双腔右心室的诊断及治疗体会

目的总结57例双腔右心室的诊断及外科治疗经验。方法术前经超声心动图和／或双源64排CT确诊50例，测右心室高、低压腔压力阶差40～106mm Hg；7例于术中确诊。57例患者中6例经右心房切口疏通右室流出道梗阻，余51例均经右心室切口疏通右室流出道梗阻。57例患者中合并室间隔缺损40例，肺动脉瓣狭窄6例，房间隔缺损3例，动脉导管未闭1例，均于手术中一并矫治。结果1例因合并低心排死亡，20例患者随访1～10年，1例残余流出道梗阻，余无临床症状。结论术前明确诊断识别其病理解剖，有无冠脉畸形，都是矫治双腔右心室的关键。     

成人法乐四联症的外科治疗

目的总结成人法乐四联症根治术外科治疗经验。方法1985年11月~2005年5月收治18岁以上成年法四病人115例,手术根治。所有病例均经右室切口疏通右室流出道(RVOT),同时修补室间隔。跨瓣环RVOT补片74例,单纯RVOT补片37例,1例肺动脉与RVOT分别予以补片,5例右室切口直接缝合。结果术后室间隔残余分流7例(直径3~7 mm),RVOT残余梗阻3例(>50 mmHg)。10例伴重度低心排。死亡6例,死亡率5.22%。结论根据成人法四特点,合理设计手术方案,成人法乐氏四联症根治可取得良好效果。     

心脏内畸形合并感染性心瓣膜炎的外科治疗

目的 探讨心脏内畸形合并感染性心瓣膜炎的诊断及外科治疗特点.方法 回顾1997年2月～2003年2月10例心脏内畸形合并感染性心瓣膜炎患者的临床资料,其中室间隔缺损4例,主动脉窦瘤破裂2例,右室流出道狭窄1例,二尖瓣关闭不全1例,主动脉瓣二叶瓣畸形2例.感染的心脏瓣膜分别为主动脉瓣4例,三尖瓣4例,肺动脉瓣1例,二尖瓣1例.合并瓣膜赘生物9例次,合并瓣膜穿孔4例次,均行外科手术治疗,清除赘生物及修复穿孔,同时行室间隔缺损修补术、主动脉瓣置换术、二尖瓣置换术、右室流出道加宽补片术1例.结果 患者术后7～14 d体温及白细胞计数降至正常,应用抗生素28 d后痊愈出院,全组无死亡病例.结论 心脏内畸形患者并发感染性心瓣膜炎,如发现瓣膜赘生物形成或瓣膜穿孔,应视病情立刻手术或抗感染治疗后手术,术后根据血细菌培养或瓣膜赘生物培养情况应用抗生素28 d,预防人工瓣膜及补片再度感染.     

经食管超声心动图在法洛四联症矫治术中的应用价值

目的：探讨经食管超声心动图在法洛四联症矫治术中的应用价值。方法：完成52例经食管超声心动图（TEE）监测下的法洛四联症矫治术，在手术前、心脏复跳后、以及停机后经食管超声（TEE）检查，作为术前评估、以及观察手术矫治情况。结果：手术矫治前TEE检查，与术前经胸彩色多普勒（TTE）检查以及手术探查矫治后诊断符合率为100％，术后2例室缺残余漏，均〈2mm，未予处理；3例右室流出道残余梗阻、2例三尖瓣重度返流，分别再次转机，予以矫治。50例痊愈出院，2例死亡。结论：经食管超声心动图在法洛四联症矫治术中有着重要意义，可以术前评估，补充和修正诊断，有无室间隔缺损修补后残余漏、右心室流出道疏通情况以及肺动脉瓣、三尖瓣状况，并可在手术中及时矫正，提高手术成功率。     

胎儿心脏五切面在法洛四联症产前筛查中的价值

目的评价胎儿心脏五切面在法洛四联症产前筛查中的应用价值。方法所有受检胎儿均行上腹部胃泡横切面、四腔心切面、左室流出道、右室流出道及三血管五个切面扫查;对31例法洛四联症胎儿期超声心动图特征进行回顾性分析。结果胎儿期法洛四联症的主要声像图特征是室间隔缺损、主动脉骑跨、肺动脉狭窄;胎儿心脏五切面有助于法洛四联症的产前筛查。结论胎儿心脏五切面在法洛四联症的产前筛查中具有重要价值。     

心脏内畸形合并感染性心瓣膜炎的外科治疗

目的探讨心脏内畸形合并感染性心瓣膜炎的诊断及外科治疗特点。方法回顾1997年2月-2003年2月10例心脏内畸形合并感染性心瓣膜炎患者的临床资料，其中室间隔缺损4例，主动脉窦瘤破裂2例，右室流出道狭窄1例，二尖瓣关闭不全1例，主动脉瓣二叶瓣畸形2例。感染的心脏瓣膜分别为主动脉瓣4例。三尖瓣4例，肺动脉瓣1例，二尖瓣1例。合并瓣膜赘生物9例次，合并瓣膜穿孔4例次，均行外科手术治疗，清除赘生物及修复穿孔，同时行室间隔缺损修补术、主动脉瓣置换术、二尖瓣置换术、右室流出道加宽补片术1例。结果患者术后7-14d体温及白细胞计数降至正常，应用抗生素28d后痊愈出院，全组无死亡病例。结论心脏内畸形患者并发感染性心瓣膜炎，如发现瓣膜赘生物形成或瓣膜穿孔，应视病情立刻手术或抗感染治疗后手术，术后根据血细菌培养或瓣膜赘生物培养情况应用抗生索28d，预防人工瓣膜及补片再度感染。     

法洛四联症:1岁以下外科根治术

目的回顾总结1岁以下婴儿法洛四联症(tetralogy of Fallot,TOF)根治术的经验。方法 1999年1月至2005年8月,58例1岁以下TOF患儿的根治手术。合并房间隔缺损8例、动脉导管未闭3 例、冠状动脉异常3例、肺动脉闭锁2例、21-三体综合征2例,既往行改良B-T分流术2例。术中发现室间隔缺损(ventricutar septal defect,VSD)嵴下型56例,干下型2例。VSD经右心室修补48例,经右房修补4 例,经右房、右室共同修补6例。VSD开窗2例,右心室流出道跨瓣环补片39例。结果平均体外循环时间为95．5 min,主动脉阻断时间为53．5 min。平均呼吸机辅助通气时间为30 h,重症监护室(intensive care unit, ICU)平均停留时间为4 d,术后平均住院14 d。延迟关胸3例,术后早期死亡2例(3．4％),死亡原因分别为严重心排血量低和顽固性交界性心动过速。术后早期主要并发症包括:心排血量低(5例),肾衰竭(4 例),室上性、交界性心动过速(2例),Ⅲ°房室传导阻滞(AVB)(1例)。随访3个月至5年,均恢复良好,心功能1-2级。结论婴儿期TOF根治术死亡率低,术后早中期随访并发症少,心功能良好。TOF可常规在婴儿期进行根治。     

经主动脉切口修补干下型室间隔缺损和主动脉瓣置换13例

目的 探讨经主动脉径路修补干下型室间隔缺损,并同期行主动脉瓣置换的外科方法.方法 回顾分析13例干下型室间隔缺损并重度主动脉瓣关闭不全患者,经主动脉切口行室间隔缺损修补及主动脉瓣置换的临床资料.结果 全组无手术死亡者,术后无低心排,无完全性房室传导阻滞,术后及术后3个月随访均无残余分流,肺动脉瓣返流较术前减轻.结论 经主动脉途径修补干下型室间隔缺损可以避免多个切口,修补确切,可以保护肺动脉瓣.     

超声四腔心加左右室流出道和三血管气管切面在先天性心脏病筛查中的应用

目的 探讨超声四腔心加左右室流出道、三血管气管标准切面在先天性心脏病筛查中的应用价值.方法 以胎儿四腔心、左右室流出道、三血管气管标准切面,快速观察胎儿心脏结构以及形态,进行胎儿先天性心脏病筛查.结果 3 567例胎儿确诊18例心脏结构异常,包括三尖瓣闭锁和室间隔缺损各3例,心内膜垫缺损和完全型大动脉转位各2例,两腔心、左心发育不良伴二尖瓣闭锁、三尖瓣关闭不全伴室缺、肺动脉狭窄、法洛四联症、永存动脉干、右位主动脉弓和右室双出口各1例.其中产后彩色多普勒超声检查证实4例,上级医院会诊、引产后尸体解剖证实14例.另有3例肌部室间隔缺损产前检查漏诊.结论 四腔心加左右室流出道、三血管气管标准切面进行胎儿心脏超声筛查可检出大部分心脏畸形,对诊断胎儿先天性心脏畸形具有重要的临床价值.     

一例Ebstein心脏畸形手术的护理配合

三尖瓣下移畸形又称Ebstein畸形,是一种复杂的少见的先天性心脏病,约占先天性心脏病的0.5%-1%,以三尖瓣隔瓣、后瓣下移为特点,形成房化右心室,导致不同程度的三尖瓣关闭不全和右室流出道梗阻.常伴右心房间隔缺损、心室间隔缺损、动脉导管未闭、肺动脉口狭窄或闭锁.我院于2013年3月首次独立实施了1例Ebstein畸形矫治术,取得了较好的效果,现将手术配合要点介绍如下.     

液氮深低温保存同种带瓣血管的临床应用

目的报告应用液氮深低温保存的同种带瓣血管（valved homograft conduit,VHC）纠治复杂心脏畸形的经验。方法患者男27例,女19例,年龄1.9～31（9.5±5.1）岁,体质量10～64（29.5±13.2）kg。病种包括右室双出口15例,矫正性大动脉转位9例,共同动脉干7例,肺动脉闭锁5例,法乐氏四联症4例,完全性大动脉转位3例,先天性主动脉瓣关闭不全2例,法乐氏四联症术后肺动脉瓣重度关闭不全1例。合并畸形包括肺动脉狭窄、房间隔缺损、动脉导管未闭、多发室间隔缺损等。手术均在全麻低温体外循环下进行。结果无手术死亡。早期死亡2例,分别为术后低心排综合症和呼吸道出血。存活44例,随访（32/44）3～96（30±19.5）月,死亡2例,1例右室双出口患者术后26个月死于感染性心内膜炎,另1例为矫正性血管转位患者术后8个月死亡,死因不明。其余30例患者心功能明显改善,超声心动图检查显示吻合口无狭窄,VHC管腔通畅,瓣膜无明显返流。结论液氮深低温保存的VHC是理想的人工材料,用于纠治复杂心脏畸形可获得较好的治疗效果。     

Prolonged survival in a female with untreated tetralogy of Fallot

It has been reported that most patients with untreated tetralogy of Fallot (TOF) die by the time they reach adulthood. We report the case of a 72-year-old female diagnosed by echocardiography and cardiac cathetherization as having TOF and diagnosed at birth with a ventricular septal defect (VSD). During childhood, she was very thin and lacking in physical strength. On first consultation at our hospital, she was suffering from mild dyspnea, classified as NYHA functional class III, and her fingers were clubbed and cyanotic. Her PaO2 was 48.0 mmHg under room air, and hypoxia was recognized. An echocardiography and cardiac cathetherization showed a VSD, hypertrophy of the right ventricle, over-riding of the aorta and stenosis of the right ventricular outflow tract with a pressure gradient of 84 mmHg. There was a bidirectional shunt with 24% flow from the left to right and 43% from the right to left ventricle. Her Qp/Qs was 0.75. Surgical treatment was recommended. However, the patient refused, because her symptoms were alleviated with home oxygen therapy. This report shows the prolonged survival of this 72-year-old female with untreated TOF.     

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.     

Tetralogy of fallot with absent pulmonary valve

BACKGROUND: Absent pulmonary valve with ventricular septal defect is a rare cardiac malformation. The aim of our study is to specify the anatomic characteristics and the clinical and echocardiographic features of this cardiac malformation and to discuss its management. METHODS: We report 8 cases of absent pulmonary valve with ventricular septal defected collected over a period of 24 years. The diagnosis was made during the first year of life in all cases in the presence of respiratory symptoms and/or cyanosis. It was confirmed by cardiac catheterization in 4 cases and echocardiography in 4 cases. RESULTS: Pulmonary vascular obstructive disease, related to aortopulmonary collateral vessels, was noted in one patient who died at the age of 20 years. One patient was lost to follow up and 6 patients were operated. Closure of the ventricular septal defect, widening of the pulmonary tract and insertion of a pulmonary valve were performed in the 6 cases. Reduction procedure of the pulmonary arteries was performed in 3 cases. One death related to early postoperative infective endocarditis was noted. At a mean follow up of 2 years, the 5 survivors are going well with no significant pulmonary stenosis. CONCLUSION: Although named "tetralogy of Fallot with absent pulmonary valve", absent pulmonary valve with ventricular septal defect is different from tetralogy of Fallot by aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory symptoms that can be severe with respiratory distress. Pulmonary arterioplasty eliminate airways obstruction and its results are satisfactory. The need for insertion of a pulmonary valve is debatable.     

低体重婴幼儿先天性心脏病的外科治疗

目的总结低体重婴幼儿先天性心脏病外科手术的经验。方法自2000年1月～2004年12月共为133例15公斤以下的低体重婴幼儿(3岁以下)先天性心脏病患儿施行外科手术治疗;其中5～12个月32例(24.1%),13～24个月46例(34.59%),25～36个月55例(41.35%);体重6～15公斤,其中6～10公斤48例,11～15公斤85例。全组非体外循环动脉导管结扎术29例;体外循环手术104例,其中室间隔缺损修补术64例,常温心脏不停跳继发孔房间隔缺损修补术16例,常温心脏不停跳肺动脉瓣狭窄直视分离术7例,部分行房室间隔缺损矫治术2例,部分行肺静脉异位引流矫治术3例,法乐氏四联症一期矫治术5例,房间隔缺损并右室流出道梗阻4例,其它复合畸形一期矫治术3例。结果全组术后住院死亡6例,死亡率4.5%。术后主要并发症包括低心排综合征,呼吸衰竭,心律失常,肺部感染,出血等。死亡原因包括重度低心排综合征,严重室性心律失常,肺动脉高压危象等。结论心外科、心儿科、麻醉、体外循环和术后监护等人员的密切配合是小儿心脏外科迅速发展的重要前提,心内外科镶嵌治疗成为婴幼儿心脏外科手术成功的重要保证。     

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.     

偏心型封堵器介入治疗嵴内型室间隔缺损临床应用及中期疗效

目的 探讨国产偏心型封堵器介入治疗嵴内型室间隔缺损(VSD)的安全性及中期疗效.方法 2005年1月至2008年7月采用国产偏心型封堵器对26例嵴内型VSD患者行介入治疗.经胸超声心动图提示VSD破口直径3～9mm,平均(4.7±4.5)mm.距肺动脉瓣>2mm,距主动脉瓣>1 mm,并分别于术后1周、1个月、3个月、6个月及12个月进行随访.结果 26例患者中21例封堵成功,5例失败,成功率为80.8%.术后即刻左室造影示少量残余分流2例,微量残余分流3例,均在术后1 d至3个月消失,术后即刻新发完全性左束支传导阻滞(LBBB)1例,余无其他严重并发症发生.封堵术后1周左房内径、左室舒张末期内径即明显缩小(P<0.01),左室收缩末期内径无明显变化,术后1个月及以上各项指标与术后1周比较差异无统计学意义.所有患者均获得随访,随访时间12～48个月.术前合并8例右束支传导阻滞(RBBB),3例LBBB,5例在术后2～14d恢复正常,6例在出院时未恢复正常,包括4例RBBB,2例LBBB.随访1个月时,1例RBBB恢复正常;随访3个月时,1例RBBB恢复正常,余在随访期间持续存在.结论 应用国产偏心型封堵器治疗嵴内型VSD是安全有效的,近中期效果良好,远期疗效尚需进一步临床观察.     

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。     

超声对胎儿单纯室间隔缺损在宫内和产后婴儿期闭合情况的研究

目的应用超声心动图观察胎儿期及产后婴儿期单纯室间隔缺损（IVSD）自然愈合的情况及影响因素。方法选取61例诊断有胎儿单纯性室缺但不愿终止妊娠的孕妇为研究对象,对胎儿进行二维和彩色多普勒超声心动图检查,记录胎儿室间隔缺损的位置、形态、大小、自然闭合的时间和闭合率,出生后存活的婴儿监测室间隔缺损（VSD）上述指标至1岁,计算宫内及产后1年的闭合率及总的闭合率、不同位置室缺的闭合率,并对不同大小室缺的闭合率进行组间比较。结果在61例中,22例（36.1%）宫内自然闭合,9例（14.8%）在产后1年内闭合,30例（49.2%）仍未闭合。室间隔缺损的大小以及缺损的位置与愈合率有关,室间隔缺小于3.0mm的闭合率明显高于室缺为3.1~5.0mm的闭合率（P=0.005）和5mm以上室缺的闭合率（P=0.002）,但室缺为3.1~5.0mm的闭合率与大于5mm的室缺的闭合率之间无显著性差异（P=0.39）。膜部和肌部缺损的闭合率明显高于干下型室缺的闭合率（P≤0.01）,肌部缺损的闭合率高于膜部缺损的闭合率,但两者之间无统计学差异（P=0.65）。结论单纯室间隔缺损有较高的宫内自然闭合率并且在产后婴儿期仍能自然闭合,这种自然愈合的过程与室间隔缺损的位置和大小显著相关。肌部和膜部缺损最容易愈合,干下型不易闭合;小缺损具较高的闭合率,较大的缺损不易闭合。