压力测定在右室双腔合并室间隔缺损手术中的应用

目的总结67例右室双腔合并室间隔缺损患儿的手术治疗经验,介绍压力测定在右室双腔手术中的具体应用。方法回顾性分析1998～2008年本院收治的67例右室双腔合并室间隔缺损患儿临床资料,均在低温、浅低温体外循环下行根治手术,术中常规测定右室、肺动脉压力。结果全组手术死亡2例,病死率为3％。痊愈患者随访3～12个月。经心脏超声心动图评估右室-肺动脉压差均逐步降低,小于20mmHg,存在室间隔残余漏3例,均小于3mm。结论右室双腔合并室间隔缺损患儿术中进行压力测定,可以指导临床手术,术中评价手术纠治效果,提高该病的近远期手术疗效。     

电视胸腔镜辅助下杂交手术治疗右室双出口合并肌部室间隔缺损1例

本院近期采用电视胸腔镜辅助下杂交手术治疗右室双出几合并肌部窒缺1例,疗效满意. 患儿,女,1岁9个月.体重9 kg,身长79 cm,凶自幼发现心脏杂音,反复罹患肺感染入院.     

室间隔缺损远离大动脉开口的右室双出口外科治疗与适应证选择

目的回顾室间隔缺损（VSD）远离2个大动脉开口的右室双出口（double—outlet right ventricle,DORV）的病理解剖特点、手术方法选择和治疗结果,对手术适应证和方法提出优化意见。方法1984年4月至2005年4月共有37例VSD远离2个大动脉开口的DORV患儿接受外科手术,年龄5个月～12岁,根据不同手术方法将病人分3组比较,其中心室内隧道修补术（intraventricular tunnel repair,IVR）组15例,Rastalli手术组10例,Fontan手术组12例。对限制性VSD,同时扩大VSD直径。结果手术住院死亡10例,死亡率27．0％（10／37例）,其中1999年4月后死亡2例,死亡率10．5％（2／19）。IVR的手术死亡率（46．7％,7／15例）明显高于Rastalli组（20％,2／10例）和Fontan组（8．3％,1／12例）。结论三尖瓣与肺动脉之问距离是否大于主动脉瓣口直径是区别选择IVR手术和Rastalli手术的主要依据。共同房室瓣、三尖瓣附属组织跨越或嵌入到VSD和主动脉之间的区域、两个心室发育不平衡是我们选择改良Fontan的主要适应证。     

个体化手术方案矫治儿童右室双出口的体会

目的 探讨儿童右室双出口(DORV)的外科治疗效果.方法 自1984年8月至2010年6月共手术治疗118例DORV,其中男62例,女56例,年龄10 d～12岁,平均(3.2±2.6)岁,体重3.0～22.5 kg,平均(9.6±3.7)kg.根据SIS-EACTS数据库分类:VSD型86例,TOF型22例,TGA型3例,VSD远离大动脉型7例.手术方法包括采用单纯心室内隧道修补21例,心室内隧道修补+右室流出道扩大补片84例,其中82例自体心包扩大补片,2例行同种带瓣外管道;Switch术4例,双向格林术6例,Fontan术1例,肺动脉环缩术1例,B-T分流术1例.回顾性分析DORV的手术方法和效果.结果 住院死亡9例,病死率7.6%,主要死亡原因为低心排综合症.2004年前手术51例,死亡7例(病死率13.7%),2005年后手术67例,死亡2例(病死率2.9%).存活病例随访2个月～10年,超声心动图检查未见左室流出道梗阻,1例患儿术后2年发现右室流出道残余梗阻,再次手术疏通右室流出道,余患儿效果良好,无晚期死亡病例.结论 根据DORV患儿的解剖特点和年龄,制订个体化的手术方案,有助于提高手术成功率.

Abstract：

Objective To summarize our experience of surgical treatment for double-outlet right ventricle (DORV) in children. Methods From August 1984 to June 2010, 118 patients with DORV underwent surgical repair at this center. Among the 118 patients, 62 were males and 56 were females.Their ages ranged from 10 days to 12 years old (mean age, 3. 2 ± 2. 6). Their weight ranged from 3. 0to 22. 5 kg (mean weight, 9. 6 ± 3. 7 kg). According to the STS-EACTS international nomenclature,86 patients were DORV associated with ventricular septal defect (VSD), 22 were DORV with tetralogy of Fallot (TOF), 3 were DORV with transposition of the greater arteries (TGA), and 7 were DORV with remote VSD. Corrective surgeries included 22 interventricular repairs, 84 interventricular repairs plus right ventricular outflow tract reconstruction (pericardium was used for the reconstruction in 82 cases, and valved extracardiac conduits was used in 2 cases), 4 switch procedures, 6 bidirectional Glenn procedures, 1 Fontan procedure, 1 Pulmonary artery banding procedure, and 1 Blalock-Taussig Shunt procedure. The surgical safety and efficacy were studied by retrospectively analyzing the clinical data of the 118 cases. Results Nine patients died after surgery (7. 6%). The leading cause of death was low cardiac output syndrome. Of the 51 patients underwent corrective surgery before 2004,7 died after surgery (13. 7%). However, only 2 died in the 67 patients who had surgery after 2005(2. 9%). The patients were followed up for 2 months to 10 years. Cardiac ultrasonography didn't show any obstruction of left ventricular outflow tract, but 1 patient developed left ventricular outflow tract obstruction 2 years after surgery and received corrective surgery. No long-term death and other complications were noted. Conclusions Surgical strategy for the children with double-outlet right ventricle should be made based on patients individual anatomic anomalies.     

按STS-EACTS数据库分类的右室双出口手术方法和结果分析

目的 上海儿童医学中心心胸外科数据库采用STS-EACTS数据库颁布的右室双出口（DORV）命名和分类方案后（以下简称为“新方案”），报道该中心外科治疗DORV的新进展。方法 2000年5月-2006年5月302例DORV患儿接受手术治疗，根据“新方案”将研究对象分4组，A组：79例，DORV为VSD型；B组：133例，DORV为四联症型；C组：34例，DORV为TGA型；D组：56例，DORV为VSD远离大动脉型。分析“新方案”与DORV手术方法和手术病死率的相关性。结果 总病死率7．9％（24／302）。A组和B组的病死率分别为1．3％（1／79）和4．5％（6／133），低于C组（23．5％，8／34）和D组（16．1％，9／56），DORV的新分类方案与住院病死率和手术方法选择有显著的相关性。结论 “新方案”有利于数据库对临床资料的搜集和整理，根据“新方案”选择优化的手术方法，可以提高DORV外科疗效。     

主动脉瓣成形术治疗室间隔缺损并中重度主动脉瓣关闭不全

目的探讨室间隔缺损(VSD)并中重度主动脉瓣关闭不全(AI)患儿行主动脉瓣成形术的疗效。方法分析13例14岁以下VSD并中重度AI患儿行主动脉瓣成形术的临床资料。其中VSD为干下型9例,膜周部型4例;中度AI 4例,重度AI 9例。结果全组均无手术死亡。出院查体脉压差均恢复正常。术后彩色多普勒超声心动图检查平均左心室舒张末径与术前比较明显缩小(P<0.05),未发现有室间隔残余漏,2例有轻度AI。随访无手术死亡,患儿心功能均为纽约心脏协会(NYHA)Ⅰ或Ⅱ级。结论儿童主动脉瓣成形术效果良好,对于VSD并中重度AI患儿应首选主动脉瓣成形术。     

2岁以下婴幼儿室间隔缺损并肺高压100例诊治体会

室间隔缺损(VSD)为最常见的先心病之一,大型VSD在婴儿期经常反复心力衰竭,随着年龄增长,肺血管阻力不断增高,最后导致器质性肺高压(PH),失去手术机会,因此在婴儿早期手术关闭VSD是目前先心病治疗的进展。     

婴幼儿室间隔缺损并肺动脉高压的外科治疗

目的 探讨婴幼儿室间隔缺损(VSD)并肺动脉高压(PH)的外科治疗。方法 21例VSD并PH患儿应用浅低温体外循环、温血心肌停搏液连续灌注及间断褥式缝合法进行VSD修补术。围术期注意维护心肺功能，进行综合性降肺动脉压处理。结果 1例(4．8％)患儿死于术后呼吸功能衰竭，1例(4．8％)患儿术后出现右心功能不全。随访18例，恢复良好；1例失访。结论 VSD并PH婴幼儿，若符合手术适应证应及早手术，并积极进行圈术期处理，手术效果较好。     

右室双出口43例临床报告

本文报道经心导管造影确诊右室双出口43例,着重讨论临床表现和诊断。31例二维超声检查21例诊断右室双出口。43例心导管造影按室缺部位分主动脉瓣下20例,14例合并肺动脉狭窄;肺动脉瓣下16例,8例合并肺动脉狭窄;双动脉口下1例;远离大动脉口6例4种类型。本组43例右室双出口仅10例临床诊断与心血管造影相符(23%)。主动脉瓣下室缺伴肺动脉狭窄14例中有10例诊断四联症。主动脉瓣下室缺无肺动脉狭窄很易误诊室间隔缺损合并肺高压。肺动脉瓣下室缺患儿早期出现紫绀和心衰与完全性大动脉错位相似。提示右室双出口的诊断主要依赖二维超声心动图和心导管造影检查。双圆锥可作为右室双出口的诊断依据之一。     

婴幼儿室间隔缺损并肺动脉高压的外科治疗

目的 评估婴幼儿VSD并肺动脉高压(PH)的纠治方法及效果.方法 收集河北医科大学第一医院心外科VSD并PH婴幼儿215例.男109例,女106例;月龄1～36个月;体质量1.5～10.0 kg.膜周型VSD 156例,嵴内型18例,干下型41例.VSD直径0.8 ～2.1 cm.并继发性ASD 16例,卵圆孔未闭8例,PDA15例,并PDA、ASD 14例,并PDA和主动脉瓣下隔膜3例,肺动脉瓣狭窄14例,右心室流出道狭窄33例,二尖瓣关闭不全22例,三尖瓣关闭不全42例,永存左上腔静脉12例.肺动脉压力4.66～11.31 kPa.患儿均在全麻体外循环下行一期根治术.结果 全组手术顺利.术后死亡3例.术后并发症:15例出现短暂低心排出量综合征,其中死亡2例;10例右束支传导阻滞;6例室上性心动过速;5例短暂Ⅲ度房室传导阻滞;5例术后发生PH危象,其中死亡1例;3例气胸;2例肺不张.随访期均行X线胸片、ECG、心脏彩超检查,术后心功能明显改善.结论 婴幼儿VSD并PH病情发展快,易发生低心排出量综合征和低氧血症,早期手术效果良好,治疗的关键在于加强心肌保护,提高手术操作技术,缩短手术时间;术后加强监护,及时处理各种术后并发症.     

Unique variant of Taussig-Bing heart: Double-outlet right ventricle with double ventricular septal defects and double overriding of great arteries

Summary A cyanotic, tachypneic newborn was diagnosed to have double-outlet right ventricle of the Taussig-Bing type. Cardiac failure did not respond to medical treatment or surgical palliation. Postmortem examination revealed two ventricular septal defects (VSDs), one a malalignment VSD in the membranous septum and adjacent tissue and the other in the anterosuperior part of the muscular septum. The D-malposed aortic root emerged mainly from the right ventricle, with aortic-mitral continuity. The larger posterolateral pulmonary root arose almost entirely from the right ventricle, confluent with the muscular VSD, and unrelated to the mitral valve. Its right ventricular aspect was obstructed by hypertrophied infundibulum.This unique malformation of the heart functioned as a double-outlet right ventricle of Taussig-Bing type. In addition, however, the malformation had elements of tetralogy of Fallot because of the malaligned VSD and hypertrophied conal musculature (although pulmonary flow was excessive), and also of complete transposition of the great arteries because of the arrangements of the two VSDs, which favored aortic flow from right ventricle and pulmonary blood flow from the left ventricle. Thus, a single heart presented similarities to three anatomic and functional entities.     

Balloon pulmonary valvuloplasty for double outlet right ventricle with valvular pulmonary stenosis

A male neonate presented with cyanosis. Echocardiograms showed a double outlet right ventricle, an absent outlet septum, a subaortic ventricular septal defect and valvular pulmonary stenosis. Balloon pulmonary valvuloplasty was performed at 19 days of age, resulting in an immediate increase of the systemic arterial oxygen saturation from 67 to 87%. As far as could be found, this is the first report of a patient with a double outlet right ventricle without the outlet septum who was clinically observed and underwent balloon pulmonary valvuloplasty.     

Closure of ventricular septal defect through the pulmonary artery

Summary A transpulmonary arterial approach to the closure of a high ventricular septal defect (VSD) has been used, between 1978 and 1982, in eight patients. The reasons were ease of access and the wish to overcome the problems associated with right ventriculotomy. The patients' ages ranged from three weeks to 15 months, their weight from 2.9 kg to 9 kg. The approach was used both when the VSD was an isolated anomaly and when there were major associated defects. It is in this latter group, four with aortic arch anomalies, two with additional double outlet right ventricle (DORV), that avoidance of ventriculotomy was most helpful. It was especially important in the two patients with DORV and a perimembranous, outlet subpulmonary VSD, where it was possible to close off the left ventricular outflow tract and pulmonary valve using a patch, without opening the right ventricle, which was subsequently to become the systemic ventricle. This technique obviates the need for ventriculotomy in the closure of some perimembranous outlet and doubly committed subarterial VSDs, and is the approach of choice for the closure of a perimembranous, outlet, subpulmonary VSD in DORV.     

Membranous septal aneurysm: an unusual cause for right ventricular outflow tract obstruction in a malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle) associated with visceral heterotaxy

The development of a septal aneurysm in the natural history of membranous ventricular septal defects usually makes the defect hemodynamically less significant. This report describes a case of severe right ventricular outflow obstruction produced by a membranous septal aneurysm in a patient who had an anterior malaligned ventricular septal defect with aortomitral discontinuity (double-outlet right ventricle). This patient did not have pulmonary stenosis other than the dynamic obstruction produced by the septal aneurysm. In this patient, the septal aneurysm produced both favorable and unfavorable hemodynamic effects. A reduction in the size of the ventricular septal defect produced a favorable effect, whereas a right ventricular outflow obstruction led to the unfavorable situation of right ventricular hypertension and hypertrophy. The large septal aneurysm in the presence of an already compromised right ventricular outflow tract related to an anteriorly malaligned septum resulted in severe obstruction.     

Pulmonary atresia with obstructed ventricular septal defect

Summary Two patients with pulmonary atresia had a loud holosystolic murmur and an obstructed ventricular septal defect. Case 1 simulated pulmonary atresia with intact ventricular septum, presenting at 3 months of age with venous congestion, severe tricuspid regurgitation, and suprasystemic right ventricular pressure. Case 2 had a right ventricular systolic pressure that was initially at the systemic level but had increased to a suprasystemic level by 3 years of age. Autopsy showed that the ventricular septal defect was large in each case but was obstructed by tricuspid valve tissue in case 1 and by a hypertrophied septal band in case 2.Supported in part by the University of Illinois Foundation Goodenberger Medical Research Grant (2-44-33-66-3-14)     

Atrioventricular septal defect: Quantitative anatomy of the right ventricle

Summary In 19 human fetal and newborn hearts with atrioventricular septal defect (AVSD), not associated with other anomalies, the two ventricles were studied morphometrically. A total of 17 different parameters were studied: nine in the right ventricle and eight in the left. In the right ventricle we analyzed ventricular wall thickness, length of right ventricular inflow and outflow tracts, and volume of right ventricular inflow and outflow tracts. The data for these parameters were compared with the patterns of normality published previously, and the volumetric data were compared with patterns of normality published previously by us. The ventricular inflow tract was shorter than the outflow tract, the difference being especially significant in the left ventricle. The length of the diaphragmatic wall of the heart in both the right and left ventricle was equal to the sum of the length of the inflow tract and the thickness of the ventricular wall at the apex.     

Intra-utero diagnosis of anomalous right ventricular muscle bundles in association with a ventricular septal defect: A case report

Summary The morphology and natural history of anomalous right ventricular muscle bundles (ARVMB) have been described in a number of postnatal studies. Whether this is a congenital or acquired cardiac lesion remains obscure. A fetal echocardiogram performed in a 32-week gestation mother showed a large ventricular septal defect and anomalous right ventricular muscle bundles, which were easily appreciated at the ostium-infundibular level. The diagnosis was confirmed postnatally and at 6 months of age the child underwent surgical repair. This report documents the presence of ARVMB in a fetus, at a time when hemodynamics cannot explain the development of right ventricular muscle bundles. It suggests that at least, the morphologic substrate for this disease is congenital.     

Failed detection of complex congenital heart disease (including double outlet right ventricle and total anomalous pulmonary venous return) by neonatal pulse oximetry screening

We report on a newborn infant with complex congenital heart disease (CHD) featuring double outlet right ventricle and hypoplastic left ventricle who had postductal oxygen saturation well above 95% and thus eluded pulse oximetry screening for CHD.