心房异构心电图特征的分析

目的探讨心房异构心电图特征及临床意义。方法对比分析126例心房异构先心病（其中右房异构95例，左房异构31例）和心房位置正常的先心病（法洛四联症、大动脉转位、复杂型紫绀型先心病各120例）患者的心电图。结果心房异构先心病异位心律的发生率为66．7％。心房正常组为1％～9％，右房异构中窦性心律占41％，异位心律以左房心律、游走心律多见。分别占24％及28％。左房异构窦性心律占9．7％，异位心律以低位房性心律、交界区心律多见，占61%。心房异构与心房结构正常的复杂型先心病之间及两种不同类型的心房异构之间心电图表现均存在显著差异（p〈0．01）。结论心房异构异位心律发生率明显高于心房位置正常者。右房异构以窦性心律、左房心律、游走心律多见，左房异构以低位房性心律、交界区心律为主。     

心房右异构1例报告

心房异构(artial isomerism)是一种少见的先天性心脏病,根据双心耳的形态结构可分为左异构和右异构.我院曾收治1例心房右异构患儿.现报告如下.     

400例胎儿先天性心脏病的主动脉弓解剖分析

目的:对胎儿先天性心脏病主动脉弓异常进行分析,提高该疾病诊断的精准性。方法:回顾近几年我院经超声心动图畸形筛查发现胎儿先天性心脏畸形引产而进行尸体解剖病例,对其主动脉弓走行进行分析总结,主动脉弓畸形分为6类:主动脉弓分支变异、双主动脉弓、左位主动脉弓、右位主动脉弓、主动脉弓其他异常及肺动脉异常起源。结果:胎儿先天性心脏病尸体解剖400例,主动脉弓异常144例,其中主动脉弓分支变异5例,双主动脉弓3例,左位主动脉弓畸形34例,右位主动脉弓畸形44例,其他畸形55例,肺动脉异常起源5例。左位主动脉弓畸形中动脉导管缺如11例,动脉导管闭锁4例,右锁骨下动脉迷走18例,孤立右锁骨下动脉1例。右位主动脉弓畸形镜面右位主动脉弓33例,左锁骨下动脉迷走7例,孤立左锁骨下动脉1例,孤立无名动脉2例,4支动脉分支对称发出1例。其他畸形包括主动脉弓缩窄45例,主动脉弓离断9例,颈部主动脉弓1例。肺动脉起源异常包括肺动脉吊带3例,Berry综合征1例,肺动脉起源于降主动脉1例。结论:主动脉弓畸形复杂多样,以主动脉弓缩窄最多见,左位主动脉弓畸形以右锁骨下动脉迷走多见,右位主动脉弓畸形主要是镜面右位主动脉弓。     

胎儿先天性心脏病的超声诊断及漏诊分析

目的探讨超声诊断胎儿先天性心脏病的可行性和临床价值。方法采用彩色多普勒超声对6000例孕20~40周的孕妇常规行胎儿四腔心,左、右室流出道,主动脉弓及动脉导管的观察。结果发现心脏畸形24例;经尸检及出生后心超证实。合关心外畸形7例。漏诊9例。结论超声诊断胎儿先天性心脏病是可行的,值得推广。     

200例胎儿先天性心脏病尸体解剖分析

目的:对胎儿先天性心脏病(先心病)进行尸体解剖分析,提高该疾病诊断的精准性。方法:回顾近几年我院经超声心动图畸形筛查,发现胎儿先天性心脏畸形引产而进行尸体解剖病例,分析胎儿心血管畸形不同类型的发病情况及特点,有关心血管畸形按心脏节段分析方法进行分析观察。结果:胎儿先心病尸体解剖200例,男性93例,女性107例,孕12~38周,胎儿先天性心脏病类型:心房/心耳畸形16例,体静脉畸形45例,肺静脉畸形28例,冠状静脉窦畸形4例;房间隔畸形55例,房室瓣畸形及骑跨24例,房室间隔缺损26例,室间隔畸形87例,左心发育不良综合征17例,右心发育不良综合征9例,法洛四联症12例,心室双出口21例,大动脉转位20例,心室单出口21例,主动脉瓣畸形14例;肺动脉瓣畸形28例;主动脉近段畸形73例,肺动脉畸形26例,动脉导管畸形18例;冠状动脉畸形6例;右旋心5例。常见畸形为室间隔缺损56例(28%)、房间隔缺损36(18%)和永存左上腔静脉35(17.5%),单心室31(15.5%),主动脉弓发育不良25(12.5%),右位主动脉弓23(11.5%)。结论:胎儿先心病室间隔缺损、房间隔缺损、永存左上腔静脉、单心室及主动脉弓发育不良发病率较高;胎儿心血管畸形变异较多,血管较细小,解剖时应仔细寻找每一个分支。     

部分肺静脉异位引流畸形的漏诊原因分析

目的：分析部分肺静脉引流（ＰＡＰＶＤ）的漏诊原因,以提高术前的准确率和防止手术失误,方法：回顾性分析自１９９１年１月至１９９９年１０月经手述评上实的ＰＡＰＶＤ１５例临床资料与手术结果,找出临床漏诊的原因。结果：本组１５例ＰＡＰＶＤ手术前仅１例诊断明确,漏诊１３例,术中失误１例。１５例术中均见伴有房间隔缺损（ＡＳＤ）,并肺动脉瓣狭窄３例,肺动脉高压３例,畸莆引流的部分以右房为最多,其次是上腔静脉,畸形引流的肺静脉以右肺静脉最常见,ＰＡＰＶＤ临床漏诊的主要原因：一是此病常伴有ＡＳＤ,临床上ＰＡＰＶＤ较难与单纯ＡＳＤ相鉴别,二是术前心脏起超检查很易漏诊,三是常见的,简单的心脏畸表手术中为有常规心内探查而造成漏诊,结论：ＰＡＰＶＤ较少见,但漏诊率高,对该病加深认识,提高警惕是防止漏诊的关键。ＭＲＩ对肺静脉异位引流有较大     

胎儿二叶式主动脉瓣畸形的超声心动图特征分析

目的：探讨确诊二叶式主动脉瓣（BAV）畸形的胎儿超声心动图特征及预后。方法：对生后确诊BAV的患者产前超声心动图进行回顾和分析,并对其特征进行总结。结果：14例生后确诊患者产前胎儿超声心动图均有异常超声征象提示,包括直接征象（主动脉瓣增厚、回声增强、融合嵴、两个交界及“鱼嘴样开口”、活动及开放受限、一字型关闭线或偏心关闭线）及间接征象（主动脉瓣上流速增快,升主动脉增宽）。其中2例患者产前均进行了3次胎儿超声心动图检查,发现主动脉瓣上流速及升主动脉内径随着孕周的增大而增加。14例患者中,2例（14.3%）出生后因主动脉瓣中重度狭窄出现生长发育受限,其中1例于2个月龄进行主动脉瓣球囊扩张治疗。结论：胎儿BAV主要依靠产前超声心动图筛查,直接超声征象结合间接超声征象可帮助诊断。超声医师疑诊胎儿BAV应建议其父母规律随访患儿主动脉瓣及主动脉情况,有助于确诊及预后咨询。     

右位心单心房合并复杂先天性心脏病心电图1例

患儿女性，11岁。因受凉后心悸、气促加重3天入院。系足月顺产，出生后即有口唇、甲床发绀，且随年龄增大逐渐加重。体检：BP110，70mmHg，P85次／min，营养发育差，杵状指明显。心界扩大，心率85次／min，心律齐，胸骨右缘第3、4肋间可闻及3／6级收缩期吹风样杂音。彩色脉冲多普勒超声心动描记术检查示心脏位于右侧胸腔，多切面扫描未见房间隔声像，主动脉、肺动脉均与右心室相连，膜周室间隔可见连续中断，大小为13mm，肺动脉瓣增厚，回声增强，开放受限。超声心动描记术诊断：镜像右位心，单心房，右心室双出口，完全性心内膜垫缺损，肺动脉瓣狭窄。X线胸片示镜像右位心，     

胎儿先天性心脏病的发病率及及新生儿卵圆孔未闭结局的研究

目的 ：探讨煤炭总医院胎儿先天性心脏病的发病率及及新生儿卵圆孔未闭结局的研究。方法：随机选取煤炭总医院2014年11月至2016年2月孕周在22至34周的孕妇共176例,所入选的研究对象,孕妇的身体状况均良好,已排除了合并高血压、糖尿病等疾病,以及存在某些化学、物理因素接触史等这些可导致胎儿异常的情况;胎儿经过产前筛查已确定均为大致正常者,孕妇年龄范围是20至40岁,平均年龄（28.4±3.4）岁,平均孕周（24.4±3.5）周。 随机选取煤炭总医院2014年11月至2016年2月新生儿病例共70例,与上述胎儿出生后均不重叠。结果：在病例收集期间,发现并确定了多例胎儿及新生儿先天性心脏病,这些典型病例包括了胎儿的完全型心内膜垫缺损、完全型大动脉转位合并房室间隔缺损及肺动脉瓣狭窄、法洛四联征和新生儿的房、室间隔缺损等,并统计出了煤炭总医院胎儿和新生儿先天性心脏病发病率约为0.4%,与目前国内外统计出的公认的发病率相符。出生后24小时内存在卵圆孔未闭的新生儿在之后的1年内是有闭合机会的,其中出生后7日内闭合概率更大。即使1年内未闭合也应继续随访,不宜认定为房间隔缺损。结论：煤炭总医院胎儿及新生儿的先天性心脏病的发病率与国内总体水平相似,也处于较高水平,并且疾病谱有其自身的特点。出生后卵圆孔未闭应加强随访,以减少家庭及社会负担。     

Left atrial isomerism associated with asplenia: prenatal echocardiographic detection of complex congenital cardiac malformations

Complex congenital heart disease with suspected isomerism of the atria was diagnosed in two fetuses of 20 and 29 weeks' gestation using two-dimensional and M-mode scanning techniques. The first pregnancy was terminated at 21 weeks' gestation and stillbirth occurred at 31 weeks' gestation in the second pregnancy. At postmortem examination, a thoracoabdominal discordancy was found; the spleen was absent and the arrangement of the abdominal vessels was as anticipated for asplenia, but the thoracic situs revealed a bilateral right-sided arrangement with left isomerism of the atria. The heart, otherwise, showed complex abnormalities as anticipated for asplenia.     

Clinical implications of atrial isomerism

Right atrial isomerism or left atrial isomerism is frequently diagnosed as situs ambiguous without further discrimination of the specific morbid anatomy. Thirty six cases of right atrial isomerism and seven cases of left atrial isomerism were collected from the records and pathological museum at the National Taiwan University Hospital. There was a necropsy report for 18 cases. In all patients one or more of the following conditions was met: (a) isomeric bronchial anatomy, (b) echocardiographic and angiocardiographic evidence of isomerism, and (c) surgical or necropsy evidence of abnormal atrial anatomy. An anomalous pulmonary venous connection was present in 55% of patients with right atrial isomerism; in left atrial isomerism one case (14%) had a partial anomalous pulmonary venous connection. Forty per cent of cases of anomalous pulmonary venous connection with right atrial isomerism had obstruction. Six (86%) of seven cases with left atrial isomerism had an ambiguous biventricular atrioventricular connection. In contrast, univentricular atrioventricular connection (26 of 36, 72%) was significantly more common in right atrial isomerism. A common atrioventricular valve was the most frequent mode of connection in both forms. Two discrete atrioventricular valves were significantly more common in left atrial isomerism. Atrioventricular valve regurgitation was detected in 14 cases. Double outlet right ventricle was the most common type of ventriculoarterial connection. The most commonly cited causes of death after either palliative or definitive operation were undetected anomalous pulmonary venous connection, pulmonary venous stricture, and uncorrected atrioventricular valve or aortic regurgitation complicated by abnormal coagulation. Although the prognosis is poor, successful operation depends on knowledge of the precise anatomical arrangement associated with atrial isomerism.     

Supraventricular tachycardia in patients with right atrial isomerism

Objectives. To clarify the prevalence and mechanism of supraventricular tachycardia in patients with right atrial isomerism.Background. Paired SA and dual atrioventricular (AV) nodes have been described in patients with right atrial isomerism. However, the clinical significance remains unclear.Methods. From 1987 to 1996, a total of 101 patients (61 male, 40 female) and four fetuses were identified with right atrial isomerism. The diagnosis of supraventricular tachycardia exclude the tachycardia with prolonged QRS duration or AV dissociation, and primary atrial tachycardia.Results. The median follow-up duration was 38 months (range 0.2–270 months). Supraventricular tachycardia was documented in 25 patients (24.8%) and one fetus (25%) (onset age ranged from prenatal to 14 years old; median 4 years old). Actuarial Kaplan-Meier analysis revealed that the probability of being free from tachycardia was 67% and 50% at 6 and 10 years of age, respectively. These tachycardias could be converted by vagal maneuvers in one, verapamil in seven, propranolol in four, digoxin in two, procainamide in one, and rapid pacing in five. Spontaneous conversion was noted in six (including the fetus). Seven cases had received electrophysiological studies. Reciprocating AV tachycardia could be induced in five and echo beats in one. The tachycardia in three patients was documented as incorporating a posterior AV node (antegrade) and an anterior or a lateral AV node (retrograde). Two of them received radiofrequency ablation. Successful ablation in both was obtained by delivering energy during tachycardia, aimed at the earliest retrograde atrial activity and accompanied by junctional ectopic rhythm. The patient with echo beats developed tachycardia soon after operation.Conclusions. Supraventricular tachycardia is common in patients with right atrial isomerism and can occur during the prenatal stage. Drugs to slow conduction through the AV node may help to terminate the tachycardia. Radiofrequency ablation is a safe and effective treatment alternative to eliminate tachycardia.     

Clinical implications of atrial isomerism.

Right atrial isomerism or left atrial isomerism is frequently diagnosed as situs ambiguous without further discrimination of the specific morbid anatomy. Thirty six cases of right atrial isomerism and seven cases of left atrial isomerism were collected from the records and pathological museum at the National Taiwan University Hospital. There was a necropsy report for 18 cases. In all patients one or more of the following conditions was met: (a) isomeric bronchial anatomy, (b) echocardiographic and angiocardiographic evidence of isomerism, and (c) surgical or necropsy evidence of abnormal atrial anatomy. An anomalous pulmonary venous connection was present in 55% of patients with right atrial isomerism; in left atrial isomerism one case (14%) had a partial anomalous pulmonary venous connection. Forty per cent of cases of anomalous pulmonary venous connection with right atrial isomerism had obstruction. Six (86%) of seven cases with left atrial isomerism had an ambiguous biventricular atrioventricular connection. In contrast, univentricular atrioventricular connection (26 of 36, 72%) was significantly more common in right atrial isomerism. A common atrioventricular valve was the most frequent mode of connection in both forms. Two discrete atrioventricular valves were significantly more common in left atrial isomerism. Atrioventricular valve regurgitation was detected in 14 cases. Double outlet right ventricle was the most common type of ventriculoarterial connection. The most commonly cited causes of death after either palliative or definitive operation were undetected anomalous pulmonary venous connection, pulmonary venous stricture, and uncorrected atrioventricular valve or aortic regurgitation complicated by abnormal coagulation. Although the prognosis is poor, successful operation depends on knowledge of the precise anatomical arrangement associated with atrial isomerism.     

Diagnosis and significance of atrial isomerism

Fifty-one patients at the Brompton Hospital were identified as having either right or left atrial isomerism by reviewing the records and catheterization, echocardiographic and, in 17 cases, autopsy data. Every study patient had 1 or more of the following conditions: isomeric bronchial anatomy; angiographic or necropsy evidence of atrial anatomy; echocardiographic diagnosis of isomerism; and anomalies of systemic or pulmonary venous connection. Patients with anomalous venous connections all had additional signs of isomerism. Neither right nor left type was predominant in either sex. The heart was in the left chest slightly more often than the right. Abdominal visceral heterotaxy was the most frequent radiographic finding. An ambiguous and biventricular connection was the commonest type of atrioventricular (AV) connection in left isomerism. A common AV valve was the most frequent mode of connection in both forms. Univentricular AV connection, double-outlet right ventricle, pulmonary atresia and discordant ventriculoarterial connection were commoner in right isomerism. Left isomerism is associated with much longer survival, its constellation of associated malformations frequently being less severe and more amenable to corrective surgery. A precise diagnosis must be made during life in order to select the most appropriate treatment.