右心发育不良综合征40例临床分析

回顾分析40例右心发育不良综合征的类型及其检查和治疗。方法对40例患儿的心电图(EKG)、 X线胸片、彩色超声心动图(UCG)、心导管、心血管造影检查结果以及治疗进行比较分析。结果以三尖瓣闭锁类的 Ib型最常见(35％)。有26例行心导管及心血管造影，与UCG符合者25例(96.2％)。在该25例中有1例UCG和 心导管检查均诊断为法洛三联症，但手术后确诊为右心发育不良综合征。本组40例中有10例进行了外科手术治疗。 结论右心发育不良综合征畸形复杂，手术前诊断需仔细地综合分析，才能为外科选择手术方式提供依据。     

超声心动图诊断法洛四联症的临床价值评估

目的评价超声心动图诊断法洛四联症（TOF）的准确性、可靠性和效益,探讨法洛四联症无创伤性术前诊断方法及手术指征。方法以手术后诊断为金标准,评价234例TOF患儿的二维超声心动图（2-DE）诊断及心导管诊断结果。结果对于TOF的诊断,2-DE诊断灵敏度97.9%,特异度99.8%,符合率99.6%;心导管诊断灵敏度99.5%,特异度100%,符合率99.9%;两者的差异无统计学意义。在TOF合并心血管畸形的诊断中,2-DE诊断CA横跨ROVT、侧支血管、PDA的灵敏度、特异度、符合率与手术后诊断存在明显差异。结论2-DE可替代心导管造影检查用于TOF的诊断。如果术前2-DE检查发现冠状动脉显示不清或怀疑走行异常、存在侧支血管、PDA与侧支血管难以鉴别及肺动脉分支发育不良时,需进一步行心导管造影检查。     

下腔静脉异位引流4例报告

下腔静脉异位引流发生率为心血管畸形的0.6％。1982年至1993年本院1200多例心导管检查及心血管造影中发现4例下腔静脉异位引流,现报告如下。 临床资料 4例中男2例,女2例;年龄6月～11岁。经胸片、心电图、超声心动图、心导管及心血管造影发现有下腔静脉异位引流。经半奇静脉,奇     

选择性心血管造影检查小儿法洛四联症伴随畸形：附384例报告

为探讨法洛四联症患儿的伴随畸形。提高手术成功率，我们于1993年2月-1999年7月对384例法洛四联症患儿行心导管及选择性心血管造影检查。结果表明166例（43.2%)法洛四联症伴有周围肺动脉狭窄。冠状动脉畸形，左室发育不良、完全性房室隔缺损等多种伴随畸形。为此作者认为，法洛四联症伴随畸形并不少见。而且部分伴随畸形对手术治疗及预后估测至关重要，因此对法洛四联症行心血管造影检查十分必要。     

儿童血管相关性咯血介入治疗的临床评价

目的探讨儿童血管相关性咯血的临床特点及围术期程序性诊治方法。方法回顾性分析16例血管相关性咯血患儿的临床资料及影像学特点,并对其治疗方案及治疗效果进行随访和评价。结果 16例患儿中男4例、女12例,1岁3例、5~13岁13例。经血管造影明确为主肺侧支循环13例(81.25%)、肺动静脉瘘1例(6.25%)、支气管动脉肺血管瘘1例(6.25%),另1例未明确诊断血管类型。11例患儿行介入封堵治疗,1例行胸腔镜下异常血管结扎术,1例行部分肺叶切除术,3例放弃治疗。11例介入封堵治疗患儿,封堵术后即时造影,2例可见少许分流;3例封堵术1年内咯血复发,均为主动脉侧支循环。2例手术治疗患儿无复发。结论肺血管异常为儿童咯血的主要病因之一,以主肺侧支循环为主要类型,介入封堵仍为儿童血管相关性咯血的一线治疗方案。     

先天性桡骨发育不良的初步治疗体会

目的 介绍治疗先天性桡骨发育不良的初步体会.方法 2006年7月至2008年12月我院收治先天性桡骨发育不良的患儿7例,共8侧(其中一例为双侧累及).男6例,女1例,年龄最小5个月,最大5.5岁,平均2.5岁.除2例为Ⅲ型,其余均为Ⅳ型.此外1例伴发颈椎畸形,1例伴有马蹄足畸形,1例伴有髋关节发育不良.首先采用手法牵拉减缓软组织的挛缩,到1岁时进行手术,手术方法主要是采用尺骨腕关节中置术,即暴露腕关节,于近侧腕骨中心切一小槽,修剪尺骨远端,然后将其插入腕骨小槽中并用克氏针固定,同时对尺侧松弛的组织进行紧缩.在进行该项手术前先要对患儿前臂桡侧挛缩程度进行评估,若桡侧挛缩程度严重,则用ILIZAROV支架牵拉软组织后再行手术.手术后石膏同定4周,术后8周取出克氏针,术后需佩戴矫形支具.随访时间3～30个月,平均12个月.结果 所有患儿在手术后畸形立即得到明显纠正,外观得到明显改善,手术后患肢功能较前也有增进,肘关节活动增多.结论 尺骨腕关节中置术结合桡侧软组织牵拉,术后再辅以矫形支具的佩戴能达到比较满意的治疗效果.     

婴儿先天性心脏病133例心导管检查术分析

目的　探讨婴儿先天性心血管畸形的心导管检查特点及并发症的防治经验。方法　分析婴儿期先天性心脏病患儿 133例 ,年龄为 5d至 12个月 ,体重 2 4～ 9 6kg。导管进路经股静脉穿刺 12 7例 ,大隐静脉剖开 6例 ,同时做动脉穿刺进路 7例。行右心导管检查 82例次 ,右室造影 96例次 ,左心系统造影 2 2例次。结果　诊断紫绀型复杂心血管畸形 74例 (其中法洛四联症 4 9例 ) ,无紫绀型心血管畸形 5 9例 ,4 7例伴严重肺动脉高压。心导管检查术非致死性有意义并发症 5 .3% ,无死亡。结论　心导管术是确诊婴儿复杂心血管畸形最准确的方法 ;心导管检查中要严密观察婴儿病情变化 ,由有经验医师细心操作 ,先天性心脏病婴儿心导管检查术比较安全。     

先天性肺静脉狭窄的诊断和治疗

目的探讨先天性肺静脉狭窄的诊断和治疗.方法应用心导管和心血管造影、超声心动图及磁共振确定诊断.结果5例中管状狭窄4例,隔膜性狭窄1例;5例狭窄部位均在肺静脉与左心房连接处.三支肺静脉狭窄1例,二支肺静脉狭窄2例,一支肺静脉狭窄2例.合并其他先天性心脏病4例.结论早期发生原因难以解释的严重肺动脉高压的先天性心脏病,应考虑合并肺静脉狭窄.心导管和心血管造影及超声检查可明确诊断.外科手术疗效尚不满意,需继续研究探讨.     

小儿完全性肺静脉畸形引流的外科治疗

目的　评判完全性肺静脉畸形引流早期手术矫治的疗效及心上型完全性肺静脉畸形引流各种吻合方法的优缺点。方法　 1976～ 1999年共收治完全性肺静脉畸形引流 14 0例 (男 81,女5 9) ,平均年龄 ( 7.13± 6.88)岁。心上型 87例 ( 62 .14 % ) ,心内型 4 4例 ( 3 1.4 3 % ) ,心下型 2例( 1.4 3 % ) ,混合型 7例 ( 5 % )。 2例经右心导管检查证实为重度肺动脉高压 ,未能实施矫治手术 ,余患儿均在全麻、低温、体外循环下行矫治术。心上型的吻合方法包括 :心外法 ( 2 1例 ) ,经上腔静脉与升主动脉间吻合法 ( 3例 ) ,经房间隔缺损吻合法 ( 18例 ) ,心外吻合加经房缺吻合 ( 2例 ) ,经左右心房联合切口吻合法 ( 4 3例 )。结果　术后早期 ( 3 0d)死亡 14例 ( 10 % ) ,其中 3例合并其他复杂的心内畸形 ,均死亡 ,故单纯TAPVC的手术死亡 11例 ,死亡率为 8.0 3 % ( 11/ 13 7)。术后主要并发症为心律失常 60例 ( 4 2 .86% )。结论　完全性肺静脉畸形引流宜尽早手术 ,心上型矫治术吻合口足够大是保证手术成功的关键 ,左右房联合切口入路可确保吻合口足够大 ,但心律失常发生率高 ,经上腔静脉与升主动脉间吻合法可减少心房损伤 ,降低心律失常发生率。     

肺动脉闭锁合并室间隔缺损的临床病理分析

目的 研究肺动脉闭锁合并室间隔缺损的解剖类型和血流动力学改变,并探讨其适宜的手术方式.方法 回顾性分析233例肺动脉闭锁合并室间隔缺损的造影结果,分析肺血来源、肺动脉发育情况和合并畸形及其与手术预后的影响.结果 233例中中央肺动脉存在,肺血单纯由未闭的动脉导管供应者112例(48.1%),其中1例为双侧动脉导管(0.5%);中央肺动脉和大的主一肺动脉侧支血管(MAPCA)均存在者104例(44.6%);无中央肺动脉,仅有MAPCA供应肺血者17例(7.3%).肺动脉闭锁部位以右室流出道和瓣膜闭锁最多见(48.1%).侧支血管的来源包括直接的主动脉一肺侧支动脉,间接的主动脉一肺侧支动脉和支气管动脉.合并畸形包括心脾综合征、房室连接不一致、心室大动脉连接不一致、多发室间隔缺损、右位主动脉弓、房间隔缺损、左上腔静脉残存、内脏异位症、上下心室、肺静脉异位引流和冠状动脉起源异常等.结论 肺动脉闭锁合并室间隔缺损患者肺血来源多样化,肺动脉发育程度不一,并可合并多种心内、心外畸形,影响手术方法的选掸和手术结果.     

Mitral and Aortic Atresia Associated with Hypoplastic Right Lung, Crossover Segment of Right Lower Lobe, and Anomalous Scimitar-like Right Pulmonary Venous Connection with Inferior Vena Cava: Clinical, Angiocardiographic, and Autopsy Findings in a Rare Case

A newborn female infant was found to have a unique and previously unreported group of anomalies: (1) mitral and aortic atresia with a highly obstructive atrial septum; (2) hypoplasia of the right lung with a crossover segment involving the right lower lobe; (3) normally connected pulmonary veins, two from the left lung and one from the right; and (4) a large anomalous branch of the right pulmonary vein of scimitar configuration that anastomosed with the normally connected right pulmonary vein and with the inferior vena cava (IVC). The scimitar vein appeared obstructed at its junction with the right pulmonary vein and at its junction with the inferior vena cava within the hepatic parenchyma. To our knowledge, this is the first report of a scimitar-like vein coexisting with mitral and aortic atresia and connecting both with the right pulmonary vein and with the inferior vena cava. The highly obstructed left atrium was partially decompressed by retrograde blood flow via the normally connected right pulmonary vein to the anomalous scimitar venous pathway and thence to the inferior vena cava via a pulmonary-to-IVC collateral vein. Received March 18, 1997; accepted August 6, 1997.     

Meandering right pulmonary vein: a case of scimitar variant

We report a healthy, asymptomatic 15-year-old girl with a meandering right pulmonary vein draining to the left atrium. A meandering pulmonary vein may or may not be associated with the scimitar syndrome. The differential diagnosis with the scimitar syndrome and other forms of scimitar variants is discussed. Received: 13 October 1998 Accepted: 15 February 1999     

Scimitar variant

Summary In the classic scimitar syndrome, a pulmonary vein draining all or part of the right lung enters the inferior vena cava. A variant is described with the same roentgenographic appearance, but with drainage of the anomalous pulmonary vein into both the inferior vena cava and the left atrium; the atrial septum was intact. This case, together with six others reported elsewhere, reminds us that the scimitar sign has both false positives and false negatives. Therefore, the diagnosis of scimitar syndrome cannot be made with certainty from a plain x-ray film.     

Isolated Left-sided Scimitar Vein Connecting All Left Pulmonary Veins to the Right Inferior Vena Cava

When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as “scimitar syndrome.” We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.     

Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome

Scimitar vein draining to the left atrium is the rarest of the pulmonary venolobar anomalies with less than a dozen reported cases. A case is reported of a patient whose plain film radiographic findings showed a typical scimitar vein but whose echocardiography showed normal pulmonary venous drainage. Cardiac catheterization confirmed drainage of the scimitar vein to the left atrium and systemic arterial supply which was embolized. The case described is used to review the history of scimitar syndrome with reflections on the significant contributions of Halasz and colleagues, who in 1956 helped define the anatomy, and Neill and colleagues, who in 1960 described the familial occurrence and clinical spectrum of the condition.     

儿童孤立性冠状动脉瘘的治疗及中期随访分析

目的对儿童孤立性冠状动脉瘘的治疗过程及中期随访结果进行总结分析。方法回顾性分析广州市妇女儿童医疗中心2009年7月至2017年7月诊断为孤立性冠状动脉瘘的17例患儿临床资料,其中男11例,女6例,年龄40日龄至12岁(中位值23个月),体重3.8~29 kg(中位体重11.3 kg)。2例存在气促表现,2例存在生长发育落后表现,2例存在反复呼吸道感染表现,1例主诉心悸,余10例无明显临床症状。其中3例心脏检查未发现明显杂音,14例发现心脏杂音。瘘口发生于右冠状动脉8例,左冠状动脉9例。瘘入右心房5例,右心室10例,左心室2例;17例中12例合并巨大冠状动脉瘤。结果1例行经皮冠状动脉瘘封堵术,1例在非体外循环下行瘘管结扎术,其余15例在体外循环下行冠状动脉瘘矫治术;平均住院时间(11.1±4.1)d。其中2例术后3 d内出现射血分数下降(最低者降至38%),术后1个月复查时射血分数均升至50%以上。7例术后出现一过性T波改变,出院时均已恢复正常;2例术后存在1~2 mm残余瘘的患儿在近期复查中残余瘘消失。围术期无一例死亡。随访10个月至9年,所有患儿无自觉症状和阳性体征,心电图均无心肌缺血表现,复查超声心动图均提示心脏收缩功能正常,冠状动脉直径均较术前缩小。结论大多数儿童孤立性冠状动脉瘘无明显症状,但可合并巨大冠状动脉瘤,应尽早手术,中远期预后良好;但因存在冠状动脉扩张,仍需长期随访。     

经左心房内吻合治疗心下型完全性肺静脉异位引流

目的 评估经左心房内径路吻合治疗心下型完全性肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的疗效.方法 2012年3月至2015年4月,采用左心房内路径吻合法治疗心下型TAPVC 14例.男9例,女5例.年龄8～42 d,平均(28.9±6.73)d;体重2.8～5.4kg,平均(3.9±1.43)kg.所有患儿均在全身麻醉、中低温体外循环下手术.充分游离左、右肺静脉、共汇静脉、垂直静脉后,在心脏自然状态下观察共汇静脉-垂直静脉与心脏位置关系,对共汇静脉-垂直静脉走形位于左房或房间隔后位的14例患儿,采用左心房内径路将肺静脉共汇-垂直静脉与左心房后壁吻合.结果 所有患儿均顺利完成手术,体外循环时间89～141 min,平均(118.7±22.14)min;主动脉阻断时间46～67 min,平均(52.1±6.54)min.术后呼吸机辅助时间3～7 d,平均(4.19±1.04)d.术后早期(46 h)1例患儿因消化道出血死亡,其余13例患儿中,低心排出量综合征8例、室上性心动过速2例、气胸2例、肺部感染7例、经积极治疗后均痊愈出院.13例患儿出院后1个月、3个月、1年随访率均为100％.结论 对共汇静脉-垂直静脉走行位于左房或房间隔后的心下型TAPVC,采用左心房内路径吻合法治疗能够取得满意的临床疗效.     

Eponym

Scimitar syndrome is a rare congenital anomaly, characterized by partial or complete anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. The syndrome is commonly associated with hypoplasia of the right lung, pulmonary sequestration, persisting left superior vena cava, and dextroposition of the heart. The pathogenesis of the syndrome is unclear, but it seems to originate from a basic developmental disorder of the entire lung bud early in embryogenesis. Two main forms of scimitar syndrome have been described. Signs and symptoms can start during infancy (infantile form) or beyond (childhood/adult form). The infantile form generally presents within the first 2 months of life with tachypnea, recurrent pneumonia, failure to thrive, and signs of heart failure. The diagnosis of scimitar syndrome is usually made based on the characteristic chest X-ray films and can be confirmed by angiography; however, it is now done mostly by transthoracic or transesophageal echocardiography, noninvasive computed tomography, or magnetic resonance angiography. Fetal echocardiography using three-dimensional power Doppler imaging permits prenatal diagnosis. Most frequently, patients are asymptomatic in the absence of associated abnormalities and can be followed conservatively. For patients with congestive heart failure, repeated pneumonia, or pulmonary-to-systemic blood flow ratios greater than 1.5 and pulmonary hypertension, it is important to reroute the anomalous right pulmonary veins and repair the associated cardiac defects in order to avoid progression to right ventricular failure. The triad of respiratory distress, right lung hypoplasia, and dextroposition of the heart should alert the clinician to think of scimitar syndrome.     

Late cardiac damage of anthracycline therapy for acute lymphoblastic leukemia in childhood

Long-termleukemia survivors (46) underwent cardiac evaluation, including physical examination, ECG, exercise testing, and echocardiography. They were 2-17 years old at diagnosis and 5-23 years old aftertreatment. Thirty-four survivors received anthracyclines (AC) (mean 203 mg/m2), 12 of them had also alkylating agents (AA) and 12 had no AC. Exercise tolerance was bellow predicted valuesin 21 (48%) survivors and 21 survivors had ECG abnormalities, which were more frequent in those treated with AC. Concomitant AC with AA was correlated with prolonged isovolumic relaxation time (IVRT) and influenced significantly the volume of left atrium (p = .02). Sixteen (52%) survivors had IVRT 90 ms. There were no significant differences in other parameters of diastolic orsystolic function. Despite the lack of clinical symptoms in the survivors treated with lower doses of AC, subtile abnormalities in myocardial function were found, mainly manifest as abnormal diastolic function. Prolonged IVRT may be a sensitive indicator for early detection of AC cardiotoxicity.     

LATE CARDIAC DAMAGE OF ANTHRACYCLINE THERAPY FOR ACUTE LYMPHOBLASTIC LEUKEMIA IN CHILDHOOD

Long-termleukemia survivors (46) underwent cardiac evaluation, including physical examination, ECG, exercise testing, and echocardiography. They were 2-17 years old at diagnosis and 5-23 years old aftertreatment. Thirty-four survivors received anthracyclines (AC) (mean 203 mg/m2), 12 of them had also alkylating agents (AA) and 12 had no AC. Exercise tolerance was bellow predicted valuesin 21 (48%) survivors and 21 survivors had ECG abnormalities, which were more frequent in those treated with AC. Concomitant AC with AA was correlated with prolonged isovolumic relaxation time (IVRT) and influenced significantly the volume of left atrium (p = .02). Sixteen (52%) survivors had IVRT 90 ms. There were no significant differences in other parameters of diastolic orsystolic function. Despite the lack of clinical symptoms in the survivors treated with lower doses of AC, subtile abnormalities in myocardial function were found, mainly manifest as abnormal diastolic function. Prolonged IVRT may be a sensitive indicator for early detection of AC cardiotoxicity.