先天性肺动脉吊带畸形二例

肺动脉吊带畸形(pulmonary artery sling)是一种少见的先天性心血管畸形。患儿出生后多有严重的呼吸系统症状,如不及时诊断和治疗,病死率高。因本病少见,临床缺乏认识,极易漏诊。我院自1991～2001年10年间共收治小儿先天性心脏病8650例,发现本病3例,经手术和尸检证实2例,报告如下。     

儿童先天性冠状动脉瘘介入治疗2例报告

先天性冠状动脉瘘(coronary artery fistulas,CAF)属少见先天性心血管畸形,在先天性心血管畸形的发病构成中的占0.2%～0.4%,是先天性心血管畸形最常见的类型,具有潜在危险性.     

先天性心脏病胎儿脑血管阻力变化研究

目的检测患有先天性心脏病的胎儿脑血管阻力变化。方法使用脉冲多普勒超声技术对患有先天性心脏病的胎儿进行大脑中动脉（middle cerebral artery，MCA）及脐动脉（umbilical artery，UA）血流动力学指数测量。包括搏动指数（pulsatility index，PI）、阻力指数（resistance index，RI）。计算脑／脐血管阻力指数比（cerebral—to-placental resistance ratio，CPR；CPR=MCA-RI／UARI）。研究包括25例胎儿先天性心脏病病例，病例组分为组1：包括胎儿左心发育不良、主动脉狭窄、完全性大血管转位，共12例；组2：包括右心血流阻塞的病例、胎儿肺动脉狭窄、肺动脉闭锁、法洛四联症及Ebstein畸形，共13例。对照组包括100）例正常胎儿与病例组按胎龄配对。结果病例组与对照组MCA-PI、UA-PI差异无统计学意义。病例组1MCA-PI低于病例组2（P=0．026）及对照组（P=0．035）。病例组2UA-PI较病例组1升高（P=0．047）。病例组2与对照组MCA-PI，UA-PI差异无统计学意义。CPR在各病例组间两两比较，差异均无显著性意义。结论运用脉冲多普勒超声技术可检测到部分患有先天性心脏病的胎儿存在脑血管阻力降低的变化，不同类型的心脏畸形可能引起胎儿脑血管不同程度的代偿反应。     

左冠状动脉起源于肺动脉二例

左冠状动脉起源于肺动脉（anomalous origin of left coronary artery from pulmonary artery,ALCAPA）是一种罕见的先天性心脏病,早期表现为心肌缺血、心力衰竭等非特异性症状,死亡率超过90％,平均死亡年龄35岁,如未予以手术治疗,极少有患者能活过50岁。因其早期症状缺乏特异性,以往对其没有很好的认识,临床上易误诊为扩张性心肌病、心内膜弹力纤维增生症、先天性的二尖瓣病变等。早期手术治疗能明显改善预后,故早期诊断有重要的临床价值。现将我院诊治的2例ALCAPA资料总结报告如下。[第一段]     

肩胛骨矫形术治疗先天性高肩胛症

目的　探讨先天性高肩胛症的手术治疗。方法　采用肩胛骨部分切除矫形治疗先天性高肩胛症患儿 2 6例 (2 7肩 ) ,随访平均 3 .9年 ,最长时间达 7年。结果　术前患肢外展上举平均 1 1 3°,术后平均改善为 1 4 5° ,多数患儿的学习生活功能不受影响。结论　肩胛骨部分切除矫形治疗先天性高肩胛症可以取得令人满意的功能     

小儿肺动脉发育异常影像学诊断价值探讨

单侧肺动脉缺如(unilateral absence of pulmonary artery,UAPA)或发育不良是小儿时期罕见的先天性肺血管畸形,因临床缺乏特异性表现,常导致误诊。诊断的主要依据为影像学检查,为提高对本病的认识,现将我院5例肺动脉发育异常患儿的临床及影像学资料并结合有关文献予以分析,兹报道如下。     

先天性巨结肠相关疾病和综合征

先天性巨结肠是小儿低位肠梗阻常见的致病因素,发病率高且有性别差异。目前认为其属于多基因遗传病,所涉及的遗传因素复杂。先天性巨结肠病因未明,但它往往出现在一些综合征中,与某些症状关联,如Shah-Waardenburg、Goldberg-Shprintzen、MEN2A、Fryns、Haddad等综合征。随着分子生物学技术的发展,目前已从综合征入手发现了一些与先天性巨结肠密切相关的致病基因,现对近几年国际上报道具有先天性巨结肠表现的综合征作一综述。     

自体Ross术治疗先天性主动脉瓣病变一例

先天性主动脉瓣病变约占先天性心脏病的5％。以下是先天性主动脉瓣病变一例报告。     

先天性心脏病并发脑脓肿的影像学诊断

目的探讨影像学对儿童先天性心脏病合并脑脓肿的诊断价值。方法回顾性分析13例先天性心脏病合并脑脓肿患儿的临床资料和影像学表现，其中男性10例，女性3例；最小年龄19个月，最大年龄14岁，平均8岁8个月。10例行CT检查，3例行MRI检查。结果13例全部为青紫型先心，其中法洛四联症（tetralogy of Fallot，TOF）9例，大血管转位（transposition of great arteries，D-TGA）2例，伴有肺动脉狭窄的单心室（single ventricle with pulmonary stenosis，SV／PS）1例，室隔完整的肺动脉闭锁伴动脉导管未闭（pulmonary atersia with intact venticle septum and patem duct artery，PA／IVS／PDA）1例。发生部位有额叶、额顶、枕顶、颞顶、枕叶、丘脑和一侧额颞顶叶。单房10例，多房3例，增强后呈环行强化。结论先心病尤其是青紫型先心，极易并发脑脓肿。影像学方法对本病能作出诊断，有利于及时治疗，减少死亡率。     

三房心1例

三房心是一种罕见的先天性心脏病,约占先天性心脏畸形的0.1%,我院近期收治1例,疗效较好,现报告如下。     

Anomalous origin of the left coronary artery from the pulmonary artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCA-PA) is a rare form of congenital heart disease. In this report, three cases with this anomaly are described; two patients presented in infancy with heart failure from myocardial ischemia and infarction, while the third was asymptomatic and ALCA-PA was diagnosed during evaluation of a residual murmur after surgery for associated cardiac defects (ventricular septal defect and patent arterial duct). All three cases underwent aorto-pulmonary tunnel repair (Tukeuchi procedure), and to our knowledge two of them are the first infantile cases reported in Turkey.     

婴儿左冠状动脉起源于肺动脉的诊断研究

目的　研究婴儿左冠状动脉起源于肺动脉（ＡＬＣＡＰＡ） 的临床特点及诊断方法。方法　总结分析１９９３～１９９８ 年收治的４ 例ＡＬＣＡＰＡ 的临床特点。结果　４ 例ＡＬＣＡＰＡ均初诊为婴儿心内膜弹力纤维增生症,发病年龄小于３ 个月,心电图Ｉ,ａＶＬ,Ｖ４～６ 导联出现异常Ｑ 波,彩色多普勒超声心动图检查示左冠状动脉缺如。最后经导管检查及心血管造影确诊。结论　本文提出的诊断方法有助于婴儿ＡＬＣＡＰＡ的诊断,且易为儿科医师掌握。     

Single coronary artery originating from the right pulmonary artery

Summary An infant with a single coronary artery originating from the right pulmonary artery branch is the subject of this report. He survived to the age of one month possibly because of the association of two ventricular septal defects. While the pulmonary vascular resistance remained high, perfusion of the heart muscle was accomplished due to the pulmonary hypertension. The electrocardiogram did not show typical signs of infarction, but poor left ventricular contractility was observed by echocardiogram. The diagnosis was made postmortem. This specific coronary malformation has not been previously described and needs to be included in the classification of congenital coronary arteries anomalies.     

Isolated origin of the left internal carotid artery from the pulmonary artery

The authors describe what is, to their knowledge, the first reported case of the anomalous origin of an internal carotid artery from the pulmonary artery. An otherwise asymptomatic 6-year-old girl, who presented with headaches and hypertension, underwent a comprehensive workup that revealed extensive meningeal and cerebral artery anastomoses to the left internal carotid artery--itself arising from the origin of the left pulmonary artery. This unique anatomical anomaly, caused by a disturbed pattern of aortic arch regression, resulted in a right-to-left vascular shunt into the pulmonary artery and a disturbance of intracranial artery flow patterns, complicating the management options.