中央分流术在治疗复杂先天性心脏病中的应用

目的 总结68例中央分流术的应用及随访,探讨中央分流术在复杂先天性心脏病治疗中的应用及效果.方法 68例患儿,男36例,女32例,年龄1 d～17岁,体重(9.75±5.83)kg.患儿在不同时期进行了中央分流手术,比较手术前后血氧饱和度的变化.术后进行随访,了解肺动脉发育和后续治疗的情况.结果 死亡8例,病死率11.76%.存活患儿症状改善,术后血氧饱和度比术前显著提高(P＜0.05).随访病例肺动脉比术前显著增粗(P＜0.05).26例患儿进行了二次手术,21例为根治手术,5例为再次姑息性手术,2例死亡,再次手术病死率7.7%.结论 作为姑息性手术的一种,中央分流术损伤小、操作简单,能改善症状,提高生活质量,为根治手术创造条件.     

肺动脉环缩术在儿童复杂先天性心脏病中的应用

目的 探讨肺动脉环缩术在儿童复杂先天性心脏病中的应用经验,并对手术效果进行评价.方法 回顾性分析2002年1月～2007年3月进行的32例肺动脉环缩术.男22例,女10例,手术年龄平均9.2个月,体重平均6.1 kg.肺动脉环缩后肺动脉周径平均(28.0±4.1)mm,术后估测环缩近远端压差(43.7±9.0)mmHg.结果 围术期死亡1例,2例患儿再次调整环缩.4例患儿出现围术期并发症.31例患儿随访1个月～5年,22例患儿进行Ⅱ期手术.结论 肺动脉环缩术可限制肺血过多,锻炼左心室,为Ⅱ期施行双心室矫治及双向腔肺分流术赢得时机.     

体肺分流术在先天性心脏病治疗中的应用进展

体肺分流术作为一种增加患儿肺血流量、改善氧合的姑息手术,发明至今无论是手术方法还是手术适应证都经历了一定的演变.该文回顾了体肺分流术的发展,综述了其近、远期手术效果及术后随访等问题,以期促进对体肺分流的认识和进一步研究.     

改良Blalock-Taussig分流术治疗复杂发绀型先天性心脏病的效果

目的 评价改良Blalock-Taussig分流术治疗复杂发绀型先天性心脏病的效果.方法　回顾性分析2006年2月-2008年12月35例在上海交通大学医学院新华医院心胸外科行改良Blalock-Taussig分流术的临床病例,术前均诊断为复杂发绀型先天性心脏病,包括肺动脉闭锁并动脉导管未闭、肺动脉闭锁并单心室、肺动脉闭锁并法洛四联症、重度法洛四联症并肺动脉发育不良及单心室并肺动脉狭窄.男19例,女16例;年龄3个月～6岁;体质量5～19 kg.患儿术前均有严重的发绀症状,胸部X线摄片提示肺血流减少,术前动脉血氧饱和度为(58.7±14.1)%.手术采用直径为4～6 mm的膨体聚四氟乙烯人造血管进行锁骨下动脉-肺动脉分流术.结果　35例患儿术后均获长期生存,改良Blalock-Taussig分流术后心脏超声检查提示人造血管均通畅,分流效果满意,患儿动脉血氧饱和度由术前的(58.7±14.1)%上升至(87.5±5.4)%,有显著改善(P＜0.01).术后随访6～12个月,患儿发绀症状明显减轻,活动耐量增加,未出现人造血管栓塞及其他严重并发症.结论　改良Blalock-Taussig分流术风险较小,对于暂时不具备根治术条件的复杂发绀型先天性心脏病患儿,可起到挽救生命、改善缺氧、促进肺动脉发育的作用,为二期手术或根治手术创造条件.     

全腔静脉-肺动脉连接术Ⅰ期或分期生理矫治复杂先天性心脏病

目的 总结全腔静脉-肺动脉连接术(TCPC)生理矫治复杂先天性心脏病的疗效,探讨选择Ⅰ期或分期TCPC手术的策略.方法 2003年12月至2009年11月,为88例患儿施行了TCPC术.男58例,女30例.低风险病例进行Ⅰ期TCPC术,高风险病例进行分期TCPC术.Ⅰ期手术组41例,平均年龄(8.4±4.5)岁;分期手术组47例,平均年龄(9.0±4.2)岁.Ⅰ期TCPC组中27例采用外管道,14例肺动脉直接下拉与下腔静脉吻合.Ⅱ期TCPC组中42例采用外管道,3例采用心房内通道,2例肺动脉直接与下腔静脉吻合.结果 早期死亡6例,病死率6.8%.Ⅰ期TCPC组死亡5例(4例死于重度低心排征,1例死于呼吸衰竭),病死率12.2%;Ⅱ期TCPC组死亡1例(死于开胸时大出血),病死率2.1%,二组差异无统计学意义(P=0.054).二组的体外循环时间、辅助通气时间、胸管停留时间、住监护室时间、术后住院大数差异无统计学意义.65例随访4个月到6.5年.1例于术后8个月死于严重的房室瓣反流、心力衰竭;1例术后3个月出现感染性心内膜炎,死于多器官衰竭.结论 Ⅰ期和分期TCPC都能达到满意的手术效果.分期TCPC的效果似较Ⅰ期TCPC好.对存在两个或两个以上危险因素者,应选择分期手术.低风险患儿也应尽可能进行分期TCPC手术.     

全腔静脉-肺动脉连接术Ⅰ期或分期生理矫治复杂先天性心脏病

目的 总结全腔静脉-肺动脉连接术(TCPC)生理矫治复杂先天性心脏病的疗效,探讨选择Ⅰ期或分期TCPC手术的策略.方法 2003年12月至2009年11月,为88例患儿施行了TCPC术.男58例,女30例.低风险病例进行Ⅰ期TCPC术,高风险病例进行分期TCPC术.Ⅰ期手术组41例,平均年龄(8.4±4.5)岁;分期手术组47例,平均年龄(9.0±4.2)岁.Ⅰ期TCPC组中27例采用外管道,14例肺动脉直接下拉与下腔静脉吻合.Ⅱ期TCPC组中42例采用外管道,3例采用心房内通道,2例肺动脉直接与下腔静脉吻合.结果 早期死亡6例,病死率6.8%.Ⅰ期TCPC组死亡5例(4例死于重度低心排征,1例死于呼吸衰竭),病死率12.2%;Ⅱ期TCPC组死亡1例(死于开胸时大出血),病死率2.1%,二组差异无统计学意义(P=0.054).二组的体外循环时间、辅助通气时间、胸管停留时间、住监护室时间、术后住院大数差异无统计学意义.65例随访4个月到6.5年.1例于术后8个月死于严重的房室瓣反流、心力衰竭;1例术后3个月出现感染性心内膜炎,死于多器官衰竭.结论 Ⅰ期和分期TCPC都能达到满意的手术效果.分期TCPC的效果似较Ⅰ期TCPC好.对存在两个或两个以上危险因素者,应选择分期手术.低风险患儿也应尽可能进行分期TCPC手术.     

吸入一氧化氮治疗先天性心脏病术后肺动脉高压

目的评价吸入一氧化氮(NO)在左向右分流性先天性心脏病(先心病)术后严重肺动脉高压的治疗效果。方法81例先天性心脏病患儿术后予以吸入NO治疗,治疗指征:肺/体动脉收缩压之比(Pp/Ps)>0.5,氧合指数(PaO2/FiO2)<150mmHg,传统降肺动脉压治疗无效。初始吸入浓度为20×10-6vol/vol,根据血气及血流动力学情况进一步调整,不超过45×10-6vol/vol,同时监测二氧化氮(NO2)浓度及高铁血红蛋白浓度。结果81例吸入NO治疗后肺动脉压力明显下降,从(54.0±15.8)mmHg降至(45.7±12.8)mmHg(P<0.01),而体动脉压力无明显变化,同时PaO2/FiO2明显改善,从100.6±40.1升至135.8±29.7(P<0.01)。监测NO2浓度<1×10-6vol/vol,高铁血红蛋白浓度<2%。未发现明显出血及其他不良反应。结论吸入NO治疗可以安全有效地降低肺动脉压力,提高氧合指数,从而改善先心病合并肺动脉高压的预后。     

先天性心脏病介入治疗的常见并发症及处理

近年来,随着先天性心脏病(先心病)的介入治疗发展迅速,介入器材不断更新,技术方法日臻成熟,应用范围渐扩大,已成为与外科手术同等重要的先心病治疗手段之一。与此同时,先心病介入治疗的并发症与安全性亦越来越引起关注。其并发症发生率各家报道不一:Zeevi等[1]报道急性并发症发     

Systemic to pulmonary arterial shunts for patients with cyanotic congenital heart disease: Current status

The various central vascular shunting operations used for palliation of patients with congenital heart disease plus decreased pulmonary blood flow are discussed. The status of each in current practice is outlined in order to provide an up-to-data reference for the pediatric radiologist. In general, the Waterston shunt is preferred for infants under six months and the Blalock-Taussig anastomosis for patients older than this.Supported by: Grant 260, Children's Bureau, Maternal and Child Health Service, Department of Health, Education and Welfare, Washington, D. C.; and grants from the National Heart and Lung Institute of the National Institutes of Health, the John A. Hartford Foundation, Inc., and the Max Kade Foundation, Inc.     

PICU儿童先天性心脏病心导管术后并发症及处理(附69例分析)

目的总结我院7年来先天性心脏病(先心病)心导管术患儿,需入重症监护病房(PICU)治疗的严重并发症的类型及处理方法。方法回顾性分析入PICU治疗的69例患儿并发症类型、发生率、死亡原因及防治等。结果需入PICU的先心病患儿心导管术并发症包括麻醉后呼吸抑制及呼吸道分泌物增加、肺瘀血、术中心率下降、严重心功能不全和/或低血压或肺水肿、严重心律失常、介入治疗后心包积液、缺氧发作、麻醉后低血压、造影剂过敏等。并发症发生率为1.5%。入住PICU时间为30.5 h,呼吸机使用时间为26.9 h。死亡4例。结论对于不同类型并发症采取不同的处理方法,减少心导管术后严重并发症的病死率。     

小分子RNA在先天性心脏病中的研究进展

小分子RNA（miRNAs）是一类在转录后水平调控基因表达的小分子非编码RNA。它们具有进化保守性、组织细胞特异性和核酸杂交高度特异性。miRNAs 在心肌细胞的增殖、分化和凋亡、心脏神经嵴细胞的迁移、心脏形态发生和心脏图式发育等诸多过程中扮演着重要角色,可为先天性心脏病发生机制的阐明提供一种全新的视角。miRNAs 在先天性心脏病发生机制中的研究可分为临床研究和动物研究两类,该文就这两类研究的研究进展及其研究结论对先天性心脏病机制的阐释以及目前国内研究的现状和局限等方面进行综述。     

Central venous catheterization in infants and children with congenital heart diseases: Experiences with 500 consecutive catheter placements

Summary In a prospective study results of central venous catheter (CVC) placements in a consecutive group of 500 patients with less than 20 kg body weight undergoing cardiac surgery were evaluated. The incidence of previous cardiac surgery was 21% and the incidence of factors preventing the primary puncture of the right jugular or innominate vein was 13.4%. The anesthesiologists were free to select the catheterization technique, site of puncture, and catheter type. All CVC insertions were performed prior to surgery under continuous circulatory monitoring and optimal positioning of the anesthetized patient.Ninety-six percent of all catheterizations were successful, 81% of them on the first attempt. In the 4% of cases where catheterization failed, a CVC had to be placed by the surgeon. Of all catheters, 66% were positioned via the right internal jugular (IJV) or innominate vein (IV), 8% via the left, 16% via an external jugular vein (EJV), and 5% via other veins. Seventy-six percent of CVC insertions were performed with the Seldinger technique. Of the four catheter types used in this study, double lumen catheters were most frequently selected (38%). Placement of 22-ga single lumen catheters was preferred in infants with less than 5 kg body weight, in spite of their tendency to kink. Observed complications (10% arterial puncture, 4% hematoma, and 1% intrathoracic bleeding) never required immediate surgical intervention. Careful selection of appropriate catheters, as well as extensive experience and knowledge of the anatomical structures involved in special heart defects, helped to keep the risk of complications low.     

10公斤以下低体重婴幼儿先天性心脏病的外科治疗

目的：探讨10 kg以下低体重婴幼儿先天性心脏病的外科治疗问题。方法：自2000年1月至2007年12月,外科治疗10 kg以下低体重婴幼儿先天性心脏病105例,体重3.5~10 kg,年龄2个月至3岁,其中合并中度以上肺动脉高压和/或反复肺部感染史56例,复杂先天性心脏病35例。中度低温体外循环下手术88例,常温非体外循环心脏跳动下手术14例,深低温停循环选择性脑保护下手术3例;根治手术101例,姑息性手术4例。结果：住院死亡5例（4.8%）,存活100例,随访2月至3年,根治手术的97例中,室间隔缺损少量残余分流2例,无其他并发症和死亡,均生长发育良好,心功能I级;姑息手术的3例症状明显减轻。结论：随着围术期管理、麻醉、体外循环和外科技术的提高,10 kg以下低体重婴幼儿先天性心脏病外科治疗是安全的,临床效果良好。     

Glucose metabolism and insulin secretion in children with cyanotic congenital heart disease

The aim of the study was to reveal differences in carbohydrate metabolism in children with cyanotic congenital heart diseases (CHD). Thirteen children with diseases of these kinds were investigated with regard to glucose tolerance and insulin secretion and comparisons were made with healthy controls of the same age. Investigations included an intravenous glucose tolerance test, insulin response to the glucose load in plasma and insulin secretion rate. The results reveal lower fasting glucose levels and signs of a higher insulin secretion rate in the relatively few patients in the CHD group where C-peptide measurements were performed, but no differences in glucose tolerance. The reasons for the differences are unclear, but the chronic increases in circulating catecholamines in combination with the impaired nutritional status of these children with CHD are probably the most important factors. We conclude that these divergences in carbohydrate metabolism should be emphasized in the care of children with CHD.     

Hyperuricaemia in cyanotic congenital heart disease

This study examines the exacerbating factors of hyeruricaemia in patients with cyanotic congenital heart disease (CCHD). We studied 59 CCHD patients aged 1 month-30 years. The following variables were assessed: serum uric acid levels, red blood cell count, haemoglobin, hematocrit, partial oxygen pressure and arterial oxygen saturation. Uric acid excretion and renal function were also measured in ten patients with serum levels of uric acid greater than 8 mg/dl (hyperuricaemia group). Serum uric acid level correlated significantly with age and severity of polycythaemia. However, it did not correlate with partial oxygen pressure or arterial oxygen saturation. Uric acid excretion was measured in hyperuricaemia group. Urinary uric acid excretion (24 h) was within normal limits in infants but markedly lower in patients over 15 years of age. The aetiology of hyperuricaemia and decreased uric acid fractional excretion and clearance in infants appears to be secondary to diminished excretion of uric acid in concert with uric acid overproduction. Hyperuricaemia in adolescents and adults with CCHD, however, results mainly from age-related impairment of uric acid excretion.     

Risk of pulmonary tuberculosis in children with congenital heart disease

Children with low-flow congenital heart lesions are reported to have an increased incidence of pulmonary tuberculosis. The aim of this study was to investigate if children with congenital heart disease have an increased incidence of pulmonary tuberculosis and to determine if patients with certain heart conditions are more susceptible to pulmonary tuberculosis than others. This retrospective study over a 6-year period showed that pulmonary tuberculosis was 2.5-fold more common in children with congenital heart disease than in normal children from the same community. Children with congenital pulmonary stenosis had a prevalence equal to those with acyanotic (ventricular and atrial septal defects) and cyanotic (transposition of the great arteries) high-flow heart lesions, whereas there were no cases of tuberculosis in children with low-flow cyanotic heart lesions such as tetralogy of Fallot. Cardiac surgery had to be postponed as a result of pulmonary tuberculosis in 7.2% of all patients in whom it was required. Over the 6-year period of the study, cardiac surgery had to be delayed in 60% of cases with pulmonary tuberculosis and congenital heart lesions so antituberculosis therapy could be completed. Physicians treating children with congenital heart lesions should maintain a high index of suspicion for the development of pulmonary tuberculosis, especially in those with acyanotic and cyanotic high-flow lesions and pulmonary stenosis.     

Living with complex congenital heart disease

A child with complex congenital heart disease in 2008 is very likely to survive a series of surgical and medical interventions, and confront an array of medical and psychosocial stressors that are presently poorly understood. As approaches to medical problems change, careful assessment of those results is essential, and the initial work of the multicentre Pediatric Heart Network is a huge step in the right direction, setting the stage for proper controlled trials of therapies. Major complications, notably ventricular failure, rhythm problems and thromboembolism, will affect nearly one-quarter of survivors, necessitating further interventions. Appropriate educational and psychosocial support for these children and their families is the next challenge for all of us in the field of paediatrics. How ironic would it be to have invested so much in early survival, only to allow the child to fail in life itself.