新生儿先天性心脏病的早期诊断及干预

目的提高先心病患儿的生活质量,探索先心病的早期诊断及干预措施。方法对1996年1月至2006年4月在深圳市第七人民医院儿科住院临床疑诊新生儿先天性心脏病患儿42例,全部经彩色多普勒超声心动图检查（部分手术）诊断,早期干预,出院后定期随访。结果42例新生儿先心病中,男22例,女20例。胎龄〈37W5例,37—42W37例。出生体重〈2．5kg6例,2．5—4kg35例,〉4kg1例。入院原因有紫绀26例,咳嗽12例,黄疸7例,窒息5例,气促4例,喂养困难3例,呕吐3例。临床表现中心脏杂音37例,占88％;紫绀26例,占62％;呼吸急促14例,占33％;呼吸暂停1例,占2％;窒息5例,占12％;心衰3例,占5％。先心病类型中非青紫型35例,占83．33％;青紫型37例,占16．67％。结论新生儿先心病部分无症状,病情观察很重要,心脏杂音、紫绀、气促、心衰是早期症状。彩色多普勒超声心动图能早期确诊及判断预后;部分先心病有自然闭合的可能;新生儿科医师不仅要早期诊断先心病,尤其是复杂性青紫型先心病,而且要及时处理合并症及帮助家长选择正确手术时机;依赖动脉导管开放而存活的完全性大动脉错位患儿禁止吸氧。     

1999~2003年北京市5岁以下儿童先天性心脏病死亡状况分析

目的:了解北京市5岁以下儿童先天性心脏病死亡的状况。方法:对1999~2003年北京市5岁以下儿童生命监测资料进行分析。结果:1999~2003年先天性心脏病已成为北京市5岁以下儿童的重要死因,其中婴儿期尤其是早期新生儿期死亡率较高。北京地区分布呈现城区死亡人数明显低于近郊和远县的特点。结论:降低先天性心脏病儿童死亡率,应以降低婴儿尤其是7 d内新生儿的死亡率为重点,做到早发现、早治疗。     

产前B超检查对先天性心脏病筛查的应用分析

目的探讨产前B超检查对先天性心脏病筛查的应用效果。方法本文纳入我院2017年1月—2018年6月进行先天性心脏病筛查的500例孕妇,所有孕妇均接受产前B超检查,对孕妇宫内胎儿的胎心结构、形态、方式等进行观察,统计胎儿胎心异常的孕妇,检查确诊后终止妊娠。对胎心检查正常的孕妇进行妊娠结局随访,分娩新生儿接受心脏彩超检查,判断有无先天性心脏病发生,评定结果以新生儿出生后彩超检查为金标准,计算出产前B超检查对先天性心脏病筛查的准确性。结果500例孕妇经产前B超检查,有6例检出胎心异常,比例为1.2%。以心脏彩超检查金标准为依据,有8例新生儿被确认先天性心脏病。产前B超检查对先天性心脏筛查病诊断的准确性为98.8%,敏感度为91.2%,特异性为99.9%,差异无统计学意义(P>0.05)。结论产前B超检查对先天性心脏病筛查的应用效果显著,可有效检出先天性心脏病胎儿,准确性较高,临床应用价值较高,可大大减少缺陷胎儿的出生,值得在超声科中推广应用。     

Non-invasive cardiac investigations in patients awaiting renal transplantation.

Patients with chronic renal failure undergoing renal transplantation have a high prevalence of cardiovascular disease. Invasive investigation may identify those at risk of cardiac death during or after renal transplantation, but which patients should undergo cardiac catheterization is currently not clear. In 95 patients awaiting renal transplantation we assessed the ability of echocardiography and exercise electrocardiography to identify patients at risk of cardiac death. Echocardiography identified impaired left ventricular (LV) systolic function in 20%, severe in 8%. Of the patients with severe LV dysfunction, 25% died before transplantation. Of those undergoing exercise electrocardiography, 44% did not achieve 85% of maximum predicted heart rate. No coronary artery disease requiring intervention was identified by exercise testing. These findings indicate that echocardiography, but not exercise electrocardiography, should be part of the assessment for renal transplantation.     

Heart transplantation in the Netherlands, an option to be considered for 5 children per year on the average]

OBJECTIVE: To assess the number of possible candidates for paediatric cardiac transplantation and the number of available donors. DESIGN: Retrospective. PATIENTS AND METHODS: The population (date of birth 1 January 1980-31 March 1998) of the Department of Paediatric Cardiology of the Wilhelmina Children's Hospital, Utrecht, the Netherlands, was reviewed for possible candidates for cardiac transplantation and the resulting number of candidates was extrapolated to the entire country. Figures of the transplant co-ordination unit of the University Hospital Utrecht over 1993-1997 were obtained, to estimate the number of available donors. Furthermore, an overview was made of the results of paediatric cardiac transplantations reported in centres around the world. RESULTS: A total number of 15 possible candidates could be selected over the studied period. Extrapolated to the entire country, about 5 candidates each year could be expected. Per year 5 donor hearts of children (< 12 yr) and 11 hearts of adult donors with a low body weight (40-65 kg) have been available for paediatric cardiac transplantation. Worldwide 5- and 10-year survival rates reach 60% and 50%, respectively, morbidity seems low and the quality of life can be considered reasonably good. CONCLUSION: The number of expected recipients corresponds with the number of available donors. Together with the promising results reported in transplant centres around the world, this seems to justify the option of paediatric cardiac transplantation for children with end-stage cardiac disease.     

Ventricular assist device implantation as a bridge to cardiac transplantation in two adolescents with end-stage cardiomyopathy and heart failure as a result of anthracycline use

In a adolescent women aged 15 and 17 years respectively, severe heart failure developed within a few months of anthracycline chemotherapy given for osteosarcoma. In the guidelines of the European Society of Cardiology, malignancy with a remission duration of less than 5 years is an absolute contraindication to cardiac transplantation. Neither patient was eligible to receive a ventricular assist device (VAD) as a bridge to cardiac transplantation in the Netherlands, but they were accepted in Germany. One patient received a cardiac transplant 13 months later and at the last follow-up check she was in good health with a remission of 3 years. The other patient developed bone metastases 6 months after the VAD implantation. Cardiac transplantation was not a treatment option for her. Dose-dependent cardiotoxicity is a serious complication of the use of anthracyclines. In severe heart failure the prognosis is often worse than in adjuvantly treated malignancies like osteosarcoma. VAD may therefore be a valid option for patients with severe heart failure after anthracycline use for a malignancy. In cases of sustained remission VAD may be the bridge to transplantation.     

老年肾移植的临床特点及治疗

目的探讨老年(≥60岁)肾移植患者的临床特点及治疗对策。方法回顾分析了自2001年9月~2007年11月我院施行的62例60岁以上肾脏移植患者的临床资料,对其原发疾病、手术的安全性、术前准备、术中及术后并发症的处理等进行分析、总结。结果本组病人的原发疾病中以高血压肾病引起的尿毒症比例较高(24/62);老年患者心脏储备功能差,心胸比均超过0.58,术后容易出现心衰(10/62);所有病例均有不同程度动脉粥样硬化,给手术中血管吻合带来一定难度;高龄患者免疫力相对较低,急性排斥发生率较低(4/62),但感染的发生率相对较高(21/62);充分的术前准备,早期及时处理并发症仍能获得较高成功率(57/62)。结论高龄患者是肾脏移植的不利条件,但并不是禁忌证;重视心脏储备功能差、免疫力相对较低等临床特点,积极预防和及时处理并发症,是提高移植成功率的关键措施之一。     

Effect of cardiac or heart-lung transplantation on the quality of life of the paediatric patient

Despite the increase in the use of heart and heart-lung transplantation as methods of treatment for children with end stage heart or lung disease, there is little documented research about the psychological implications of such procedures or about the effects of transplantation on quality of life. Twenty-eight children were studied before and 3 months after heart or heart-lung transplantation and compared with 28 normal children. Developmental and cognitive function were within the normal range, although performance was at a significantly lower level on a number of parameters compared with the normal group. There were no significant changes in any of the developmental or cognitive parameters after transplantation. Pre-operatively the prevalence of problem behaviour at home was significnatly higher in the transplant group compared with the normal group, but there was a significant reduction in the prevalence of problem behaviour following transplantation. Early postoperative findings indicate an improvement in quality of life after heart or heart-lung transplantation but longer term follow-up is now necessary.     

先天性心脏病患儿脑利肽水平的临床研究

目的探讨先天性心脏病患儿血浆脑利钠肽（BNP）水平与心功能的关系及其临床意义。方法回顾性分析70例先心病患儿的临床资料,根据先天性心脏病的类型分为两组：A组为左向右分流型先心病,B组为发绀型先心病。常见左向右分流型先心病又按心功能分级及肺动脉高压情况分为2类。用化学发光免疫分析法测定血浆BNP水平。同时选取30例正常儿童作为对照组。结果⑴除法洛四联症外,其他类型的先心病患儿血浆BNP水平均高于正常对照组;⑵常见左向右分流型先心病患儿心功能Ⅲ～Ⅳ级合并肺动脉高压者与心功能Ⅰ～Ⅱ级无肺动脉高压者,血浆BNP水平存在明显差异（P〈0.01）。结论血浆BNP水平不仅与心功能存在明显正相关,而且对判断肺循环血流量具有重要意义。     

BNP及NT-proBNP在小儿心力衰竭诊断中的应用

心力衰竭是小儿常见急性疾病,若未及时处理,可危及患儿生命.近年来,小儿心力衰竭,尤其是早期小儿心力衰竭,缺乏简单、经济、实用的辅助检测方法.目前研究发现脑钠肽（BNP）及N端脑钠肽（NT-proBNP）在早期心力衰竭即可增高,故成为心力衰竭的特异性诊断标志物.但尚无统一的心力衰竭诊断阈值标准,而且更缺乏BNP及NT-proBNP在儿童心力衰竭诊断的大样本量研究.随着目前对BNP及NT-proBNP研究的不断深入,其血浆测定值可有望成为诊断小儿心功能的重要辅助检查手段.本文拟就目前BNP及NT-proBNP在小儿心力衰竭诊断中的应用进行综述.     

Safety of DTaP-IPV-HIb-HBV hexavalent vaccine in very premature infants

OBJECTIVES: To assess the clinical safety of DTaP-IPV-HIb-HBV hexavalent immunization in very premature infants and to verify if the first administration of vaccine is by itself a reason for close monitoring hospitalized VLBW infants born at less than 31 weeks' gestation. PATIENTS AND METHODS: Eighty-one preterm newborns less than 31 weeks' gestational age, admitted in the NICU, were eligible to be immunized with hexavalent vaccine under close monitoring, including pre-and post-immunization continuous monitoring of heart rate, oxygen saturation, respiratory rate, resistance index at the anterior cerebral artery and ECG cQT interval. RESULTS: Of the 81 eligible premature newborns, 36 were graduated from the NICU before the least date for immunization, at 7 weeks of age. The other 45 were vaccinated in the NICU and entered the study. Twenty-three of them were under medical treatment for chronic disease at the time of the immunization while 22 were healthy and stable. Five infants (11%) had apnoea/bradycardia/desaturation, related to vaccine administration and required medical support. All five infants were in the group of newborns with chronic disease (21.7% prevalence of adverse reactions in this group). No significant variation of cQT or RI before and after the immunization was observed either in the whole groups of patients or in the five infants who showed cardio-respiratory events related to vaccination. CONCLUSIONS: Hexavalent DTaP-IPV-HIb-HBV immunization is not associated with cardiac electric activity and cerebral blood flow variations in both stable and unstable very premature infants. However, it can cause apnoea/bradycardia/desaturation in premature babies with chronic disease. Therefore, if the baby is in the NICU for chronic diseases at 2 months post-birth, it should be monitored for apnoea, bradycardia and desaturation in association with vaccination. Hospitalized healthy preterm infants without chronic disease and therapy seem to be less vulnerable to cardio-respiratory adverse reactions. Nevertheless, it is advisable to immunize and monitor them at 8 weeks before discharge instead of possibly delaying immunization for several weeks and not monitor them.     

Implantable cardioverter-defibrillator in the treatment of two patients with an increased risk of sudden cardiac death

A 23-year-old female with familial long-QT syndrome and a 48-year-old male with familial dilated cardiomyopathy were given an implantable cardioverter-defibrillator (ICD) as prophylaxis. About half a year after the implantation, there was an appropriate and successful ICD-discharge in both patients in connection with ventricular tachycardia. Treatment with an ICD can be life-saving in patients with cardiac rhythm disorders. The most common indication is ventricular tachycardia or fibrillation due to ischaemic heart disease, but an ICD may also be indicated in patients with cardiomyopathy, congenital heart disease, hereditary arrhythmia or a planned heart transplantation.     

新生儿期先天性心脏病的临床分析

目的 探讨新生儿期先天性心脏病的早期诊断与治疗。 方法 对根据超声心动检查结果确诊并分类的72例新生儿期先心病病例进行回顾性分析。 结果 非青紫型先心病占75.0%(54/72),青紫型占25.0%(18/72);主要临床表现为青紫66.7%(48/72)、心脏杂音63.9%(46/72)及气促61.1%(44/72);其中合并心衰16.7%(12/72),并发其他系统畸形8.3%(6/72);胸片及心电图异常分别为59.7%(43/72)及62.5%(15/24);86.1%(62/72)好转出院;6.9%(5/72)早期转院手术;6.9%(5/72)放弃治疗。 结论 对可疑病例及时作超声心动检查有利于新生儿期先心病的早期诊断;及早诊治并根据病情适时手术可改善患儿的生存质量。     

东莞市不同户籍学龄儿童先天性心脏病患病率及诊疗状况调查

目的调查东莞市6~13岁不同户籍学龄儿童先天性心脏病的患病率及诊疗状况,为东莞市学龄儿童先心病防治策略的制定提供依据。方法采用心脏听诊结合现场心脏彩超检查的方法,对东莞市2011年11月-2012年11月期间所有在读小学生进行先天性心脏病筛检和问卷调查及诊疗状况分析。结果共筛检540 574名小学生,其中本地户籍儿童214 634名(39.7%),外地户籍儿童325 940名(60.3%)。先天性心脏病总患病率为2.14‰,本地户籍和外地户籍儿童患病率分别为1.97‰和2.26‰,两者差异无统计学意义(P0.05)。筛检前已治率在本地和外地户籍儿童分别为63.51%和47.21%(P0.05)。在542例需要进一步治疗的患儿中,本地和外地户籍患儿各为154和388例,分别占各自总患者数的36.49%和52.79%(P0.05)。结论东莞市本地户籍与外地户籍学龄儿童先天性心脏病患病率无明显差异,与国内外报道的相同。年龄横断面先天性心脏病患病率亦无明显差异。但外地户籍患儿的治疗率明显低于本地户籍患儿,提示对大量外来人口子女先心病的诊疗有待进一步加强,以免延误治疗时机。     

原位心脏移植术后早期的血流动力学实验研究

目的通过心脏移植的实验研究,比较双腔静脉法同种异体原位心脏移植术与全心脏移植法同种异体原位心脏移植术的出血量,移植后心脏的早期血流动力学方面的不同。方法供、受体杂种犬16只,将实验动物分成双腔静脉心脏移植术组和全心脏移植术组,每组各8只,分别在全麻、中度低温体外循环下进行手术。记录术中出血量,术后采用GE—vivid7型彩色多普勒超声诊断仪,心电监护仪观察血流动力学指标。结果5例移植心脏自动复跳（双腔静脉心脏移植术组3例,全心脏移植法组2例）,3例移植心脏通过电除颤复跳（双腔静脉心脏移植术组1例,全心脏移植法组2例）,双腔静脉法心脏移植与全心脏移植法出血量对比为（475±20．81ml vs 545±26．45ml）,术后早期血流动力学参数两组均比较稳定。结论提高手术技术,避免吻合口出血,加强心肌保护是心脏移植手术的关键,双腔静脉法心脏移植在出血量方面少于全心脏移植术,两组心脏移植术后早期血流动力学方面无明显差异。     

柳州市2003-2011年5岁以下儿童死亡监测结果分析

【目的】 分析柳州市2003-2011年5岁以下儿童死亡率和死亡原因,寻找儿童死亡变化的趋势与规律,为降低5岁以下儿童死亡率干预措施提供理论依据。 【方法】 对柳州市辖六县、四区2003-2011年5岁以下6 071例儿童死亡监测资料进行回顾性分析。 【结果】 9年监测资料显示,新生儿、婴儿、5岁以下儿童死亡率下降速度均较快。2011年新生儿死亡率为4.58‰,婴儿死亡率7.43‰,5岁以下儿童死亡率9.91‰,与2003年相比,新生儿死亡率下降了6.91%,婴儿死亡率下降了9.73%,5岁以下儿童死亡率下降了10.57%。5岁以下儿童死亡前5位死因顺位依次为:早产/低体重、先天性心脏病、出生窒息、先天异常、意外死亡。 【结论】 加强对基层妇幼保健人员 新生儿复苏等适宜技术培训,大力宣传与指导孕期保健知识,是降低5岁以下儿童死亡率的有力措施,减少出生缺陷的发生,提高人口素质的有力措施。     

风湿性心脏病合并心脏恶液质综合征的外科治疗

目的　探讨风湿性心脏病合并心脏恶液质综合征的外科治疗方法和特点。方法　对 1996年 4月至 2 0 0 2年12月符合心脏恶液质综合征诊断标准的 18例风湿性心脏病患者外科治疗的临床资料进行回顾性分析 ,其中二尖瓣置换术 14例 ,二尖瓣及主动脉瓣置换术 4例 ,18例均同时进行 De Vega三尖瓣成形术。结果　术后主要并发症为低心排综合征及在此基础上并发的多脏器功能衰竭 ,早期死亡 4例 ,均为多脏器功能衰竭。结论　外科治疗可提高风湿性心脏病合并心脏恶液质综合征病人的治疗成功率和长期存活率 ,正确恰当的围术期处理是手术成功的关键。     

儿童肝豆状核变性的筛查研究进展

儿童肝豆状核变性是少数可以治疗的遗传性疾病之一,在我国并非罕见,早期诊断并及时治疗,病情可获有效控制,避免肝移植及神经系统后遗症。本文综述了肝豆状核变性的筛查研究进展,以期引起临床医生对本病的足够重视。     

小儿先天性心脏病介入治疗的麻醉处理

目的研究和探讨不同麻醉方法对小儿先天性心脏病介入治疗的临床效果。方法2007年2月～2009年9月在笔者所在医院接受介入治疗的小儿先天性心脏病患者共108例,随机分为对照组和治疗组,每组均为54例。对照组采用氯胺酮加少量咪唑安定进行麻醉,治疗组采用丙泊酚（静安）加少量氯胺酮进行麻醉。结果对照组和治疗组的手术时间分别为（97±5．3）min和（95±6．2）min,术中SpO2的值分别维持为97％～100％和98％～100％,两组差异无统计学意义（P〉0．05）。对照组清醒时间为（50±6．4）min,治疗组为（15±3．4）min,两组差异有统计学意义（P〈0．01）;对照组麻醉后共有13例出现不良反应,占24．07％;治疗组麻醉后共有2例出现不良反应,占3．7％,差异有统计学意义（P〈0．05）。结论 小儿先天性心脏病患者介入治疗时选用丙泊酚复合少量氯胺酮进行麻醉比用氯胺酮复合少量咪唑安定进行麻醉清醒更快,不良反应少,可安全应用于该类手术。     

Neonatal respiratory distress caused by primary ciliary dyskinesia

Two newborns, both boys, presented with unexplained respiratory distress. One developed recurrent pneumonias in the first neonatal week and was diagnosed with primary ciliary dyskinesia at the age of 2.5 years. The other had respiratory problems besides a situs inversus totalis and was diagnosed with primary ciliary dyskinesia in the neonatal period. Although 65-90% of children with primary ciliary dyskinesia present with neonatal respiratory distress, the disease is often diagnosed after a considerable delay. Primary ciliary dyskinesia should be considered in newborns with unexplained respiratory problems and in children with recurrent respiratory problems. The disease is diagnosed by taking a nasal brush biopsy of the cilia and examining it using electron microscopy or using phase contrast microscopy. Early diagnosis and adequate treatment may prevent further lung damage.