Spontaneous closure of ventricular septal defects in the first year of life.

BACKGROUND AND PURPOSE: The recent increase in the prevalence of ventricular septal defect (VSD) has been ascribed to the improved detection of small defects with echocardiography and the wider use of screening. The aim of this study was to determine the prevalence and timing of spontaneous closure of specific types of VSD in neonates using echocardiographic screening and follow-up. METHODS: Two-dimensional color Doppler echocardiography was performed in 3,472 clinically normal full-term neonates born at Cathay General Hospital to detect isolated VSD. The relative prevalence of muscular versus perimembranous defects and their outcome in the first year of life were evaluated. RESULTS: VSD was found in 74 neonates (34 male, 40 female), resulting in a prevalence of 21.3/1,000 live births. There were 48 muscular, 25 perimembranous, and one subpulmonic defects. Of the 74 patients, 11 were lost to follow-up. Within the observation period of 12 months, spontaneous closure occurred in 40 patients in the muscular group and in six patients in the perimembranous group. The overall rate of spontaneous closure was 73% by the end of the first year. Only five patients with perimembranous defects received digoxin therapy. CONCLUSIONS: The prevalence of VSD in this series of neonates was 21.3/1,000 live births. The most common location of VSD in the neonatal period was in the region of the muscular septum. Muscular defects were more likely to close spontaneously than perimembranous defects. Most muscular defects underwent spontaneous closure during the 12-month follow-up period.     

Atrial and ventricular septal defects - epidemiology and spontaneous closure.

OBJECTIVE: To present data on the epidemiology and spontaneous closure for septal defects (secundum atrial septal defect (ASD) and/or ventricular septal defect (VSD)). METHODS: Data from the EUROCAT Registry of Congenital Malformations for Funen County, Denmark were analyzed. All infants born from 1986 to 1998, diagnosed with a secundum ASD and/or a VSD as the only cardiac malformation, were included. RESULTS: There were 78 infants with an ASD, 195 with a VSD, and 19 had both an ASD and a VSD. The overall prevalence of septal defects was 4.1 per 1000 livebirths. Among the VSDs 45% were perimembranous and 55% were muscular defects. Associated non-cardiac malformations, syndromes, or karyotype anomalies were present in 54 cases (19% of total) but with a significantly lower proportion among cases with muscular VSDs (7%, p < 0.05). Eighty-one percent of the cases had an isolated cardiac malformation. Five of 61 cases (8%) with isolated ASD were closed surgically before five years of age and 43 (70%) closed spontaneously within the first five years of life. Of the 65 cases with isolated perimembranous VSDs, 13 were closed surgically (20%) and 13 closed spontaneously (20%). Of the 99 cases with isolated muscular VSDs no defects were closed by surgery and 64 (65%) closed spontaneously. The rate of spontaneous closure for perimembranous and muscular VSDs were significantly different (p < 0.0001). CONCLUSIONS: Secundum ASD has a high rate of spontaneous closure. Perimembranous and muscular VSDs seem to be two different diseases with different epidemiology and natural history.     

Atrial and ventricular septal defects – epidemiology and spontaneous closure

Objective.?To present data on the epidemiology and spontaneous closure for septal defects (secundum atrial septal defect (ASD) and/or ventricular septal defect (VSD)).

Methods.?Data from the EUROCAT Registry of Congenital Malformations for Funen County, Denmark were analyzed. All infants born from 1986 to 1998, diagnosed with a secundum ASD and/or a VSD as the only cardiac malformation, were included.

Results.?There were 78 infants with an ASD, 195 with a VSD, and 19 had both an ASD and a VSD. The overall prevalence of septal defects was 4.1 per 1000 livebirths. Among the VSDs 45% were perimembranous and 55% were muscular defects. Associated non-cardiac malformations, syndromes, or karyotype anomalies were present in 54 cases (19% of total) but with a significantly lower proportion among cases with muscular VSDs (7%, p < 0.05). Eighty-one percent of the cases had an isolated cardiac malformation. Five of 61 cases (8%) with isolated ASD were closed surgically before five years of age and 43 (70%) closed spontaneously within the first five years of life. Of the 65 cases with isolated perimembranous VSDs, 13 were closed surgically (20%) and 13 closed spontaneously (20%). Of the 99 cases with isolated muscular VSDs no defects were closed by surgery and 64 (65%) closed spontaneously. The rate of spontaneous closure for perimembranous and muscular VSDs were significantly different (p < 0.0001).

Conclusions.?Secundum ASD has a high rate of spontaneous closure. Perimembranous and muscular VSDs seem to be two different diseases with different epidemiology and natural history.     

第二代动脉导管未闭封堵器封堵 特殊类型室间隔缺损2例报告

目的　总结分析应用第二代动脉导管未闭封堵器（ADO-Ⅱ）对特殊类型室间隔缺损（VSD）封堵的技巧。方法　研究对象为2011年8月至9月在上海交通大学医学院附属上海儿童医学中心行介入治疗的患儿2例,因应用常规VSD封堵器封堵困难,遂选取ADO-Ⅱ进行治疗。常规建立动静脉轨迹后,采用主动脉内释放,然后行左室、升主动脉造影和心脏超声检查,如封堵器位置好,无残余分流、瓣膜反流则释放。结果　例1左室造影显示为膜周VSD,左室面7.6 mm,较大假性室隔瘤形成,右室分流口弥散,最大约2.3 mm,缺损上缘距主动脉为6.1 mm。导丝建轨后,7 F长鞘无法通过分流口,最后选用5F长鞘,“6 mm×4 mm”的ADO-Ⅱ封堵成功,术后心脏超声三尖瓣轻微分流。例2心室造影为肌部VSD,左室面6.3 mm,右室分流口为2 mm,上缘距主动脉16 mm。因VSD走行异常且分流口小,最终选冠脉导丝建轨成功,应用4 F长鞘,“4 mm×4 mm”ADO-Ⅱ封堵成功。2例患儿术后1d复查心脏超声和心电图,无异常,观察5 d后出院随访,并口服阿司匹林［3～5 mg/（kg·d）］。结论　对于一些形态较特殊的VSD,常规VSD封堵器无法成功封堵时,可选择ADO-Ⅱ进行封堵,手术操作简单、安全、可靠而并发症少。     

Right Fetal Cardiac Axis: Clinical Significance and Associated Findings

Objective: To ascertain the clinical significance of right fetal cardiac axis.Methods: Fetal cardiac axis was assessed prospectively in ultrasound examinations of 16,562 fetuses over a 6-year period.Results: Twenty-two fetuses had a right cardiac axis. When classified by ventricular and atrial configuration, six fetuses had mirror-image hearts with situs inversus, 12 had rotation of the heart axis alone, and four had inversion of the ventricles. Fourteen of the 22 had underlying structural cardiac defects, most of which were atrioventricular septal defects, double outlet right ventricles, or common atria. The chromosomes and/or phenotypes of all 22 were normal. All four fetuses with polysplenia and asplenia died. Major extracardiac defects were few (two) but lethal.Conclusion: Right cardiac axis in the fetus is associated with a high incidence of structural cardiac defects. In the absence of severe extracardiac defects, polysplenia, or asplenia, neonatal outcome was good.     

经导管室间隔缺损封堵术后传导阻滞的处理及随访

目的随访经导管室间隔缺损(VSD)封堵术后并发的传导阻滞，探讨其发生机制及预后。 方法2002 06—2005 07山东省立医院小儿心脏科经导管介入治疗89例VSD患儿，对临床资料、心电图、心脏超声、心血管造影资料及随访结果进行分析。 结果89例患儿应用Amplatzer膜部VSD封堵器67例，国产对称型VSD封堵器20例，Amplatzer导管封堵器1例，pfm弹簧圈1例。术后出现间歇性完全性房室传导阻滞2例次，其中1例应用临时起搏器治疗，分别于术后第10天、第20天恢复窦性心律；完全性左束支阻滞3例次，完全性右束支阻滞3例次，左前半分支阻滞2例次，不完全性右束支阻滞5例次，加速性交界性心律合并完全性左束支阻滞1例次。 结论传导阻滞是VSD介入治疗术后的常见并发症，经治疗预后良好，及时发现、及时处理是诊疗的关键，对心内传导系统远期的影响仍需进一步评价。     

胎儿部分型及过渡型房室间隔缺损的产前超声诊断特点

目的 探讨产前超声诊断胎儿部分型及过渡型房室间隔缺损的价值。方法 回顾性分析11例经尸体解剖和产后超声心动图证实的部分型及过渡型房室间隔缺损胎儿的产前超声表现,总结其声像图特征。 结果胎儿超声心动图显示,7例部分型和4例过渡型房室间隔缺损在四腔心切面均显示原发孔型房间隔缺损,二尖瓣和三尖瓣在室间隔的附着点位于同一水平,关闭时与室间隔构成“T”字形图像。7例部分型房室间隔缺损中1例伴有左室-右房通道;4例过渡型房室间隔缺损均伴有室间隔上部缺损。11例胎儿中伴有二尖瓣反流4例,二、三尖瓣反流1例,双侧桡骨缺如1例。 结论产前超声观测心脏“T”字形声像图特征对诊断胎儿部分型和过渡型房室间隔缺损具有重要临床价值,但应注意与冠状静脉窦形成的假象鉴别。     

115例胎儿心脏畸形的产前诊断分析

目的:探讨胎儿心脏畸形的特点及其与染色体异常的关系。方法:对我院产前超声诊断的115例胎儿心脏畸形进行侵入性产前诊断,分析心内外畸形及染色体异常的类型和比例。结果:115例胎儿中心脏单发畸形74例,多发畸形41例;未合并心外畸形54例,合并心外畸形61例。染色体异常31例,包括21三体15例和18三体11例,右室双出口、室缺、完全型心内膜垫缺损及三尖瓣反流合并染色体异常比例分别占63.6%、44.4%、35.7%及33.3%。心脏单发畸形中24.3%合并染色体异常,心脏多发畸形中31.7%合并染色体异常,两者比较差异无统计学意义(P0.05);未合并心外畸形中14.8%合并染色体异常,合并心外畸形中37.7%合并染色体异常,两者比较差异有统计学意义(P0.05)。结论:胎儿心脏畸形常合并心外畸形及染色体异常,染色体异常以非整倍体为主,其中右室双出口、室缺、完全型心内膜垫缺损及三尖瓣反流合并染色体异常较常见,合并心外畸形时,染色体异常的比例增加。     

胎儿心脏锥干畸形产前诊断评价

目的评价胎儿心脏锥干畸形产前超声心动图诊断准确性。方法运用胎儿超声心动图进行先天性心脏病产前诊断，并与心脏病理诊断或新生儿心脏超声诊断进行比较分析。结果2063例胎儿进行心脏超声检查，产前发现胎儿心脏锥干畸形28例，诊断时平均孕周26．6周（16～40周），产前诊断包括大血管转位5例，法洛四联症5例，右室双出口6例，永存动脉干9例，室间隔缺损合并主动脉骑跨可能1例，2例检查发现大血管位置异常但胎儿超声心动图未予明确诊断。24例经胎儿心脏病理检查或新生儿心脏超声检查确诊，4例失访（包括1例TOF／TA？，2例“大血管发育异常”，1例TGA）。比较产前诊断与产后诊断，分析大血管位置关系诊断准确性为75．0％（18／24）。结论胎儿超声心动图可正确诊断胎儿心脏锥干畸形，需运用多种超声切面及彩色脉冲多普勒确定大血管位置关系。     

Results of in utero atrial septoplasty in fetuses with hypoplastic left heart syndrome

OBJECTIVES: Neonates with hypoplastic left heart syndrome and intact or highly restrictive atrial septum have a high rate of mortality. We sought to assess the effect of prenatal intervention intended to create atrial septal defects in fetuses with this diagnosis. METHODS: We reviewed the medical records and imaging of all fetuses undergoing intervention for atrial septal defect creation in the setting of hypoplastic left heart syndrome and intact atrial septum. The procedures were performed with a percutaneous cardiac puncture under the guidance of ultrasonography. For the 21 interventions, patient and procedural characteristics were analyzed to identify predictors of neonatal outcome. RESULTS: Of 21 procedures attempted between 24 and 34 weeks' gestation, 19 were technically successful. Fetal demise occurred in two cases. The size of the created defect varied and measured at least 3 mm in six fetuses. Among 19 neonates, a larger atrial septal defect was associated with higher oxygen saturation and less need for intervention prior to surgical single-ventricle palliation. CONCLUSIONS: Technically successful atrial septal defect creation in fetuses with hypoplastic left heart syndrome and intact atrial septum results in atrial septal defects of varying size; defects of at least 3 mm in diameter appear to confer postnatal benefit.     

Fetal cardiovascular cross-sectional morphology of tetralogy of Fallot in rats

In order to clarify cross-sectional morphology of tetralogy of Fallot in the fetus, 202 rat fetuses with tetralogy of Fallot induced by maternal administration of bis-diamine were studied using cesarean section, the whole body freezing method and by photographing cross sections of the fetal heart on a freezing microtome. Some characteristic features such as large subaortic ventricular septal defects, aortic overriding over the ventricular septum, aortomitral fibrous continuity, and narrow infundibulum and pulmonary valve were found. Moreover, three subtypes were noticed. In classic tetralogy of Fallot with stenotic infundibulum, the size of the pulmonary arteries was smaller in proportion to the infundibular stenosis. In the second type with absent infundibular septum and severe valvular pulmonary stenosis, the pulmonary arteries were most hypoplastic. In the third type with absent pulmonary valve, the pulmonary arteries were dilated forming aneurysms. In the last type only, ventricles were dilated and grossly hypertrophic.     

三个心脏超声切面在常见先天性心脏病产前诊断中的作用

目的 探讨三个胎儿超声心动图标准切面：四腔心切面、五腔心切面、三血管平面在发现和诊断胎儿常见先天性心脏病中的作用及对于先天性心脏病产前筛查的意义。方法 回顾2003年5月至2004年7月胎儿心脏畸形各病例的心脏超声图像，记录每一病例三个超声切面（四腔心切面、五腔心切面、三血管平面）的彩色多普勒超声图像表现，分析并总结各切面异常表现的特点。结果 （1）研究共包括胎儿心脏畸形病例26例，疾病类型包括房室间隔缺损、单纯性室间隔缺损、单心室、单心房、左心发育不良综合征、法洛四联症、右心室双出口、纠正性／完全性大动脉转位、动脉单干、主动脉瓣狭窄、肥厚性心肌病（梗阻型）、心脏肿瘤。（2）各心脏畸形病例在这三个心脏超声切面的扫查中至少有1个切面显示异常。每一类心脏畸形在这三个切面图像上均有特征性表现。（3）所有病例中二维四腔心切面（常规产科筛查切面）显示异常的比例为73％，四腔心切面未显示异常的病例包括：完全性大血管转位3例、法洛四联症1例、右心室双出口1例、动脉单干1例、主动脉骑跨伴室间隔缺损1例。（4）各标准切面获得率分别为：96．2％、88．5％、84．6％。结论 （1）四腔心切面、五腔心切面、三血管平面这三个胎儿彩色多普勒心脏超声切面探查在各类胎儿常见的先天性心脏病超声产前检查与诊断中起重要的作用。（2）与运用单个二维四腔心切面探查比较，三个切面的探查方法可提高常见先天性心脏病，尤其是胎儿心脏锥干畸形的检出率。（3）三个切面探查操作较为简便，有望成为一项胎儿先天性心脏病的筛查方法。     

Amplatzer septal occluder-induced transient complete atrioventricular block.

Percutaneous transcatheter atrial septal defect (ASD) closure is a widely used technique that has replaced open-heart surgical closure in many centers. The most common implant is the Amplatzer septal occluder which seems to be a highly effective and safe device. However, there are reports of complications associated with its implantation. We report a 9-year-old boy who presented with complete atrioventricular block after undergoing percutaneous closure of a large secundum ASD with an Amplatzer septal occluder. We treated the patient with oral prednisolone. The patients atrioventricular conduction improved to second-degree Mobitz type 1 block on post-procedure day 24 and first-degree block on day 35. We conclude that patients with Amplatzer septal occluder-induced complete atrioventricular block generally have a good outcome, although it may take several weeks for improvement.     

Prenatal diagnosis of atrioventricular discordance with ventriculoarterial concordance by fetal echocardiography: A case report

ObjectiveTo report an extremely rare case of atrioventricular discordance and ventriculoarterial concordance associated with a ventricular septal defect which was diagnosed prenatally.Case reportBy fetal echocardioraphy at 20 weeks' gestation, we diagnosed a rare case of atrioventricular discordance and ventriculoarterial concordance associated with a ventricular septal defect. This is the first case reported from Vietnam prenatally. We present our management of this pregnancy and the baby's neonatal course. This rare anomaly remains a challenge for the baby's early neonatal course before initial neonatal discharge.ConclusionA combined multidisciplinary and individualized approach for the optimal management of this complicated pregnancy and further neonatal surgical treatment plans for the baby are recommended.     

胎儿先天性心脏病和风疹病毒感染的关系

目的 探讨和分析孕妇风疹病毒感染与胎儿先天性心脏病(简称先心病)的关系,为提高先天性风疹综合征产前诊断率寻求方法.方法 对超声心动图诊断并要求引产的38例先心病胎儿行脐带穿刺,采用酶联免疫吸附试验测定脐血风疹病毒特异性抗体IgM. 结果 38例先心病胎儿中18例脐血风疹病毒IgM(+),占47.4%,其余20例IgM(-).18例风疹病毒IgM(+)胎儿按心脏异常结构出现频率排序:室间隔缺损10例、肺动脉发育异常9例、房室瓣异常6例、大动脉转位5例、主动脉骑跨4例.IgM(+)和IgM(-)组中属于圆锥动脉分隔异常或圆锥动脉干间隔旋转不足或方向相反的病例数分别为11例(61.1%)和5例(25.0%)(P<0.05). 结论 风疹病毒与胎儿先心病的发生有一定关联,可能影响胎儿心室分隔和圆锥动脉干发育.B超提示胎儿室间隔缺损、肺动脉发育异常、心肌瓣膜异常、大动脉转位时,应考虑到风疹病毒感染的可能.     

彩色多普勒超声心动图检测胎儿先天性心脏病

目的　探讨彩色多普勒超声心动图检测胎儿先天性心脏病 (先心病 )的可行性。　方法　 2 96例胎儿分为两组 :14 2例有高危因素的胎儿 ,15 4例正常妊娠胎儿。应用多普勒测定胎儿心脏各切面 ,并在生后作超声心动随访。　结果　在 14 2例有高危因素的胎儿中 ,发现胎儿先心病 5例 ,其中右心发育不良综合征 1例、法洛四联症 2例、单纯性室间隔缺损 (室缺 ) 2例。漏诊 2例 ,为单纯性室缺1例及房间隔缺损 1例。 15 4例正常妊娠胎儿中未发现先心病。生后超声心动随访或引产尸检结果与产前诊断相符。　结论　彩色多普勒超声心动图在诊断胎儿先心病方面是比较准确和安全的 ,对高危孕妇需加强胎儿先心病的检测     

The human fetal heart in the second trimester of gestation: a gross morphometric study of normal fetuses

One hundred fifty-three midtrimester fetuses obtained from normal pregnancies terminated for nonmedical reasons were studied to establish normal standard measurements. Mean, standard deviation, range, and percentiles of body weight, foot length, crown-rump length, biparietal diameter, cardiac weight, thickness of ventricles, and ventricular septum were derived by computer analysis. Statistical analysis showed no sex difference. Equations for predicting body weight, cardiac weight, and septal thickness were derived with the use of fetal age and biparietal diameter as variables. The mean ratio of septal to left ventricular wall thickness was 1.14 +/- 0.34; a ratio of 1.3 or greater was found in 25% and a ratio of 1.5 or greater in 10% of this cohort. The results are at variance with the reported high incidence of septal hypertrophy seen in normal developing fetal hearts. We suggest that septal hypertrophy, ratio of 1.5 or greater, may reflect disturbances in fetal or placental circulation and warrants careful monitoring of the pregnancy.     

Four-dimensional ultrasonography of the fetal heart with spatiotemporal image correlation

OBJECTIVE: This study was undertaken to describe a new technique for the examination of the fetal heart using four-dimensional ultrasonography with spatiotemporal image correlation (STIC). STUDY DESIGN: Volume data sets of the fetal heart were acquired with a new cardiac gating technique (STIC), which uses automated transverse and longitudinal sweeps of the anterior chest wall. These volumes were obtained from 69 fetuses: 35 normal, 16 with congenital anomalies not affecting the cardiovascular system, and 18 with cardiac abnormalities. Dynamic multiplanar slicing and surface rendering of cardiac structures were performed. To illustrate the STIC technique, two representative volumes from a normal fetus were compared with volumes obtained from fetuses with the following congenital heart anomalies: atrioventricular septal defect, tricuspid stenosis, tricuspid atresia, and interrupted inferior vena cava with abnormal venous drainage. RESULTS: Volume datasets obtained with a transverse sweep were utilized to demonstrate the cardiac chambers, moderator band, interatrial and interventricular septae, atrioventricular valves, pulmonary veins, and outflow tracts. With the use of a reference dot to navigate the four-chamber view, intracardiac structures could be simultaneously studied in three orthogonal planes. The same volume dataset was used for surface rendering of the atrioventricular valves. The aortic and ductal arches were best visualized when the original plane of acquisition was sagittal. Volumes could be interactively manipulated to simultaneously visualize both outflow tracts, in addition to the aortic and ductal arches. Novel views of specific structures were generated. For example, the location and extent of a ventricular septal defect was imaged in a sagittal view of the interventricular septum. Furthermore, surface-rendered images of the atrioventricular valves were employed to distinguish between normal and pathologic conditions. Representative video clips were posted on the Journal's Web site to demonstrate the diagnostic capabilities of this new technique. CONCLUSION: Dynamic multiplanar slicing and surface rendering of the fetal heart are feasible with STIC technology. One good quality volume dataset, obtained from a transverse sweep, can be used to examine the four-chamber view and the outflow tracts. This novel method may assist in the evaluation of fetal cardiac anatomy.     

Two-dimensional and M mode echocardiography in the human fetus

One hundred unselected pregnant women underwent M mode and two-dimensional fetal echocardiographic studies between 14 and 43 weeks' gestation. The following cross-sectional views were obtained: high parasternal short axis, parasternal long axis, four chamber view, and an extended long axis to assess the aorta. These views successfully identified great vessel orientation and size (90%), atrial and ventricular size and function (90%), presence of atrioventricular valves (92%) and semilunar valves (75%), and presence and continuity of ventricular and atrial septa (91%) and the aortic arch (68%). Factors that limited visualization were persistent unfavorable lie, maternal obesity, and oligohydramnios. Disturbances in cardiac rhythm were the prevalent abnormal findings (16%). In addition, several anatomic defects were detected in utero and confirmed at autopsy. These included a biventricular cardiomyopathy, atrial septal defect, pericardial effusion, and hypoplastic heart. The potential applications of our ability to detail human fetal cardiac anatomy and dimensions are discussed.     

Trisomy 18 in monozygotic twins with discordant phenotypes.

The incidence of trisomy 18 in monozygotic twins is approximately 1 per million. We report a pair of liveborn monozygotic twins with trisomy 18. Both twins had esophageal atresia with tracheoesophageal fistula (type C) and intrauterine growth retardation. Twin A had cleft lip, choanal atresia and perimembranous ventricular septal defect. Twin B had hypoplastic left heart syndrome. The twins died without aggressive intervention at the age of 2 months and 52 hours, respectively. These 2 babies had significantly discordant phenotypes, which suggests an epigenetic or environmental effect. Bioethical considerations remain important in the care of babies with multiple congenital anomalies.