室性期前收缩心电图定位及形态的临床评价

目的探讨室性期前收缩心电图定位及形态与临床诊断的关系。方法对109例小儿室性期前收缩定位及形态(QRS波间期、电压、期前收缩指数、室性期前收缩频率等)与有无器质性心脏病关系进行比较分析。结果室性期前收缩定位显示器质性心脏病室性期前收缩多来自左室,无器质性心脏病多来自右室,两组比较有显著性差异(χ2=37.25P<0.01)。器质性心脏病组除出现室性期前收缩外,其他异常心电图的程度及发生率远高于无器质性心脏病组,两组比较有显著性差异(χ2=18.64P<0.01)。结论小儿室性期前收缩定位对判断该室性期前收缩是否有病理意义具有参考价值,室性期前收缩外有其他明显异常心电图改变,显示有病理性意义。室性期前收缩患儿临床无症状,且心电图不伴其他异常改变者,可考虑功能性室性期前收缩。     

先天性心脏病肺动脉高压患儿左室舒张功能与血浆脑利钠水平的相关性研究

目的：探讨先天性心脏病 (CHD)合并肺动脉高压 (PAH) 患儿脑利钠肽（BNP）水平及与左室舒张功能的关系。方法：对95例CHD继发有PAH的患儿和42例无PHA的CHD患儿（对照组）的多普勒超声心动图资料与其血浆BNP水平进行对比分析。结果：与对照组相比, PAH组的左室舒张末内径（LVDd ）、右室舒张末内径（RVDd） 和肺动脉内径（PAd）明显增大(P<0.05),三尖瓣返流（VTR）速度增快及肺动脉收缩压（PASP）升高(P<0.05)。与对照组比较,PASP组患儿二尖瓣口多普勒血流频谱A峰流速（AV）、A峰流速积分（AVI）和E峰流速积分（EVI）及AV/EV和AVI/EVI比均逐渐明显增大(P<0.01);左室等容舒张时间明显延长(LIVRT)(P<0.05)。血浆BNP水平随着PASP增高而升高,与对照组相比差异有显著性（P<0.01）。PAH组先心病患儿其肺动脉压与二尖瓣口血流频谱AV/EV比值呈正相关（P<0.01）,二尖瓣口血流参数与血浆BNP水平亦呈正相关（P<0.01）。结论：CHD合并PAH患儿左室舒张功能与血浆BNP水平呈正相关;BNP在PAH引起左室舒张功能障碍的发生发展过程中发挥了重要作用。［中国当代儿科杂志,2010,12（1）：13-16］     

支气管哮喘患儿心功能变化及其临床意义

目的：探讨支气管哮喘患儿发作期左、右心功能变化及其与哮喘严重程度的关系。方法：用美国Acuson128XP/10型彩色多普勒超声诊断仪对24例危重、极危重哮喘患儿(危重组)及40例非危重哮喘患儿(普通组)进行左、右心功能测定,并与34例正常儿童(对照组)进行比较。结果：非危重组哮喘患儿在发作期以右心舒张功能受损为主,右室收缩功能亦受损,左心收缩及舒张功能正常;危重组哮喘患儿以左心收缩功能受损为主,左室舒张功能受损,右心收缩及舒张功能亦受损。结论：支气管哮喘患儿存在一定程度的心功能受损,并与哮喘严重程度密切相关。     

高海拔地区重症肺炎婴幼儿心脏功能检测的意义

目的探讨高海拔地区重症肺炎婴幼儿左右心室收缩及舒张功能变化的特点。方法应用彩色多普勒超声诊断仪测量(海拔2260 m)2000年1月~2005年1月本院住院30例(男16例,女14例)西宁地区婴幼儿重症肺炎和32例(男17例,女15例)健康婴幼儿左右心室收缩及舒张功能参数,并进行比较分析。结果病例组右室射血前期/射血加速时间(RPEP/AT)明显延长,右心室射血分数(RVEF)、肺动脉最大血流速度(Vpa)降低,右心室(RV)、右心房(RA)扩大。二、三尖瓣A峰、A/E增高,左室等容舒张时间(LIRT)、右室等容舒张时间(RIRT)明显延长(Pa<0.01)。结论高海拔地区婴幼儿重症肺炎存在肺动脉高压,及明显右心室收缩功能及左右心室舒张功能异常,左心室收缩功能无明显异常。     

Tei指数和三尖瓣血流频谱评价左向右分流型先天性心脏病患儿右心功能的价值

目的探讨Tei指数和三尖瓣血流频谱评价左向右分流型先天性心脏病(CHD)患儿右心功能的价值。方法根据心室负荷将47例左向右分流型CHD患儿分为左心型组(30例)和右心型组(17例),应用三尖瓣血流频谱及Tei指数评价右心室功能,并与50例正常儿童进行对比研究。结果与正常组比较,CHD患儿42例三尖瓣血流频谱呈双峰型,均为舒张早期最大流速(EV)/舒张晚期最大流速(AV)>1;左心型组AV、A波流速积分(AVI)升高,右心型组EV、AV、E波流速积分(EVI)、AVI、E波减速时间(EDT)增大;左心型组右室等容舒张时间(IRT)和等容收缩时间(ICT) IRT延长;右心型组ICTI、RT和ICT IRT延长,右室射血时间(RVET)降低;两组CHD患儿右室Tei指数均增大;Tei指数与ICTI、RTI、CT IRT间呈显著性正相关(r=0.642,0.734,0.862 P均<0.01),与RVET间呈负相关(r=-0.481 P<0.01)。结论Tei指数能更简便、敏感、准确、综合评价右心室整体功能,与三尖瓣血流频谱结合可更全面了解CHD患儿右心功能。     

米力农对先天性心脏病并肺动脉高压及心力衰竭患儿的术前干预作用

目的 探讨米力农对小儿先天性心脏病(CHD)并肺动脉高压(PAH)及心力衰竭(CHF)术前治疗的效果.方法 收集2006年1月-2008年7月上海交通大学附属儿童医院ICU收治的CHD患儿40例.年龄1个月～3岁.均为左向右分流型CHD,并PAH及CHF.将患儿随机分为研究组与对照组,各20例,二组病种、年龄、心功能及PAH程度比较无显著性差异.二组患儿均应用强心、利尿及扩血管治疗,另外,研究组20例加米力农静脉维持[0.5 μg/(kg·min)],对照组20例加多巴胺和多巴酚丁胺各5μg/(kg·min)静脉维持.分别于用药前、静脉维持用药72 h及停药后2 h通过彩色多普勒超声心动图检测二组各项心脏收缩、舒张功能及肺动脉压力指标.结果 研究组用药72 h各项心脏收缩功能指标(心脏指数、左室射血分数及左事短轴缩短率)和舒张功能指标(左室收缩时间间期、右室收缩时间间期及二尖瓣E峰和A峰比值)均显著优于对照组(Pa<0.05),肺动脉压力下降程度显著高于对照组(P<0.05);研究组改善心功能有效率显著高于对照组(P<0.05).结论 米力农对CHD并PAH及CHF患儿具有正性肌力和扩血管作用,能明显改善心功能和降低肺动脉压力,为手术纠正心脏畸形提供良好时机.     

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目的探讨重症肺炎时血清脑钠素(BNP)的变化及其与心脏收缩功能的关系。方法对2002年8月至2004年4月在山东省立医院确诊的70例肺炎患儿及20例正常健康儿行超声心动图检查,同时将50只大鼠制成肺炎大鼠30只和正常大鼠20只,也行上述检查;采用酶联免疫吸附法检测上述各组BNP水平。结果肺炎心衰组患儿及左室射血分数(LVEF)下降大鼠组血清BNP水平显著高于健康对照组及正常大鼠对照组,且与LVEF、左室短轴缩短率(LVFS)呈负相关(P<0.05);肺炎心衰组患儿与健康组患儿比较,LVEF下降大鼠与正常大鼠比较,主动脉血流峰值流速(PFVA)、肺动脉血流峰值流速(PFVP)、主动脉血流速度积分(Viao)、肺动脉血流速度积分(Vipa)、LVEF、LVFS显著减少(P<0.05)。结论严重肺炎可引起心衰,血清BNP测定可作为诊断心功能不全的生化指标。     

超声心动图评价心力衰竭患儿左、右心舒张功能

目的　探讨充血性心力衰竭（ＣＨＦ）患儿左、右心舒张功能及其相互关系。方法　对５１ 例ＣＨＦ患儿和６０ 例正常对照儿童用多普勒超声心动图测量左、右室充盈参数。结果　与正常对照组比较,ＣＨＦ组患儿左、右室充盈参数均显示舒张受损,且左、右室充盈参数呈良好相关。结论　ＣＨＦ组患儿均存在左、右心舒张功能障碍,且程度类型相似     

肺炎心衰时脑钠素改变及其与心功能关系的临床与实验研究

目的 探讨重症肺炎时血清脑钠素(BNP)的变化及其与心脏收缩功能的关系。方法 对2002年8月至2004年4月在山东省立医院确诊的70例肺炎患儿及20例正常健康儿行超声心动图检查,同时将50只大鼠制成肺炎大鼠30只和正常大鼠20只,也行上述检查;采用酶联免疫吸附法检测上述各组BNP水平。结果 肺炎心衰组患儿及左室射血分数(LVEF)下降大鼠组血清BNP水平显著高于健康对照组及正常大鼠对照组,且与LVEF、左室短轴缩短率(LVFS)呈负相关(P<0.05);肺炎心衰组患儿与健康组患儿比较,LVEF下降大鼠与正常大鼠比较,主动脉血流峰值流速(PFVA)、肺动脉血流峰值流速(PFVP)、主动脉血流速度积分(Viao)、肺动脉血流速度积分(Vipa)、LVEF、LVFS显著减少(P<0.05)。结论 严重肺炎可引起心衰,血清BNP测定可作为诊断心功能不全的生化指标。     

高原地区小儿右室肥厚型心肌病20例

对高原地区20例小儿右室肥厚型心肌病（RNCM）的临床、ECG、胸片、超声心动困（UCG）及1例尸解资料进行分析，结果显示发病以婴幼儿、男性多见，临床表现多为心力衰竭（心衰），UCG示右室及室间隔肥厚（100％），左室肥厚（55％），不伴有肺动脉高压，右室舒张功能受损，合并右室扩大者预后差。     

Symptomatic heart disease in infants: Comparison of three studies performed during 1969–1987

We compare the data of three studies of hospitalized infants with cardiac disease performed between 1967 and 1987. The studies were from the New England Regional Infant Cardiac Program (1967–1974), the Brompton Hospital (1973–1982), and the Northern Great Plains Regional Cardiac Program (1982–1987). Considering differences in classification among the studies, the distribution of cardiac anomalies during the first year of life is similar among the three studies. Although about 30% of infants are admitted during the first week of life and nearly 40% between 3 and 6 months, the proportion of infants admitted between 6 and 12 months was larger (25%) in the latest than in the earliest study (10%). There were also differences in the distribution of the diagnoses at various ages, reflecting changes in the patterns of care during the three eras.     

Atrial septal defect in Malta

OBJECTIVE: To study diagnostic and surgical trends in atrial septal defect (ASD) in a population-based study, and estimate birth prevalence and spontaneous closure rates. METHODOLOGY: All patients in Malta diagnosed as having ASD and born between 1990 and 1994 were identified from various sources. This took place in the setting of a regional hospital supplying diagnostic services for the entire population. Echocardiographic follow-up was also undertaken for lesions not requiring intervention. RESULTS: A total of 190 patients born in this period were diagnosed as having ASD. Age at diagnosis and age at surgery have decreased significantly over the period under study (P < 0.0001). The mode of diagnosis has become entirely noninvasive, and the perioperative mortality decreased dramatically over time. For the period 1990-94, the incidence at birth for defects not requiring intervention was 2.0/1000 live births, defects requiring intervention 0.4/1000 live births. A total of 92% of 50 defects not requiring intervention closed spontaneously, and the remainder had spontaneously decreased in size on follow-up. CONCLUSIONS: ASD is a relatively benign malformation in which early and noninvasive diagnosis can be achieved, with an extremely low interventional mortality.     

Low donor‐to‐recipient weight ratio does not negatively impact survival of pediatric heart transplant patients

Tang L, Du W, Delius RE, L’Ecuyer TJ, Zilberman MV. Low donor‐to‐recipient weight ratio does not negatively impact survival of pediatric heart transplant patients.
Pediatr Transplantation 2010: 14:741–745. © 2010 John Wiley & Sons A/S. Abstract: A major limitation to success in pediatric heart transplantation is donor organ shortage. While the use of allografts from donors larger than the recipient is accepted, the use of undersized donor grafts is generally discouraged. Using the UNOS database, we wanted to evaluate whether using smaller donor hearts affects the short‐ and long‐term survival of pediatric heart transplant patients. A retrospective analysis of data entered into the UNOS database from April 1994 to May 2008 was performed. Pediatric heart transplant recipients (ages 0–18 yr) with DRWR <2.0 were identified and divided into two groups: Low‐DRWR (<0.8) and Ideal‐DRWR (0.8–2.0). Patients’ demographics, pretransplant diagnoses, age at transplantation, severity of pretransplant condition, and rate of complications prior to hospital discharge after transplantation were noted. Fisher’s exact, chi‐square, and Wilcoxon rank sum tests were used to compare patients’ baseline characteristics. Kaplan–Meier curves and Cox proportional hazard regression were used to compare patients’ survival and to identify independent risk factors for outcomes. There were 3048 patients (204 with Low‐ and 2844 with Ideal‐DRWR). The Low‐ratio group patients were older (8.3 vs. 6.9 yr; p = 0.001), there was a slight male predominance in the Low‐DRWR group (p = 0.055). The Low‐DRWR group had longer transplant wait time than the Ideal‐DRWR group (97 vs. 85 days; p = 0.04). The groups did not differ in race, primary diagnoses, severity of pretransplant condition (medical urgency status, need for ventilation, inotropic support, ECMO, nitric oxide, or dialysis, the PVR for those with bi‐ventricular anatomy), or post‐transplant complications (length of stay, need for inotropic support, dialysis, and rate of infections). The Low‐DRWR patients had less episodes of acute rejection during the first‐post‐transplant month. Infants with DRWR 0.5–0.59 had lower 30‐day survival rate (p = 0.045). There was no difference in short‐ and long‐term survival between the patients with DRWR 0.6–0.79 and DRWR 0.8–2.0. Use of smaller allografts (DRWR 0.6–0.8) has no negative impact on the short‐ and long‐term survival of pediatric heart transplant patients.     

CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES A Clinical Study of 101 Cases

ABSTRACT. The anatomy, natural history, clinical features and symptomatology of 101 cases of congenitally corrected transposition of the great arteries (C-TGA) diagnosed over a 20-year period at Hospital for Sick Children, Toronto, Ontario, is reported. Only 1 patient had no associated lesion and only 14 an intact ventricular septum. Pulmonary stenosis was present in a little more than half of the patients. Left A-V valve involvement existed in 21 patients. In spite of the hich incidence of associated lesions the prognosis was better than expected. Seventy-six patients were alive at the end of the study, no one being critically ill. No death occurred among the patients with intact ventricular septum. Patients with pulmonary stenosis as a group developed symptoms later, were less handicapped and had a better prognosis than those without a pulmonary stenosis. One third of the patients had dysrhythmias. Eight patients having a 3rd degree AV-block. Auscultatory findings were not of too great a help when diagnosing C-TGA. Forty-seven patients had a single second sound, of these 37 had pulmonary stenosis. In 60 % of the patients, where information was available, the second sound was reported to be of normal intensity. Our surgical experience is documented and discussed. Palliative procedures were done with successful results and low mortality. Totally corrective procedures were performed in 9 patients with 4 deaths.     

Evaluation of a child with suspected congenital heart disease

Congenital heart disease (CHD) is the most common congenital malformation in the United Kingdom (UK). Despite major advances in diagnosis and management over the last decade, CHD remains a leading cause of infant morbidity and mortality. Current existing screening tools fail to identify up to 39–50% of children with CHD before discharge from hospital. Pulse oximetry screening has been well studied and seems a promising screening tool. Combined use of pulse oximetry with fetal anomaly screening and routine newborn examination potentially could detect up to 75–92% of critical CHDs in asymptomatic infants. Notably pulse oximetry screening has yet to be incorporated as part of universal screening programme in the United Kingdom. Echocardiography remains the gold standard investigation but may not be always available. A detailed history and thorough clinical examination are of paramount significance in suspected CHD in infants. As the clinical presentation can mimic or overlap with other common conditions in infancy (sepsis, respiratory or metabolic condition), a high index of suspicion with a systematic approach is vital for the timely diagnosis and management.     

Heart transplantation in infants with idiopathic hypertrophic cardiomyopathy

Abstract:  Whereas it is well known that idiopathic HCM can present in newborns and infants, little information is available on HT in this very young age group. We report a series of 17 infants with idiopathic HCM, including two neonates with rapidly progressive severe HF for whom HT was necessary. When HF manifests in a newborn/infant with idiopathic HCM and extreme cavity size reduction, the possibility of a rapidly progressive clinical course should be anticipated and HT may become the only available therapeutic solution.     

非编码RNA与先天性心脏病发生机制研究进展

心脏是胚胎发育和功能发育的第一个器官,形成过程极为复杂,包括心管的融合、环化以及分隔形成等多个步骤,需要相关基因严格按照一定的时间和空间顺序表达,才能最终形成功能完整的四腔心结构.细胞机制的复杂性更多地取决于基因调控,而不是基因本身的数量.随着全基因组分析和RNA-Seq等技术的发展完善,人们发现,人类基因组的很大一部...