婴幼儿巨大室间隔缺损合并重度肺动脉高压的围手术期护理

报告了86例婴幼儿巨大室间隔缺损合并重度肺动脉高压的围手术期护理。术前对不同认知能力和心理需要的患儿进行针对性的心理护理、喂养指导、吸O2、药物降压。术后主要做好呼吸系统的护理,保持呼吸道通畅,预防因体温过高、腹胀、疼痛、哭闹引起的肺动脉高压危象,提高了重度肺动脉高压的治愈率。86例患儿除1例因低心排综合征死亡外,均治愈出院。     

Criss-cross heart with double-outlet right ventricle,subpulmonary ventricular septal defect,and bicuspid pulmonary valve

Criss-cross heart (CCH) is an extremely rare complex congenital heart malformation. It accounts for less than 0.1% of congenital heart diseases. Here, we describe a unique case of CCH with double-outlet right ventricle, huge subpulmonary ventricular septal defect, bicuspid pulmonary valve, and right-hand aortic arch. The anatomic features were observed with echocardiography, and the diagnosis was confirmed at surgery. Many variations of CCH have been described. The present case expands the spectrum of this entity and may provide new insight into this complex anatomy.     

室间隔缺损并中重度肺动脉高压患者围手术期处理

目的:探讨室间隔缺损并中重度肺动脉高压患者的手术时机及围手术期处理。方法:28例室间隔缺损并中重度肺动脉高压患者,术前给予吸氧、前列腺素E1等药物治疗后均行室间隔缺损修补术,部分重度肺动脉高压患者应用单向活瓣补片修补室间隔缺损。结果:术后死亡2例,其中1例为低心排血量综合征并肺动脉高压危象,1例为低心排血量综合征并肺部感染。余26例安全度过围手术期并痊愈出院。结论:选择手术时机,术中完善手术修复和恰当的围术期处理是室间隔缺损并中重度肺动脉高压患者治疗的关键。     

室间隔缺损并中重度肺动脉高压患者围手术期处理

目的:探讨室间隔缺损并中重度肺动脉高压患者的手术时机及围手术期处理.方法:28例室间隔缺损并中重度肺动脉高压患者,术前给予吸氧、前列腺素E1等药物治疗后均行室间隔缺损修补术,部分重度肺动脉高压患者应用单向活瓣补片修补室间隔缺损.结果:术后死亡2例,其中1例为低心排血量综合征并肺动脉高压危象,1例为低心排血量综合征并肺部感染.余26例安全度过围手术期并痊愈出院.结论:选择手术时机,术中完善手术修复和恰当的围术期处理是室间隔缺损并中重度肺动脉高压患者治疗的关键.     

Membranous subpulmonic stenosis associated with ventricular septal defect and aortic insufficiency.

We present a case report of a patient with conal septal hypoplasia (supracristal) ventricular septal defect (VSD) complicated by aortic insufficiency (AI) and subpulmonic stenosis from a fibrous membrane. The development of AI with VSD is a well-recognized problem. However, the association of VSD, AI, and significant right ventricular outflow tract obstruction (RVOTO) is less common. Mechanisms of RVOTO include prolapse of an aortic valve cusp across the VSD, as well as infundibular hypertrophy or muscle bundles. Technical echocardiographic issues can make the diagnosis of VSD, AI, and RVOTO challenging. The presence of a discrete fibrous subpulmonary membrane is uncommon in this setting.     

Relief of severe pulmonary hypertension after closure of a large ventricular septal defect using low dose inhaled nitric oxide

A 16-month-old girl developed severe pulmonary hyptertension after closure of a large ventricular septal defect. All conventional therapeutic measures failed; an attempt to add nitric oxide at a continous low dose to the inspired gas allowed resolution of pulmonary hypertension and low cardiac output. This report documents that continuous inhalation of low dose nitric oxide is capable of selective resolution of pulmonary hypertension following cardiac surgery for a large septal defect in a child. This suggests that a transient dysfunction in the release of nitric oxide by the pulmonary endothelial cell is responsible for the vasoconstriction.     

Two-dimensional echocardiographic diagnosis of double outlet left ventricle with subaortic ventricular septal defect, pulmonary stenosis, and a hypoplastic left ventricle.

Double outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both the pulmonary artery and the aorta arise exclusively or predominantly from the morphologic left ventricle. We describe a case of DOLV with situs solitus, d-loop ventricles, d-malposition of the great arteries (S, D, D), severe pulmonary stenosis, and a subaortic ventricular septal defect in which the left ventricle was also severely hypoplastic. The anatomic features were shown with 2-dimensional echocardiography, and the diagnosis was later confirmed at cardiac catheterization. Many morphologic variations of this malformation have been described but to the our knowledge DOLV with a hypoplastic left ventricle has not been reported before.     

Idiopathic calcific constrictive pericarditis causing pulmonary stenosis associated with a ventricular septal defect mimicking tetralogy of Fallot

We describe an unusual case of pulmonary stenosis caused by calcific constrictive pericarditis associated with a congenital ventricular septal defect in a 16-year-old boy who had a 2-week history of progressive dyspnea, cyanosis, fatigue, and bilateral leg edema. Echocardiographic findings led to an initial diagnosis of tetralogy of Fallot; however, findings on chest radiography and CT were suggestive of calcific constrictive pericarditis with pulmonary stenosis, which was then confirmed on cardiac catheterization. Total pericardiectomy and repair of the ventricular septal defect resulted in a satisfactory outcome. Follow-up examinations at 6 and 20 months showed that the patient was asymptomatic and considered to have class I New York Heart Association functional status. To our knowledge, this is the first reported case of calcific constrictive pericarditis with pulmonary stenosis associated with a ventricular septal defect.     

Prenatal diagnosis of pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare complex congenital heart defect. Major artery-pulmonary collateral arteries (MAPCAs) are characteristic of PA-VSD. Prenatal diagnosis can be achieved in most cases of PA-VSD with recent advances in echocardiography. However, it is extremely rare that all MAPCAs can be observed on the echocardiograph. Here, we report a case of prenatally diagnosed type C PA-VSD in which all the MAPCAs could be seen on the echocardiograph, with the diagnosis supported by autopsy evidence.     

1例经皮同期封堵治疗室间隔缺损并房间隔缺损病人的护理

经导管封堵治疗膜周部室间隔缺损（VSD）是近年先天性心脏病介入治疗的热点之一。随着VSD封堵器械的改进，其安全性不断提高，在临床上得到日益广泛的应用。而应用国产双盘状房间隔缺损（ASD）封堵器封堵器治疗ASD的安全性和良好疗效已得到公认。我科于2006年11月14日对1例膜周部VSD合并ASD的病人进行经导管同期治疗，取得了良好的效果，现将护理体会报道如下。     

MSCT诊断肺动脉闭锁伴室间隔缺损

目的 探讨MSCT诊断肺动脉闭锁伴室间隔缺损（PA/VSD）的价值。方法 回顾性分析81例PA/VSD患者的临床及影像资料。将患者术前经胸超声心动图（TTE）、MSCT检查结果与手术结果对比。结果 PA/VSD A1型23例,A2型17例,B型34例,C型7例。MSCT诊断PA/VSD分型的准确率为93.82%（76/81）,高于TTE[59.26%（48/81）;χ²=26.95,P<0.01];MSCT诊断粗大主动脉及肺侧支循环动脉（MAPCAs）来源准确率为100%（93/93）,高于TTE[51.84%（51/93）;χ²=54.25,P<0.01]。MSCT检出心内畸形50处（50/53,94.34%）,TTE检出53处（53/53,100%）,二者差异无统计学意义（χ²=1.37,P=0.24）;MSCT检出心脏-大血管连接异常66处（66/66,100%）,TTE检出65处（65/66,98.48%）,二者差异无统计学意义（P>0.05）;MSCT检出心外大血管异常106处（106/106,100%）,高于TTE[82.08%（87/106）;χ²=20.87,P<0.01]。MSCT测量McGoon比值、肺动脉指数、全部新的肺动脉指数与手术所见比较差异均无统计学意义（P均>0.05）。结论 MSCT可准确诊断PA/VSD分型及肺血管发育情况,为临床诊疗提供指导。     

肺动脉闭锁合并室间隔缺损的影像学诊断及评价

目的:评价肺动脉闭锁合并室间隔缺损(PAVSD)的影像学表现及各种检查方法的应用价值。材料与方法:经心血管造影(CAG)和/或手术证实的PAVSD 28例,均行心脏X线平片和超声心动图(UCG)检查,12例行MRI检查。结果:X线平片示肺动脉段直角状凹陷和肺野内局限性肺纹理异常为诊断较特异征象;UCG诊断24例,未能显示中央肺动脉;MRI可显示PAVSD心内结构及中央肺动脉及其发育状况,并可观察到部分粗大侧支血管;经未闭动脉导管造影可显示左、右肺动脉连接处隔膜状狭窄。结论:MRI与X线平片和UCG联合应用,可提高本病无创诊断水平,术前仍需要行CAG检查。     

Rastelli手术治疗合并室间隔缺损和肺动脉狭窄的完全性大动脉转位2例

目的报告应用解剖矫正术治疗合并心室间隔缺损和肺动脉狭窄的完全性大动脉转位。方法采用Rastelli术对15岁和17岁2例完全性大动脉转位合并心室间隔缺损和肺动脉狭窄患者,进行解剖矫正。采用涤纶片做心内隧道,牛颈静脉带瓣管道做心外管道。结果术后无死亡。随访2~5个月,疗效满意。结论Rastelli术解剖矫正完全性大动脉转位合并心室间隔缺损和肺动脉狭窄,可获得满意的效果。     

彩色多普勒对室间隔缺损术后残余分流的预后评估

室间隔缺损术后残余分流（室缺残余分流）是先天性心脏病室间隔缺损（或含室缺的其它先天性心脏病）手术治疗的常见并发症。彩色多普勒超声检查是诊断室缺残余分流最为敏感的无创检查方法。本文对我院2003年8月～2007年3月进行手术治疗后并发室缺残余分流的86例患者进行跟踪随访分析，旨在利用彩色多普勒超声检查对室缺残余分流的预后进行评估，为临床判断预后提供帮助。     

胎儿期肺动脉闭锁合并室间隔缺损的超声心动图特征

肺动脉闭锁合并室间隔缺损(pulmonary atresia with ventricularseptal defect,PA-VSD)是一种少见的复杂性先天性心脏病,其发病率在出生的活婴中约为十万分之4[1]。产前超声检查对PA-VSD的筛查及优生优育具有重要意义。本组回顾分析经尸体解剖证实的3例PA-VS胎儿的超声心动图特征,旨在探讨产前超声在筛查胎儿期PA-VSD中的价值。     

彩色多普勒超声在扩展先天性室间隔缺损合并重度肺动脉高压患者手术适应证中的研究

目的 彩色多普勒超声在扩展先天性室间隔缺损(VSD)合并重度肺动脉高压(PH)患者手术适应证中的应用价值.方法 选取先天性VSD并重度PH患者30例,肘静脉注射腺苷75 μg·kg-1·min-1,应用彩色多普勒超声心动图检测主动脉平均压、肺动脉平均压、肺动脉收缩压、肺动脉舒张压、肺循环阻力、体循环阻力、肺循环阻力/体循环阻力比值、肺动脉/主动脉收缩压比值、肺循环/体循环血流量比值,注射5分钟后肺血管阻力下降>30%和肺动脉平均压下降>10%为急性血管扩张试验阳性.结果 17例急性血管扩张试验阳性者,通过临床药物降压治疗有效后成功手术;13例药物试验阴性者继续降压治疗无效.结论 通过腺苷急性血管扩张试验,可以判断肺动脉高压的可逆性,彩色多普勒超声心动图在扩展先天性室间隔缺损合并重度肺动脉高压患者手术适应征中起到初步筛选的作用.     

Quantitative evaluation of the pattern of shunt flow in the right ventricle and pulmonary artery of dogs with experimental ventricular septal defect.

Cineangiographic studies in patients with ventricular septal defect (VSD) have occasionally demonstrated that part of the blood across the defect is ejected immediately into the pulmonary artery (PA) passing through the outflow tract of the right ventricle (RV), but without being trapped in it. We attempted to make a quantitative evaluation of the flow of a partial shunt pathway (a direct VSD-PA pathway) that drains that part of the blood from the defect. Our method depended on a thermal dilution technique to obtain the ejection fraction of the RV and to observe a simultaneous pair of dilution curves at the roots of the aorta and PA after introduction of tracer into the left atrium. An analytical process was specially designed by incorporating a stable one-pass deconvolution technique. The method was applied to eight anesthetized dogs with acutely produced experimental VSD on the entrance of the outflow tract of the RV. The flow through the direct VSD-PA pathway was, in most cases, greater than 50 and up to 85% (mean of the eight, 57 +/- 5% SE) of the total left-to-right shunt flow. This would imply that less than 50%, and down to as little as 15%, of the total amount of shunt flow contributed to extra work of the RV in these cases. In addition, the impact on the pulmonary vasculature due to such a large amount of pulsatile flow through the direct VSD-PA pathway may accelerate the development of hypertrophy of the pulmonary vessel wall.     

A case of unicuspid aortic valve associated with a single coronary artery and ventricular septal defect

The patient was a 26-year-old man who had undergone patch closure of a ventricular septal defect at 2 years of age. After this surgery, his condition was satisfactory until he visited our hospital for treatment of paroxysmal supraventricular tachycardia in November 2000. Echocardiography revealed moderate to severe aortic valve regurgitation and dilatation of the left ventricle and ascending aorta. These echocardiographic abnormalities were attributed to a bicuspid aortic valve. Coronary angiography suggested the presence of a single coronary artery originating from the left Valsalva sinus. We performed Bentall’s operation in January 2003. The intraoperative findings revealed that the aortic valve consisted of an extensively calcified single cusp, and there was a single coronary artery originating from the left Valsalva sinus. Because adult patients with a unicuspid aortic valve are rare, and no cases of unicuspid aortic valve associated with a single coronary artery have been reported, we herein report this case with a review of the literature.This paper was presented at the 19th meeting of the Chubu Chapter of the Japan Society of Ultrasonics in Medicine.