴＯＸ）感染的关系,采用ＥＬＩＳＡ、ＰＣＲ技术分别检测ＣＨＤ患儿血清、心肌体及ＤＮＡ。结果：ＴＯＸ＿ＩｇＧ 阳性率在ＣＨＤ组和对照组之间差异有显著性;而ＴＯＸ－ＩｇＭ阳性率在两组之间差异无显著性。在ＴＯＸ－Ｉｂ3     

先天性心脏病患儿脑干听觉诱发电位的研究

目的：评价先天性心脏病（先心病）对患儿脑干发育的影响。方法：研究了先心病患儿脑干听觉诱发电位（ＢＡＥＰ）反应，并设正常儿为对照组。结果：年龄＜１２个月，青紫型先心病（ＣＣＨＤ）患儿Ⅰ波潜伏期（ＰＬ）正常，Ｉ－Ｖ波峰间潜伏期（ＩＰＬ）较正常儿显著延长，非青紫型先心病（ＮＣＣＨＤ）患儿Ｉ波ＰＬ、Ｉ－Ｖ波ＩＰＬ均显著延长；４～６岁者，ＮＣＣＨＤ和ＣＣＨＤ无反复缺氧发作者Ｉ波ＰＬ和Ｉ－Ｖ波ＩＰＬ与正常儿无差异，ＣＣＨＤ反复缺氧发作者Ｉ－Ｖ波ＩＰＬ较正常儿显著延长，异常率２３％。结论：先心病可延迟婴儿期脑干发育，缺氧发作可损害脑干功能，对缺氧发作者应尽早进行干预     

先天性心脏病合并重症肺炎患儿辅助性T细胞功能的动态变化

目的 探讨儿童左向右分流的先天性心脏病合并重症肺炎时辅助性T细胞亚群Th1、Th2细胞的变化特点,为免疫干预提供依据.方法 采用病例对照研究,选取患儿75例,分为4组:先天性心脏病合并重症肺炎组30例;先天性心脏病组15例;普通肺炎组15例;对照组15例.采用流式细胞术检测细胞内细胞因子IFN-γ、IL-4.结果 对照组、普通肺炎组、先天性心脏病组和先天性心脏病合并重症肺炎组之间外周血CD4+细胞表达IFN-γ水平逐渐降低、IL-4逐渐升高,差异有统计学意义(P＜0.01);各组间IFN-γ/IL-4分别为6.30±3.65、4.14±2.06、1.76±1.23、1.12±1.08,两者比值逐渐下降,差异有统计学意义(P＜0.01).30例先天性心脏病合并重症肺炎的患儿治疗1周后复查,大部分病例随着病情的好转,出现IFN-γ上升,IL-4下降,IFN-γ/IL-4上升,其中IL-4的下降和IFN-γ/IL-4的上升差异有统计学意义(P＜0.05).结论 儿童左向右分流的先天性心脏病合并重症肺炎时外周血CD4+细胞表达Th2型细胞因子占优势,Th1/Th2平衡失调,经治疗,病情改善,Th1/Th2的失衡状态好转.     

体外循环对先天性心脏病术后患儿脑认知功能的影响

先天性心脏病(CHD)是婴幼儿最常见的先天性畸形疾病。随着医疗水平显著提高,CHD患儿出生后的生存率明显提升。然而,CHD术后患儿诸多并发症又是另一种挑战,如心力衰竭、心律失常、肺动脉高压及脑认知功能发育障碍等,其中关于脑认知功能障碍的这一并发症逐渐得到广大学者的认识和重视。目前,已经通过细胞分子学方法、磁共振成像技术、光学成像技术、脑电活动监测技术及神经发育量表等研究方法来探索体外循环对CHD患儿脑认知功能的影响。患儿心脏术后会出现不同程度的脑认知功能障碍,这与体外循环相关的全身炎性反应、脑缺血再灌注损伤、体外循环时间、主动脉阻断时间、深低温停循环时间、温度的动态变化、血液稀释程度及血气管理策略等因素密切相关。现就体外循环对CHD术后患儿脑认知功能的影响进行综述,为临床诊疗提供参考,以改善CHD患儿的预后。     

Beraprost Therapy in Children with Pulmonary Hypertension Secondary to Congenital Heart Disease

We present a report on children with severe pulmonary hypertension secondary to congenital heart disease who received 6 months of beraprost therapy. The children had an increase in intracardiac left-to-right shunt and a reduction of the pulmonary-to-systemic vascular resistance ratio, whereas the pulmonary artery pressure was not significantly changed.     

Noninvasive Ventilation for the Management of Pulmonary Hypertension Associated with Congenital Heart Disease in Children

The use of noninvasive ventilation to support children with secondary pulmonary hypertension has not previously been reported. We present four children with secondary pulmonary hypertension in association with complex congenital and acquired cardiorespiratory anomalies who have been successfully managed in hospital and then discharged into the community on noninvasive ventilation, thus placing them in environments more suited for growth and development.     

Development of Personality and Intelligence in Children with Congenital Heart Disease

One hundred and twenty-eight children with congenital heart disease (CHD) and 89 healthy controls were compared according to their development of personality and intelligence. Presence of physical disability in daily life was the independent variable for dividing children with CHD into two groups: children with physical handicap due to CHD (n = 77) have lower IQ scores than healthy children. Unexpectedly, in the parents' opinion they show no difference in behaviour patterns. On introspective examination, these cardiac patients showed an increased feeling of inferiority and of basic anxiety and a more impetuous behaviour as their way of self-protection, but a reduced need for independence due to parental overprotection was not confirmed. None of these observations could be detected in children with CHD who are physically fit (n = 51).     

Pulmonary Agenesis with Congenital Heart Disease

The combined anomalies of pulmonary agenesis and congenital heart disease are exceedingly rare and a highly lethal association. We describe four cardiovascular operations in three patients with this association. Open-heart surgery was performed in two patients, and palliative heart surgery was performed in two patients. One patient died 100 days after the operation because of severe tracheal stenosis and renal failure, but the other two patients are doing well. Surgical access through a thoracotomy, instead of a median approach, was adequate in all patients. Careful evaluation and a precise understanding of the anatomy are necessary for the surgical management of these patients.     

Psychosocial Outcomes for Preschool Children and Families After Surgery for Complex Congenital Heart Disease

The purpose of the current study was to assess the psychosocial outcomes of preschool-aged survivors (ages 3–6 years) of hypoplastic left heart syndrome (HLHS; n = 13) and transposition of the great arteries (TGA; n = 13). Parents completed the following measures: Pediatric Quality of Life Inventory, Impact on the Family Scale, Parenting Stress Index, Parent Behavior Checklist, and Child Behavior Checklist. Quality of life scores did not differ from those of healthy controls. Parents of children with HLHS reported more negative impact of the child’s illness on the family and more parenting stress than parents of children with TGA. Parents of both groups of children were more permissive in their parenting style than parents of healthy controls. Children with HLHS had higher rates of attention and externalizing behavior problems than children with TGA. The results highlight the need for practitioners working with these children and families to ask about parental stress, family functioning, and behavioral expectations for the child in the context of routine medical/cardiac follow-up.     

Hepatic Cell Necrosis with Congenital Heart Disease in the Newborn

Hepatic cell necrosis is a recognized complication of circulatory failure. Histological examination of the adult liver in such cases reveals centrilobular necrosis and hepatic congestion. Two cases of hepatic cell necrosis in infancy are reported: a four-day-old girl with the hypoplastic left heart syndrome, and a nine-day-old girl with interruption of the aortic arch. In the former, midzonal hepatic necrosis (and not centrilobular necrosis) was demonstrated at autopsy. Both patients exhibited severe hepatic insufficiency, but hepatic function improved rapidly with treatment of the coexistent heart failure. It is suggested that there is milder form of hepatic cell necrosis than that previously reported in autopsies.     

Measurement of Tricuspid Annular Diastolic Velocities by Doppler Tissue Imaging to Assess Right Ventricular Function in Patients with Congenital Heart Disease

We assessed the clinical utility of using diastolic tricuspid annular velocities obtained by Doppler tissue imaging as a noninvasive index of right ventricular function in patients with congenital heart disease. Doppler tissue imaging at the tricuspid annulus and pulsed Doppler echocardiography of the right ventricular inflow were performed in 71 children with congenital heart disease, with and without elevated right ventricular pressure. Cardiac catheterization was performed in all patients with congenital heart disease, and the hemodynamic determinants of the tricuspid annular and inflow velocities were determined. In patients with congenital heart disease, the ratio of the late-to-early diastolic tricuspid annular velocity (Aa/Ea) showed a highly significant correlation with right ventricle pressure/left ventricle pressure (r = 0.79, p < 0.0001), right ventricular end diastolic pressure (r = 0.46, p < 0.0001), and the first derivatives of the change in right ventricle pressure during diastole (r = 0.72, p < 0.0001). However, the late-to-early diastolic tricuspid inflow velocity (A/E) did not correlate with any invasively measured index of right ventricular function. Aa/Ea, derived from tricuspid annular velocities as measured by Doppler tissue imaging, is a valuable, noninvasive tool for detecting an elevated right ventricular pressure in patients with congenital heart disease.     

B-Type Natriuretic Peptide Levels in Congenital Heart Disease

The objective of this study was to evaluate the potential role of B-type natriuretic peptide (BNP) levels in children with congenital heart disease undergoing cardiac catheterization. Measurement of plasma BNP concentration has been shown to be useful in the diagnosis, risk stratification, and management of adult patients with congestive heart failure, but little is known about the role of BNP in children with structural congenital heart disease. We measured plasma BNP levels using the Triage BNP test in patients with congenital heart disease referred for diagnostic or interventional cardiac catheterization. Plasma BNP concentration was measured in 96 children and 11 adults 19 years old (7.9 ± 8.3 years) undergoing heart catheterization for underlying congenital heart disease. BNP levels ranged from <5 to >1300 pg/ml, with a median BNP concentration of 19.0 pg/ml. Baseline BNP concentrations were >100.0 pg/ml on 19 occasions in 17 patients. The pressure difference between the left ventricle and ascending aorta was 10–110 mmHg in 21 patients. BNP concentrations for this cohort ranged from <5.0 to 1060.0 pg/ml and correlated with the degree of left ventricular outflow obstruction (correlation coefficient, 0.661; p = 0.001). This study suggests that with additional research, BNP concentration may prove to be a useful clinical tool in managing children and adults with congenital heart disease.     

Universal Screening for Extracardiac Abnormalities in Neonates with Congenital Heart Disease

Extracardiac or genetic abnormalities (EGA) represent a factor in the morbidity of patients with congenital heart disease. We evaluated the way neonates with CHD are screened at our institution and determined the yield for the screening tests. We reviewed the charts of 223 neonates with structural CHD. Subjects were categorized into 6 groups: univentricular, left-sided obstructive lesions, right-sided obstructive lesions, septal defects, conotruncal defects (CTD), and other. We reviewed which patients underwent cranial ultrasonogram (CUS), abdominal ultrasonogram (AUS), and/or genetic studies (GS) as well as their results. There was a high prevalence of EGA in each group by CUS (32% to 42%), AUS (32% to 69%), and GS (10% to 60%). There was considerable variability in the proportion within each group that underwent screening tests, and the consistency of screening often was not congruent with the likelihood of abnormal results. Approximately 50% of our patients had ≥1 EGA identified, resulting in a cost-yield ratio of $4,508/patient with EGA. Screening for EGA at our institution is not uniform and is often at odds with the prevalence of such patients. Given the high prevalence of EGA, we advocate for a universal screening program for neonates with CHD using cranial/abdominal ultrasonography and genetic testing.