10 kg以下婴幼儿先天性心脏病合并肺动脉高压的外科治疗

目的 探讨低体重婴幼儿先天性心脏病合并肺动脉高压的手术前准备、手术方法、体外循环管理和术后处理的注意事项,以减少婴幼儿先心病术后并发症的发生.方法 回顾性分析22例10 kg以下先心病合并肺动脉高压(pH)婴幼儿在体外循环(CPB)下行心内直视手术的围手术期处理临床资料.结果 22例患儿年龄11～26月,平均(19.4±8.5)月;体重5.5～10.0 kg,平均(7.6±2.3)kg;室间隔缺损(VSD)20例,其中合并房间隔缺损(ASD)2例,ASD合并动脉导管未闭(PDA)2例;肺动脉高压轻度8例,中度11例,重度3例.所有患儿均手术一期矫治,彻底纠正心内畸形,CPB转流时间32～110 min,平均58min;主动脉阻断时间11～30 min,平均22 min;心脏全部自动复跳,均顺利脱机;1例因术后低心排综合征死亡,病死率4.5%,余均康复出院,无并发症发生.结论 婴幼儿先心病合并肺动脉高压者应充分做好手术前准备工作,手术操作熟练,重视体外循环管理、围手术期处理,尤其是新技术、新药的合理应用,都有助于提高婴幼儿心脏手术的成功率,减少术后并发症的发生.     

微创右腋下小切口心脏不停跳治疗先天性心脏病

目的　总结先天性心脏病的微创治疗经验。方法　自 1999年 10月～ 2 0 0 2年 12月 ,采用微创右腋下小切口 ,在常温体外循环心脏不停跳下行心内直视手术治疗先天性心脏病 3 5例 ,其中房间隔缺损 2 1例 ,室间隔缺损 12例 ,法洛三联征 2例。结果　全组无一例死亡 ,均治愈。结论　微创治疗先天性心脏病安全 ,暴露好 ,手术失血少 ,时间短 ,损伤轻 ,术后病人痛苦小 ,恢复快 ,切口隐蔽 ,美观     

室间隔缺损246例外科治疗临床分析

目的总结室间隔缺损外科手术治疗经验，探讨室间隔缺损的诊断、手术适应证和围术期处理方法，提高手术疗效，减少手术并发症。方法采用中低温体外循环下行室间隔缺损修补术246例；同期矫治其他合并畸形50例。结果全组死亡2例，发生主要并发症5例。结论室间隔缺损外科手术治疗成功率高，并发症少，一经明确诊断，如无手术禁忌证，均应早期手术治疗。     

24例先天性心脏病室间隔缺损手术治疗分析

目的 总结先天性心脏病室间隔缺损的外科手术治疗及术后处理的经验.方法 24例室间隔缺损手术治疗患者,均在低温体外循环下手术,必要时应用深低温低流量及改良超滤技术.直视缝合3例,补片修补21例.结果 所有患者术后无死亡.术后发生并发症6例,发生率25％,其中低心排血量综合征1例,心律失常2例,肺不张1例,肺炎2例,术后复查心脏超声,除1例有少量残余分流外,其余患者心功能均正常,均痊愈出院.结论 室间隔缺损一旦确诊,应尽早手术,术中减轻体外循环损伤,注重脏器功能保护,术后加强呼吸道的管理,预防低心排血量综合征和肺动脉高压危象的发生是减少并发症、降低病死率的关键.     

心脏不停跳室间隔缺损修补术105例体会

目的 探讨心脏不停跳室间隔缺损修补手术技术及其意义.方法 采用体外循环心脏不停跳下对105例室间隔缺损患者施行直视修补术.结果 本组无死亡病例,无严重并发症.术中发现右冠状动脉气栓2例,经对症处理后顺利停机.术后7～10 d痊愈出院.随访时间5个月至2年,胸部X线片示肺血流量减少,心影缩小,无残余分流.结论 体外循环心脏不停跳技术安全可行,适用于多数室间隔缺损患者,避免了主动脉阻断所引起的心肌缺血及再灌注损伤,能有效防止残余漏和传导系统损伤的发生.     

婴幼儿室间隔缺损修复术后早期心律失常原因分析

目的 分析探讨婴幼儿先天性心脏病手术后早期心律失常的危险因素,以期降低其发生率. 方法回顾性分析我院近3年的婴幼儿室间隔缺损手术的临床资料,比较分析早期心律失常发生的情况及原因. 结果分析患儿348例,术后48 h以内有97例出现各型心律失常(27.9%);手术前心胸比值≥0.6、合并肺部感染、合并肺动脉高压、术中主动脉阻断时间≥90 min、术后血氧分压≤60 mmHg、酸中毒、低心排者心律失常发生率更高;患儿年龄、VSD直径、手术方式等因素的不同,其心律失常发生率差异无统计学意义. 结论心律失常是室间隔缺损修复手术后的常见早期并发症,心功能差、体外循环和阻断主动脉时间长、术后低心排是术后心律失常的重要原因.     

微创不停跳心内直视手术体外循环管理体会

目的 探讨微创不停跳心内直视手术的体外循环管理体会.方法 对36例先天性心脏病患者采用微创切口建立体外循环,在常温不阻断升主动脉,心脏不停跳下进行心内直视手术.结果 本组无死亡病例,术后无低心排发生,无严重的心律失常,术后血流动力学稳定,无空气栓塞并发症,也无明显神经系统的并发症.结论 微创不停跳心内直视手术有良好的心肌保护效果,是一种安全有效的手术方法.     

婴幼儿室间隔缺损合并肺动脉高压的外科治疗效果

目的分析60例婴儿室间隔缺损合并肺动脉高压手术结果,探讨影响婴幼儿室间隔缺损合并肺动脉高压外科治疗疗效的因素。方法 2009年2月-2010年3月湖南省儿童医院对60例室间隔缺损合并肺动脉高压患儿采用在中度低温、中低流量体外循环下行VSD修补术,VSD采取补片修补55例,直接缝合5例;ASD和PDA予以直接缝闭,行手术治疗。结果全组患儿治愈59例,1例患儿死于低心排出量综合征,死亡率为1.7%。随访6个月~2年,无其他并发症和死亡病例。结论婴儿室间隔缺损合并肺动脉高压经内科治疗无效时应及时手术治疗,术前积极予以支持疗法,术中精细操作可有效地降低手术患儿死亡率和术后并发症。     

心脏直视术后病人的营养支持

自１９９５年１月至１９９５年１２月间，对３４例先天性心脏病及风湿性心脏病病人的术后营养支持进行研究。其中机械瓣膜置换术１２例，动脉导管未闭结扎术５例，房间隔缺损修补术２例，室间隔缺损修补术１０例，法乐四联症矫治术４例，完全性心内膜垫缺损修补术１例。结...     

小儿继发孔房间隔缺损的外科治疗

心房间隔缺损是小儿临床上常见的一种先天性心脏病，且较容易根治，本文报告22例经手术证实的继发孔房间隔缺损，全组病儿均在低温体外循环下施房间隔缺损直视修补术，临床效果满意，术后无残余分流，痊愈出院，     

Birth weight and cardiovascular malformations: a population-based study. The Baltimore-Washington Infant Study

Mean birth weights were evaluated in infants with D-transposition of the great arteries, tetralogy of Fallot, endocardial cushion defect, hypoplastic left heart syndrome, pulmonary stenosis, aortic stenosis, coarctation of the aorta, ventricular septal defect, and atrial septal defect in a population-based case-control study of congenital cardiovascular malformations in residents of Maryland, Washington, D.C., and northern Virginia (1981-1987). Study subjects were liveborn singletons without extracardiac anomalies. After adjustment for potentially confounding maternal, gestational, and infant factors, significant birth weight deficits were found for infants with tetralogy of Fallot, endocardial cushion defect, hypoplastic left heart syndrome, pulmonary stenosis, coarctation of the aorta, ventricular septal defect, and atrial septal defect. After adjustment, infants with these malformations (except coarctation of the aorta and atrial septal defect) were also significantly more likely than were controls to have low birth weight for gestational age. These findings strengthen previous evidence that certain cardiovascular malformations and low birth weight may be causally related.     

Descriptive epidemiology of selected congenital heart defects, Hawaii, 1986-1999

Congenital heart defects are the most common type of birth defect and contribute the most to infant mortality due to birth defects. This study examined the relationship between several demographic factors and selected congenital heart defects among the unique multiethnic population in Hawaii during 1986-99, using data from a population-based birth defects registry. Rates were significantly higher in 1993-99 than in 1986-92 for transposition of the great arteries and Ebstein's anomaly, and significantly lower for tetralogy of Fallot. Significantly elevated rates were found with maternal age of > or =35 years for ventricular septal defect, atrial septal defect, endocardial cushion defect, and hypoplastic left heart syndrome. When cases with a known chromosomal abnormality were excluded, elevated rates among the older maternal age group remained for ventricular septal defect, atrial septal defect, and hypoplastic left heart syndrome. Whites had significantly higher rates than one or more of the other racial/ethnic groups for Ebstein's anomaly and coarctation of aorta, and significantly lower rates for tetralogy of Fallot, atrial septal defect, pulmonary valve atresia/stenosis, tricuspid valve atresia/stenosis, and anomalous pulmonary venous return. Significantly higher rates were found among males for transposition of great arteries, aortic valve stenosis, and interrupted aortic arch and, among females, for ventricular septal defect, endocardial cushion defect, and anomalous pulmonary venous return. Some of these differences were consistent with the literature while others were not.     

正常胎儿心脏房-室长度比值的超声检测报告

目的 房-室间隔缺损的发病与胎儿染色体异常密切相关.本文旨在应用超声对胎儿心脏房-室长度比值检测,以提高房-室间隔缺损(心内膜垫缺损)患儿的产前筛出率.方法 应用两维超声在胎儿心尖四腔心切面上,对133例孕龄为16～40周孕妇的正常胎儿心脏与心室长度的测量,并用公式房-室长度比=(心脏长度-心室长度)/心室长度,获得房-室长度比值的正常参考值范围.结果 133例正常胎儿心脏房.室长度比为0.48 4±0.08(范围0.32～0.64),该值相对独立,不随孕龄的增加而改变.结论 胎儿心脏房-室长度比是一个简单易得,而且比较可靠的筛查胎儿房室间隔缺损的指标,对于筛选胎儿先天性心脏病具有潜在的应用价值.发现该比值异常时,可指导临床进一步做染色体组型检查.     

Mixed form of total anomalous pulmonary venous connection: apropos of a case

As opposed to partial anomalous of pulmonary venous connection, it is frequent and benigns, the total anomalous of pulmonary venous connection is extremely rare and more serious. The anomalous is severe because all pulmonary venous connection, instead of left heart it go to the right heart. The age of diagnosis is closely tied up anatomics characteristics, so various clinical cases are present. The TAPVC of new born is a surgical emergency, especially where it's block up and release++ cardiorespiratory distress syndrome secondary to OAP. For great children, the total anomlous of pulmonary venous connection can be assumed to a case of atrial septal defect. This study intend to clear up this clinical and anatomical polymorphism and to report an exceptional-form of this congenital anomaly which is mixed total anomalous of pulmonary venous connection.     

围生儿先天性心脏病的发生情况分析

目的：探讨围生儿先天性心脏病的发病情况与发病相关因素。方法：收集绍兴市5家出生缺陷监测医院在2005—2008年监测到的390例围生儿先天性心脏病资料进行分析。结果：围生儿先天性心脏病发生率为86．72／万,位于所有出生缺陷的首位,其类型前3位是房间隔缺损、动脉导管未闭、室间隔缺损。产妇年龄≥35岁组比〈35岁组围生儿先天性心脏病发生率高（P〈0．05）。结论：产前超声检查胎儿心脏是对先天性心脏病预防的最好补充,建议对≥35岁的孕妇把胎儿心脏超声作为常规检查内容。     

先天性心脏病复合畸形的临床介入治疗策略探讨及评价

目的研究先天性心脏病复合畸形经心导管介入治疗的病例选择、手术方法、疗效、安全性及评价。方法选择2008年1月至2008年12月在本院接受同期介入治疗的11例复合型先天性心脏病患儿为研究对象。其中,男性患儿为3例,女性为8例,平均年龄为（4.08±2．64）岁,最小为5个月,最大为10岁。本组先天性心脏病复合畸形类型包括：3例室间隔缺损（ventricular septal defect,VSD）合并动脉导管未闭（patent ductus arteriosus,PDA）,3例房间隔缺损（atrial septal defect,ASD）合并肺动脉瓣狭窄（pulmonary stenosis,PS）,2例房间隔缺损合并动脉导管未闭,1例房间隔缺损合并室间隔缺损,2例动脉导管未闭合并主动脉缩窄（coarctation of aorta,COA）,其中1例为同期介入治疗。介入治疗复合畸形的顺序为：肺动脉瓣球囊扩张术或主动脉球囊扩张术、室间隔缺损封堵术、动脉导管未闭封堵术、房间隔缺损封堵术（本研究遵循的程序符合本院人体试验委员会所制定的伦理学标准,得到该委员会批准,取得受试对象的知情同意,并与试验患儿监护人签署临床研究知情同意书）。结果11例患儿均成功封堵,手术顺利。术后X射线心脏造影及彩超提示,封堵器成形好,未见残余分流。术中、术后均未见严重并发症。其中,1例室间隔缺损术后心电图提示不完全右束支传导阻滞,给予地塞米松治疗后,心电图复查正常。结论介入治疗单纯性先天性心脏病的技术目前比较成熟,成功率高,但复合型先天性心脏病同期进行介入治疗,其操作较复杂,技术难度较高。本研究证明,若掌握恰当手术指征,制定合理操作策略,手术过程仔细、谨慎,介入治疗的可行性、安全性是较肯定的。     

微创切口小儿心内直视手术56例临床分析

目的 报告一组小儿先天性心脏病采用微创切口手术治疗的经验。方法 全组56例病儿中继发孔房间隔缺损（ASD）25例,室间隔缺损（VSD）26例,右室双腔心（DCRV）2例,肺动脉狭窄（PS）3例。右前外侧小切口不停跳、不阻断主动脉心内直视手术23例,胸部正中小切口33例。结果 除1例因术中显露差改作常规切口,全组56例无手术死亡,无严重并发症。病儿术后恢复快,平均住院时间8d。结论 微创切口小儿心内直视手术创新小、美观、痛苦少、恢复快、住院时间短、安全有效,尤其适应于继发孔房缺及膜部或膜周室缺修补手术。     

超声检查胎儿心脏畸形漏诊原因分析

目的:分析产前超声检查胎儿心脏畸形漏诊的原因,探讨提高诊断准确率的方法。方法:回顾性分析该院44 391例经产前超声检查(包括彩超和黑白超)的胎儿资料。中晚孕期超声检查胎儿心脏采用"三切面法"即四腔心切面和左、右室流出道切面,晚孕期黑白超声仅观察四腔心切面。对超声检查发现可疑心脏异常的胎儿再进行详细的超声心动图检查。结果:出生后超声心动图发现产前漏诊胎儿心脏畸形50例,占受检胎儿的1.13‰。其中复杂性先天性心脏病11例(占0.25‰);房间隔、室间隔缺损等先天性心脏病39例(占0.88‰)。室间隔缺损的直径为(3.64±1.42)mm(1.6～6.7 mm),房间隔缺损的直径为(4.40±1.39)mm(2～7 mm)。结论:适宜的检查时间、获取标准的检查切面、仔细辨识图像特征、熟悉病变特点有助于减少漏诊。但由于胎儿先天性心脏病本身的复杂性和超声检查存在的局限性,不可能在产前检出所有的心脏畸形,应向孕妇及家属说明。     

单向活瓣及肺动脉注入前列腺素E1(PGE1)治疗室缺合并肺动脉高压

目的探讨基层医院室缺合并重度肺动脉高压(PH)的外科治疗方法。方法 41例室缺合并肺动脉高压患者采用单向活瓣补片修补室间隔缺损,同时肺动脉内留置导管泵入前列腺素E1(PGE1);术后超声心动图动态监测分流情况,并比较术前术后肺动脉压(PAP)及血气分析指标变化。结果 41例患者无1例死亡,术后2个月内单向活瓣关闭、分流消失;术后PAP明显下降,血气分析指标明显改善。结论单向活瓣补片及肺动脉置管泵入前列腺素E1(PGE1)能有效提高基层医院室缺合并肺动脉高压的治疗效果,增加围手术期安全性。     

Risk comparison for prenatal use of analgesics and selected birth defects,National Birth Defects Prevention Study 1997–2011

PurposeTo compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects.MethodsWe used data from the National Birth Defects Prevention Study (1997–2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects.ResultsCompared to acetaminophen, mothers reporting NSAIDs were significantly more likely to have offspring with gastroschisis, hypospadias, cleft palate, cleft lip with cleft palate, cleft lip without cleft palate, anencephaly, spina bifida, hypoplastic left heart syndrome, pulmonary valve stenosis, and tetralogy of Fallot (aOR range, 1.2–1.6). Opioids were associated with tetralogy of Fallot, perimembranous ventricular septal defect, and ventricular septal defect with atrial septal defect (aOR range, 1.8–2.3), whereas use of both opioids and NSAIDs was associated with gastroschisis, cleft palate, spina bifida, hypoplastic left heart syndrome, and pulmonary valve stenosis (aOR range, 2.0–2.9).ConclusionsCompared to periconceptional use of acetaminophen, selected birth defects occurred more frequently among infants of women using NSAIDs and/or opioids. However, we could not definitely determine whether these risks relate to the drugs or to indications for treatment.