一期大动脉调转术治疗完全性大动脉转位

目的:探讨一期大动脉调转术治疗完全性大动脉转位(TGA)的手术适应证及手术效果.方法:2006-05-2007-08对15例患儿[年龄0.5～14(2.51±0.76)个月,体重3～9(3.73±0.86)kg]实施一期大动脉调转手术.15例中室间隔完整TGA(TGA/IVS)13例,均并发房间隔缺损(ASD),同时并发动脉导管未闭(PDA)11例;TGA 并发室间隔缺损(TGA/VSD)2例,均同时并发PDA和ASD.12例并发中度肺动脉高压,3例并发重度肺动脉高压,术前均使用前列腺素E1.手术在全麻、中低温、低流量体外循环下完成.在2大动脉瓣上方横断,将左、右冠状动脉开口移植至肺动脉近端.经肺动脉分叉下方,吻合肺动脉近端和升主动脉远端.用自体心包修复主动脉近端缺失部分,并在新主动脉开放后吻合主动脉近端和肺动脉远端.结果:3例TGA/IVS患儿术后肺部感染,2例(分别为13个月、3个月龄)患儿术后12 h内突发心室颤动死亡,病死率为13.3%.未发生与冠状动脉移植相关并发症.术前肺动脉高压患儿,特别是重度肺动脉高压,术后肺动脉压力均明显下降.13例术后顺利恢复出院.随诊3～12个月,全部患儿心功能恢复良好,无远期并发症和死亡.结论:一期大动脉调转手术对于TGA有较好早期效果.对于年龄超过4个月部分患儿,即使并发肺动脉压,仍可取得满意治疗效果.     

完全性大动脉转位的急诊外科治疗

本文对先天性畸形完全性大动脉转位外科治疗的术前准备与术式选择进行了回顾介绍，供临床参考。     

矫正型大动脉转位的二维超声心动图诊断

本文应用二维(2DE)及多普勒(DE)超声心动图检查19例矫正型大动脉转位(C-TGA),在左室长轴、四腔心、大动脉和心室短轴切面及剑下探查,能准确显示内脏、心室、房室瓣、大动脉位置及其相互关系。合并先天性心内畸形占89.4%。与手术或心血管造影对照诊断正确者18例,占94.7%。结果认为2DE 对 C-TGA 有特异的诊断价值。     

完全性大动脉转位行大动脉调转术16例麻醉分析

目的总结完全性大动脉转位行大动脉调转术的麻醉处理体会。方法回顾性分析16例完全性大动脉转位行大动脉调转术的麻醉资料。全组患儿均在中低温低流量体外循环下行大动脉调转术及冠状动脉移植术。人室后肌注氯胺酮行基础麻醉，麻醉药物选用芬太尼、咪达唑仑和维库溴铵，术中监测心电图、动脉血压、中心静脉压、左房压、体温、经皮血氧饱和度、呼气末二氧化碳、尿量和血气分析等，主动脉开放后给予多巴胺、肾上腺素、硝酸甘油、米力农、异丙肾上腺素等血管活性药以维持血流动力学稳定，根据中心静脉压和左房压控制体外循环后液体回输速度。结果麻醉经过平稳，无麻醉并发症。术中2例死于重度低心排，不能脱离体外循环。结论完全性大动脉转位患儿年龄小，全身情况及心功能差，充分的术前准备、平稳的麻醉诱导及维持、正性肌力药及血管扩张药的合理使用是顺利完成手术的关键。     

大动脉转位的二维超声诊断

目的　应用二维超声心动图诊断大动脉转位 (TGA)。方法　以超声节段法诊断 TGA。结果　 11例 TGA中 ,SLL 型 5例 ,IDD型 1例 ,SDD型 4例 ,Taussig-Bing病 1例。合并畸形中以 VSD、ASD伴 PS最多 ,占 88%。结论　二维超声诊断大动脉转位 ,是一种无创性可靠的方法 ,可取代有创性 X线造影来诊断 TGA,对于正确认识与鉴别此种疾病有着重要的临床意义     

完全性和矫正性大动脉转位的眼声心动图诊断及误诊分析

作者报告经手术或心血管造影证实的完全在动脉转位４例、矫正性大动脉转位９例的超声结果，超声诊断符合率７６．９％（１０／１３例）。大动脉转位并发畸形以室间隔缺损及肺动脉狭窄最多见，其次为房间隔缺损。本组误诊为右室双出口３例，其原因与室缺较大、大动态远离间隔、空间隔移位和切面选择等有关。作者认为大动脉转位选择剑突下切面观察最佳。     

完全性大动脉转位冠状动脉解剖类型55例分析

目的 总结５５例大动脉转位手术病例及自然死亡病例,就完全性大动脉转位（ＴＧＡ）的冠状动脉解剖苤同类型冠状动脉与大血管间相对位置关系。不典型冠状动脉发生率与合并心内畸型的关系作一分析。方法 ５５例病例分成两组：Ａ组室间隔完整型（ＴＧＡ＋ＩＶＳ）共２７例,Ｂ组伴有室间隔缺损（ＴＧＡ＋ＶＳＤ）共２８例,其中３８例作外科纠治术,术中直视下观察冠状动脉走向,其余有室间隔缺损（ＴＧＡ＋ＶＳＤ）共２８例,其中３     

时空关联成像技术诊断胎儿完全性大动脉转位的价值

目的 进一步提高完全性大动脉转位（CTCA）的诊断水平。方法对15例二维超声筛查疑诊CTCA的中晚孕期胎儿行三维超声时空关联成像技术（STIC）,并与尸解或出生后彩色多普勒超声检查结果比较。结果15例胎儿均获得满意胎儿心脏容积数据,其中14例与尸解剖或产后复查结果相一致。结论STIC技术为诊断胎儿CTCA的较好方法。     

“绿色通道”救治新生儿室间隔完整型完全性大动脉转位

目的:回顾性总结经先天性心脏病(先心病)三级预防网络"绿色通道"救治新生儿室间隔完整型完全性大动脉转位的临床经验。方法:收集2010年1月至2012年12月,经"绿色通道"转诊至我院的8例室间隔完整型完全性大动脉转位新生儿临床资料,总结分析临床疗效及转归。结果:8例患儿,男性5例,女性3例。入院年龄2～12 d,平均(5.9±2.7)d,手术日龄2～14 d,平均(8.1±2.6)d,体质量2.3～7.5 kg,平均(3.5±0.6)kg。术前严重酸中毒,呼吸衰竭1例行气管插管治疗,1例合并肾衰竭,所有患儿均直接行(arterial switch operation,ASO),手术成功率100%。术后延迟关胸5例,围术期死亡1例(12.5%),死因为多脏器功能衰竭。术后3～6个月,2次随访。手术治愈的7例患儿生长发育良好,左心室心功能正常范围,平均左心室射血分数(LVEF)分别为(67.0±2.8)%和(70.3±4.0)%,左心室心肌质量指数也显著增高。结论:完全性大动脉转位患儿应尽早诊断,提高产前诊断率加强术前内科治疗,纠正代谢紊乱,维护心功能,把握手术时机,尽快根治是成功的关键。通过先心病三级预防框架实施"绿色通道"救治室间隔完整型完全性大动脉转位,对提高危重患儿存活率,减少新生儿死亡有重要意义。     

先天性心脏病大动脉右转位二维超声图像特征

目的 :探索大动脉右转位先天性心脏病 (先心病 )二维超声心动图 (2DE)图像特征及规律性。方法 :应用 2DE检查 344例大动脉右转位先心病 ,316例经心血管造影对照 ,193例经手术证实。结果 :344例大动脉右转位与 8种先心病共存。在 2DE胸骨旁短轴切面上 332例 (占 96 .5 % ) 2条大动脉呈 2个环状回声 ,12例 (占3.5 % )只显示主动脉 1个环状回声。 30 2例肺动脉狭窄 (PS)中 2 90例显示 2个环状回声后方内径明显小于前方内径 ,12例只显示主动脉 1个环状回声。 4 2例肺动脉高压 (PH)中 31例 2个环状回声后方内径明显大于前方内径 ,11例 2个环状回声内径大致相等。结论 :大动脉右转位与 8种先心病共存 ;在 2DE胸骨旁大动脉短轴切面上显示 2条大动脉呈 2个环状回声多见 ,显示主动脉 1个环状回声少见 ;根据 2个环状回声内径比较可判定是PS还是PH。     

Development of the coronary arteries in a murine model of transposition of great arteries

Transposition of great arteries in humans is associated with a wide spectrum of coronary artery patterns. However, no information is available about how this pattern diversity develops. We have studied the development of the coronary arteries in mouse embryos with a targeted mutation of perlecan, a mutation that leads to ventriculo-arterial discordance and complete transposition in about 70% of the embryos. The perlecan-deficient embryos bearing complete transposition showed a coronary artery pattern consisting of right and left coronary arteries arising from the morphologically dorsal and ventral sinuses of Valsalva, respectively. The left coronary artery gives rise to a large septal artery and runs along the ventral margin of the pulmonary root. In the earliest embryos where transposition could be confirmed (12.5 d post coitum), a dense subepicardial vascular plexus is located in this ventral margin. In wild-type mice, however, capillaries are very scarce on the ventral surface of the pulmonary root and the left coronary artery runs dorsally to this root. We suggest that the establishment of the diverse coronary artery patterns is determined by the anatomical arrangement and the capillary density of the peritruncal vascular plexus, a plexus that spreads from the atrio-ventricular groove and grows around the aortic or pulmonary roots depending on the degree of the short-axis aortopulmonary rotation. This simple model, based on very few assumptions, might explain all the observed variation of the coronary artery patterns in humans with transposition, as well as our observations on the perlecan-deficient and the normal mice.     

Outcome in adult patients after arterial switch operation for transposition of the great arteries

Background

The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist.

Methods and results

We studied 145 adult patients (age > 16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (> 45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7–5.4, P < 0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood.

Conclusions

Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.     

Diastolic ventricular interaction in patients after atrial switch for transposition of the great arteries: A speckle tracking echocardiographic study

Background

We tested the hypothesis that diastolic ventricular interaction occurs after atrial switch operation for transposition of the great arteries (TGA) and that subpulmonary LV diastolic function is influenced by septal geometry.

Methods

Twenty-nine patients (male 19) after atrial switch operation for TGA aged 20.8 ± 4.1 years and 27 healthy controls were studied. Two-dimensional longitudinal systolic strain, systolic (SRs), early diastolic (SRe), and late diastolic (SRa) strain rates of both ventricles were determined using speckle tracking echocardiography. Early diastolic trans-atrioventricular velocity (E) and myocardial early diastolic myocardial velocity (e) at the ventricular free wall-annular junction were measured. Geometry of the morphologic left ventricle was quantified by the diastolic eccentricity index (EI).

Results

In both systemic and subpulmonary ventricles, SRe and SRa were significantly lower and trans-atrioventricular E/e ratios higher in patients than controls (all p < 0.001). In patients, RV SRe correlated with left ventricular (LV) SRe (r = 0.49, p = 0.008), and RV SRa correlated with LV SRa (r = 0.46, p = 0.01). Significant leftward shifting of the septum in patients was reflected by the greater LV EI (p < 0.001). In patients, LV EI correlated with age- and sex-adjusted z score of LV end-diastolic volume. As a group, LV EI correlated negatively with LV SRe (r =−0.62, p < 0.001) and LV SRa (r = − 0.51, p < 0.001), and positively with mitral E/e ratio (r = 0.33, p = 0.02).

Conclusions

Systemic RV diastolic dysfunction occurs after atrial switch operation and correlates with subpulmonary LV diastolic dysfunction. The observed diastolic ventricular interaction may potentially be mediated through alteration of septal geometry.     

Transposition of the great arteries

Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle supporting the systemic circulation. It has been also reported previously undiagnosed congenitally corrected transposition of the great arteries. We present the complete transposition of the great arteries in an 8-year-old Romanian child with a septal defect of the ventricle (functionally single ventricle).     

Simple D-transposition of great arteries operated at the age of 11 years

The simple transposition of the great arteries is a lethal congenital heart disease. The life expectancy of unoperated patients is about 9 months.We report the original observation of a girl with unoperated simple transposition of the great arteries, who survived until the age of 11 years. An atrial switch was successfully performed according to the technique of Senning–Mustard.     

完全性大动脉转位超声心动图图像特征及规律性研究

目的 探寻完全性大动脉转位(TGA)超声心动图(ECG)图像特征及规律性.方法 应用ECG检查110例TGA,所有病例均经心血管造影或(和)手术证实.结果 根据ECG图像特征对104例做出正确诊断,诊断准确率为94.5%,5例误诊右心室双出口,1例误诊单心室.TGA的ECG图像特征及规律性明显:①二维超声心动图(2DE)胸骨旁左室长轴切面显示主动脉在前方发自右心室,肺动脉在后方发自左心室.②2DE胸骨旁大动脉短轴切面显示两条大动脉呈两个环状回声,称2DE"双环征",根据两环相互位置关系判断转位的类型并根据两环内径鉴别肺动脉狭窄(PS)和肺动脉高压(PH).③合并室间隔缺损的TGA多见,室间隔完整的TGA少见.后者均与房间隔缺损(ASD)和动脉导管未闭(PDA)并存.④在临床上有室间隔缺损的TGA合并PS患者存活时间较长;合并PH患者生存时间明显缩短.室间隔完整的TGA患儿,生存时间长短与ASD和PDA的直径大小有关,缺损直径越大患儿生存时间相对较长.结论 TGA的ECG图像特征及规律性明显,ECG对TGA有特异性诊断价值,对新生儿TGA完全可以取代有创的心血管造影检查.     

Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries.

AIMS: To investigate magnitude and determinants of risks during pregnancy in women with Mustard or Senning repair for complete transposition of the great arteries (TGA). METHODS AND RESULTS: Using a nationwide registry (CONCOR), 70 women with Senning (23%) or Mustard (77%) repair for TGA were enrolled. A total of 28 patients had 69 pregnancies (two twins), including 17 spontaneous miscarriages and three elective abortions. During 39 of the 49 completed pregnancies, complications were observed. The most important cardiac complication was clinically significant arrhythmia (n=11, 22%), especially occurring in patients with a prior history of arrhythmia. Important general pregnancy complications were preeclampsia (n=5, 10.2%) and pregnancy-induced hypertension (n=4, 8.2%). Obstetric complications included premature rupture of membranes (n=7, 14.3%), premature labour (n=12, 24.4%), premature delivery (n=16, 31.4%), and thrombo-embolic complications (n=2, 4.1%). Mean (singleton) pregnancy duration was 36+/-5 weeks. Eleven of the 51 children (21.6%) were small for gestational age. Foetal and neonatal mortality combined was 11.8% (n=6). No recurrence of congenital heart disease in the offspring was documented. CONCLUSION: In this largest report on pregnancy in women with atrial-corrected TGA to date, a high incidence of obstetric complications and mortality in the offspring was observed.     

Long-term growth following neonatal anatomic repair of transposition of the great arteries

Despite generally normal prenatal growth, surviving infants with transposition of the great arteries (TGA) frequently develop severe and progressive growth impairment which is not always fully reversed by elective atrial repair within the first year of life. This study was undertaken to determine the effect of neonatal anatomic repair of TGA on long-term growth. Twenty-three children with uncomplicated TGA were followed for a mean of 60 (12–90) months after anatomic repair at a mean age of 11 (1–40) days. Standardized measurements of weight, height, and head circumference for both patients and normal siblings were expressed as percentiles as well as in Z scores (in standard deviations from the mean for age and sex) based on internationally recognized standards. At latest follow-up, 22 (96%) of the patients were above the 3rd percentile for weight and 21 (91%) for both height and head circumference, with 13 (57%), 11 (48%), and 13 (57%) above the 50th percentile for each respective parameter. The mean Z scores (± SD) for weight, height, and head circumference for the patient group were – 0.1 ± 1.2, – 0.2 ± 1.3, and – 0.1 ± 1.1, respectively, and did not differ significantly from those of the reference population (p > 0.05 for each comparison). Paired comparisons of mean Z scores for each growth parameter with those of 35 normal siblings demonstrated no significant difference for weight or height and a small but significant difference for head circumference. Age at surgical repair (within the first 6 weeks of life), duration of follow-up and the development of moderate supravalvar pulmonary stenosis were not statistically related to long-term growth. These results indicate that in patients without extracardiac abnormalities, neonatal anatomic repair of uncomplicated TGA results in normal long-term growth.