Arterial Switch Operation

动脉开关操作
  • 文章类型: Journal Article
    目的:这项研究的目的是评估在北京儿童医院进行动脉转换手术的16年经验,并确定早期和晚期死亡率和晚期发病率。探讨晚期并发症和再干预的危险因素,最后评估新主动脉窦管交界处重建技术是否减少了动脉转换手术的晚期并发症。
    方法:回顾性分析2006年1月至2022年1月在北京儿童医院行大动脉转位手术的185例患者和同期在阜外医院行改良大动脉转位手术的30例患者的临床资料。采用倾向评分匹配法对阜外医院新主动脉窦管结重建患者与北京儿童医院30例非新主动脉窦管结重建患者进行匹配。
    结果:有13例早期死亡(7.03%)和5例晚期死亡(3.01%)。19例患者(11.45%)发生新的主动脉瓣反流,28例患者(16.87%)发生主动脉根部扩张。右室流出道晚期梗阻33例(19.88%)。晚期再干预18例(10.84%)。多因素分析显示主肺内径不匹配,以前的肺动脉带,和轻度中度或以上出院时新发主动脉瓣反流是晚期新发主动脉瓣反流和主动脉根部扩张的独立危险因素。低手术体重是新发主动脉瓣反流的独立危险因素。和二尖瓣天然肺动脉瓣是主动脉根部扩张的独立危险因素。手术年龄大、主动脉根部扩张是晚期右室流出道梗阻的独立危险因素。手术年龄较大,2014年前手术,晚期右室流出道梗阻,晚期主动脉根部扩张是晚期干预的独立危险因素。倾向评分匹配显示新主动脉窦管交界处重建组未随访新主动脉瓣反流和主动脉根部扩张,非新主动脉窦管交界处重建组出现7例主动脉根部扩张和5例新生主动脉瓣反流,分别,差异均有统计学意义(P=0.003;P=0.015)。
    结论:新发主动脉瓣反流的发生率增加,主动脉根部扩张,随着儿童年龄的增长,右心室流出道梗阻是未来主要关注的结果,可能意味着更多的延迟再干预.新主动脉窦管交界处重建技术可降低新发主动脉瓣反流和主动脉根部扩张的发生率,改善动脉转换手术的晚期预后。必须仔细随访新主动脉瓣和根部功能,尤其是主肺直径不匹配的患者,以前的肺动脉带,出院时轻度新发主动脉瓣反流,低手术重量,和二尖瓣天然肺动脉瓣结构。
    OBJECTIVE: The aims of this study were to evaluate the 16-year experience with  arterial switch operation at Beijing Children\'s Hospital and to determine early and late mortality and late morbidity, to explore risk factors for late complications and reintervention, and finally to evaluate whether the neoaortic sinotubular junction reconstruction technique reduces late complications of arterial switch operation.
    METHODS: The clinical data of 185 patients with transposition of the great arteries who underwent arterial switch operation in Beijing Children\'s Hospital from January 2006 to January 2022 and 30 patients who underwent modified arterial switch operation with neoaortic sinotubular junction reconstruction technique in Fuwai Hospital during the same period were retrospectively analysed. Propensity score matching was also used to match the neoaortic sinotubular junction reconstruction patients in Fuwai Hospital with 30 non-neoaortic sinotubular junction reconstruction patients in Beijing Children\'s Hospital.
    RESULTS: There were 13 early deaths (7.03%) and five late deaths (3.01%). Nineteen patients (11.45%) developed new aortic valve regurgitation and 28 patients (16.87%) developed aortic root dilation. Late right ventricular outflow tract obstruction occurred in 33 patients (19.88%). Late reintervention occurred in 18 cases (10.84%). Multivariate analysis showed that aorto-pulmonary diameter mismatch, previous pulmonary artery banding, and mild moderate or above new aortic valve regurgitation at discharge were independent risk factors for late new aortic valve regurgitation and aortic root dilation. Low surgical weight was an independent risk factor specific to new aortic valve regurgitation, and bicuspid native pulmonary valve was an independent risk factor specific to aortic root dilation. Older surgical age and aortic root dilation were independent risk factors for late right ventricular outflow tract obstruction. Older surgical age, operation before 2014, late right ventricular outflow tract obstruction, and late aortic root dilation were independent risk factors for late intervention. Propensity score matching showed that new aortic valve regurgitation and aortic root dilation were not followed up in the neoaortic sinotubular junction reconstruction group, while seven cases of aortic root dilation and five cases of new aortic valve regurgitation occurred in the non-neoaortic sinotubular junction reconstruction group, respectively, and the differences were statistically significant (P = 0.003; P = 0.015).
    CONCLUSIONS: The increased incidence of new aortic valve regurgitation, aortic root dilation, and right ventricular outflow tract obstruction as children age is a major concern outcome in the future and may mean more late reintervention. neoaortic sinotubular junction reconstruction technique may reduce the incidence of new aortic valve regurgitation and aortic root dilation, and improve the late prognosis of arterial switch operation. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with aorto-pulmonary diameter mismatch, previous pulmonary artery banding, mild new aortic valve regurgitation at discharge, low surgical weight, and bicuspid native pulmonary valve structures.
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  • 文章类型: Journal Article
    目的:这项研究的目的是评估我们机构的16年动脉转换手术(ASO)的经验,并确定早期和晚期死亡率和晚期发病率,以及再次手术和导管介入的需要,最后,探讨晚期并发症的危险因素及再干预。
    方法:对2006年1月至2022年1月在本中心接受ASO治疗的185例大动脉转位(TGA)患者的临床资料进行回顾性研究。
    结果:有13例早期死亡(7.03%),5例晚期死亡(3.01%),和6失去了后续。166名住院幸存者的中位随访时间为88.5(2190)个月。中度或以上新发主动脉瓣反流(NAR;在本文中,NAR代表中度或更大的反流,除非另有说明)发生在19例(11.45%),28例(16.87%)发生主动脉根部扩张(ARD)。晚期右室流出道梗阻(RVOTO)33例(19.88%)。有18例患者(10.84%)接受了晚期再干预,最常见的干预指征是RVOTO,在同时进行弓修复和NAR或ARD的患者中,再发生主动脉缩窄。接收机工作特性分析发现,NAR对ARD的预测能力最强,其次是RVOTO,其次是二尖瓣天然肺动脉瓣(BPV),主肺直径不匹配(APDMM)最弱。多变量分析表明,APDMM,先前的肺动脉带(PAB),出院时轻度NAR是晚期NAR和ARD的独立危险因素。低手术体重是NAR特有的独立危险因素,BPV是ARD特有的独立危险因素。手术年龄和ARD是晚期RVOTO的独立危险因素。手术年龄较大,2014年之前运营,RVOTO后期,晚期ARD是晚期干预的独立危险因素。晚期未发现冠状动脉功能障碍的再干预事件。但1例患者在再次手术后因冠状动脉栓塞而发生心肌梗死。
    结论:近几十年来,TGA患者ASO后的早期和晚期生存率得到了显著改善。增加NAR的比率,ARD,复发性主动脉缩窄,随着儿童年龄的增长和RVOTO是未来关注的主要结果,可能意味着更多的延迟再干预。必须仔细随访新主动脉瓣和根部功能,尤其是APDMM患者,上一个PAB,出院时轻度NAR,低手术重量,和BPV结构。
    OBJECTIVE: The purpose of this study was to evaluate our institution\'s 16-year arterial switch operation (ASO) experience and to determine early and late mortality and late morbidity, as well as the need for reoperation and catheter intervention, and finally, to explore risk factors for late complications and reintervention.
    METHODS: The clinical data of 185 transposition of the great arteries (TGA) patients who received ASO treatment in our center from January 2006 to January 2022 were continuously included for retrospective study.
    RESULTS: There were 13 early deaths (7.03%), 5 late deaths (3.01%), and 6 lost to follow-up. The median follow-up time for the 166 hospitalized survivors was 88.5 (2190) months. Moderate or above new aortic valve regurgitation (NAR; in this article, NAR represents moderate or greater reflux unless otherwise specified) occurred in 19 cases (11.45%), and aortic root dilation (ARD) occurred in 28 cases (16.87%). Late right ventricular outflow tract obstruction (RVOTO) occurred in 33 cases (19.88%). There were 18 patients (10.84%) who underwent late re-intervention, and the most common indication for intervention was RVOTO, followed by recurrent aortic coarctation in patients undergoing concurrent arch repair and NAR or ARD. Receiver operating characteristics analysis found that NAR had the strongest predictive power for ARD, followed by RVOTO, followed by bicuspid native pulmonary valve (BPV), and aorto-pulmonary diameter mismatch (APDMM) was the weakest. Multivariate analysis showed that APDMM, previous pulmonary artery banding (PAB), and mild NAR at discharge were independent risk factors for late NAR and ARD. Low surgical weight was an independent risk factor specific to NAR, and BPV was an independent risk factor specific to ARD. Older surgical age and ARD were independent risk factors for late RVOTO. Older surgical age, operation before 2014, late RVOTO, and late ARD were independent risk factors for late intervention. No reintervention events for coronary dysfunction were found in the late stage, but one patient occurred myocardial infarction due to coronary embolism after reoperation.
    CONCLUSIONS: Early and late survival rates after ASO in TGA patients have been remarkably improved in recent decades. Increased rates of NAR, ARD, recurrent coarctation of the aorta, and RVOTO as children age are major future outcomes of concern and may imply more late reinterventions. Careful follow-up of neo-aortic valve and root function is imperative, especially in patients with APDMM, previous PAB, mild NAR at discharge, low surgical weight, and BPV structures.
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  • 文章类型: Journal Article
    在16年的时间内,对225例Taussig-Bing异常的原发性动脉转换手术,确定与死亡率和再干预相关的危险因素。从2002年到2017年,在上海儿童医学中心接受了225例Taussig-Bing异常的初级动脉转换手术。收集围手术期资料及随访结果。采用单因素和多因素分析探讨与早期死亡相关的危险因素。竞争风险分析用于识别与再干预相关的风险因素。早期死亡率为12.9%(29/225),长期生存率令人满意(10年生存率85.0%)。修复时的中位年龄为77天(四分位距,IQR,48-139)。中位随访时间为4.6年(范围0.1-18.3年)。87名儿童(38.7%)同时接受了主动脉弓修复术。延长体外循环时间(a-OR1.18,95%置信区间[CI],1.09-1.28,p<0.001)被发现是早期死亡的独立危险因素。较大的修复体重往往是保护因素(a-OR0.66,95%CI,0.425-1.02,p=0.060),壁内冠状动脉(a-OR4.81,95%CI,0.927-24.9,p=0.062)往往是早期死亡的危险因素。5年总再干预的累积发生率为18.9%(95%CI,10.3%-27.4%),10年为32.3%(95%CI,17.0%-47.6%)。没有发现长期整体再干预的独立危险因素。延长的主动脉阻断时间是长期右侧再干预的独立危险因素(调整后的风险比[a-HR]1.12,95%CI1.005-1.25,p=0.041)。新主动脉瓣反流是一个值得关注的问题,10年时中度或更高的新AR的发生率为16.1%(95%CI7.6%-24.7%)。在Taussig-Bing异常的初级动脉转换手术中,壁内冠状动脉仍然是手术挑战。ASO时体重较大往往是早期死亡的保护因素。再干预通常是必要的,但可以以令人满意的结果进行。
    To identify risk factors associated with mortality and reintervention on primary arterial switch operation for Taussig-Bing anomaly in 225 cases over a 16-year period. From 2002 to 2017, 225 children with Taussig-Bing anomaly received a primary arterial switch operation at the Shanghai Children\'s Medical Center. Perioperative data and follow-up results were collected. Univariate and multivariable analysis was used to explore risk factors associated with early mortality. The competing risk analysis was used to identify risk factors related to reintervention. Early mortality was 12.9% (29/225) with a satisfactory long-term survival rate (10-year survival rate 85.0%). The median age at repair was 77 days (interquartile range, IQR, 48-139). The median duration of follow-up was 4.6 (range 0.1-18.3) years. 87 children (38.7%) received concomitant aortic arch repair. Prolonged cardiopulmonary bypass time (a-OR 1.18, 95% confidence interval [CI], 1.09-1.28, p < 0.001) is found to be an independent risk factor for early death. Larger weight at repair tends to be a protective factor (a-OR 0.66, 95% CI, 0.425-1.02, p = 0.060) and intramural coronary artery (a-OR 4.81, 95% CI, 0.927-24.9, p = 0.062) tends to be a risk factor for early mortality. The cumulative incidence rate of overall reintervention was 18.9% (95% CI, 10.3%-27.4%) at 5 years and 32.3% (95% CI, 17,0%-47.6%) at 10 years. No independent risk factors were identified for long-term overall reintervention. Prolonged aortic-cross clamp time was an independent risk factor for long-term right-sided reintervention (adjusted hazard ratio [a-HR] 1.12, 95% CI 1.005-1.25, p = 0.041). Neo-aortic regurgitation was a concern with an incidence rate of moderate or greater neo-AR of 16.1 % (95% CI 7.6%-24.7%) at 10 years. Intramural coronary artery remains a surgical challenge in primary arterial switch operation for the Taussig-Bing anomaly. Larger weight at ASO tends to be a protective factor for early death. Reintervention is frequently necessary but can be performed with satisfactory results.
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  • 文章类型: Journal Article
    目的:冠状动脉解剖对接受动脉转换手术(ASO)的患者预后的影响目前存在争议,在更复杂的患者中,这种手术的危险因素可能会发生变化。本研究旨在探讨冠状动脉异常对大动脉转位(TGA)和Taussig-Bing异常(TBA)患者ASO院内和出院后预后的影响。
    方法:我们回顾性分析了2007年1月至2019年12月接受ASO的206例患者。手术年龄中位数为33[四分位距(IQR):20-71]天。中位随访时间为7.2年(IQR:4.0-10.3年)。
    结果:86例患者(41.7%)出现冠状动脉异常,其中9人(4.4%)有单冠状动脉。其他冠状动脉特征包括5例(2.4%)患者的壁内病程,1例(0.5%)患者口狭窄,5例(2.4%)患者的副冠状动脉孔。有32例(15.5%)住院死亡,8例(4.6%)出院后死亡,总生存率为81.3%,1年、5年和10年分别为80.7%和79.9%,分别。自2013年以来,ASO导致的死亡率急剧下降。单支冠状动脉患者的院内死亡率较高,但这一发现没有统计学意义。较早的手术时间(OR:2.756)和较长的体外循环时间(OR:2.336)与住院死亡率显着相关,而冠状动脉模式没有。壁冠状动脉(HR:10.034)和ASO时年龄大于1岁的患者(HR:9.706)是出院后死亡率的独立预测因素。
    结论:ASO仍然是有冠状动脉异常的TGA的首选方法,在总体生存率和再次手术的自由方面具有可接受的院内和出院后结局。然而,壁冠状动脉是出院后死亡的独立危险因素。在生命的第一年内及时手术有助于提高ASO的总体中期生存率。
    OBJECTIVE: The influence of the coronary artery anatomy on the prognosis of patients receiving an arterial switch operation (ASO) is currently controversial, and the risk factors for this operation may change in more complicated patients. This study aimed to investigate the influence of coronary artery anomalies on the in-hospital and post-discharge outcomes of ASO in patients with transposition of the great arteries (TGA) and Taussig-Bing anomaly (TBA).
    METHODS: We retrospectively reviewed 206 patients who underwent ASO from January 2007 to December 2019. The median age at operation was 33 [interquartile range (IQR): 20-71] days. Median follow-up time was 7.2 years (IQR: 4.0-10.3 years).
    RESULTS: Coronary anomalies were present in 86 patients (41.7%), with 9 (4.4%) of them having a single coronary artery. Additional coronary features included intramural courses in 5 (2.4%) patients, ostial stenosis in 1 (0.5%) patient, and accessory coronary artery orifices in 5 (2.4%) patients. There were 32 (15.5%) in-hospital deaths and 8 (4.6%) post-discharge deaths, yielding an overall survival of 81.3%, 80.7% and 79.9% at 1, 5 and 10 years, respectively. Mortality due to ASO has been drastically decreased since 2013. Patients with a single coronary artery had higher rate of in-hospital mortality, but this finding was not statistically significant. The earlier surgical era (OR: 2.756) and a longer cardiopulmonary bypass time (OR: 2.336) were significantly associated with in-hospital mortality, while coronary patterns were not. An intramural coronary artery (HR: 10.034) and a patient age of older than 1 year at the time of ASO (HR: 9.706) were independent predictors of post-discharge mortality.
    CONCLUSIONS: ASO remains the procedure of choice for TGA with coronary anomalies with acceptable in-hospital and post-discharge outcomes in terms of overall survival and freedom of reoperation. However, intramural coronary artery is an independent risk factor for post-discharge mortality. Timely surgery within the 1st year of life helps improve overall midterm survival of ASO.
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  • 文章类型: Journal Article
    背景:我们假设术前患者特征和分支肺动脉(PA)大小可能会影响大动脉转位患者接受动脉转换手术(ASO)的术后分支PA再干预率。
    方法:回顾性单中心研究包括262名连续(2008-2017年)接受ASO的新生儿。人口特征,超声心动图,并对临床结果进行了回顾。竞争风险分析对分支PA再干预和特定原因风险回归的发生率进行建模,以进行预测分析。
    结果:年龄和体重中位数为7(范围,5-11)天和3.4(范围,3.1-3.8)kg,分别。28例(10.7%)患者需要进行各种类型的早期分支PA再干预(在重症监护病房住院期间同时进行修正或再干预)。这些患者通气时间延长(P<.001),重症监护病房持续时间(P<.001),右心室功能较差(P=0.043),住院死亡率高(P=.010)。与基线测量相比,ASO后分支PA尺寸立即显着降低。中位随访时间为20.8(范围,0.9-44.7)个月。分支PA再干预在没有早期再干预的幸存者中很常见(9.4%),在早期再干预者中更为频繁(25%)。在初次分支PA再干预后,超过三分之一的患者需要随后的再干预(全部基于导管)。多变量分析显示左侧PA的术前维度(风险比,0.527[95%CI,0.337-0.823];P=.005),和右PA(危险比,0.503[95%CI,0.318-0.796];P=.003)与末梢分支PA再干预独立相关。
    结论:分支PA再干预是常见的,在ASO后通常需要手术或基于导管的再干预。ASO后PA分支直径明显变小。较小的术前分支PA预测晚期分支PA再干预,表示此效果的几何公差余量较小。
    BACKGROUND: We hypothesized that preoperative patient characteristics and branch pulmonary artery (PA) size might influence the rate of postoperative branch PA reintervention in patients with transposition of the great arteries who undergo the arterial switch operation (ASO).
    METHODS: The retrospective single-center study included 262 consecutive (2008-2017) newborns who underwent the ASO. Demographic characteristics, echocardiography, and clinical outcomes were reviewed. Competing risk analysis modeled incidence of branch PA reintervention and cause-specific hazard regression for predictors analyses.
    RESULTS: Median age and weight were 7 (range, 5-11) days and 3.4 (range, 3.1-3.8) kg, respectively. Various types of early branch PA reinterventions (concomitant revision or reintervention during the intensive care unit stay) were required in 28 (10.7%) patients. These patients had prolonged ventilation (P < .001), intensive care unit duration (P < .001), worse right ventricular function (P = .043), and high in-hospital mortality (P = .010). Branch PA dimensions significantly decreased immediately after ASO compared with baseline measurements. The median follow-up duration was 20.8 (range, 0.9-44.7) months. Branch PA reintervention was common among survivors without early reinterventions (9.4%), and even more frequent among those with early reinterventions (25%). Subsequent reintervention (all catheter-based) was necessary for more than one-third of patients after initial branch PA reintervention. The multivariable analysis showed preoperative dimension of the left PA (hazard ratio, 0.527 [95% CI, 0.337-0.823]; P = .005), and right PA (hazard ratio, 0.503 [95% CI, 0.318-0.796]; P = .003) were independently associated with late branch PA reinterventions.
    CONCLUSIONS: Branch PA reintervention was common and often required surgical or catheter-based reinterventions after ASO. PA branch diameters became significantly smaller after ASO. Smaller preoperative branch PA predicted late branch PA reintervention, indicating a smaller margin of geometrical tolerance to this effect.
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  • 文章类型: Journal Article
    这项研究调查了2期动脉转换手术(ASO),以治疗晚期转诊患者的室间隔完整的大动脉转位(TGA-IVS)。
    我们回顾性分析了2007年2月至2018年8月在我们机构接受2期ASO的TGA-IVS或TGA限制性室间隔缺损患者。包括41例患者:21例(51.2%)经历了长期2期ASO,20例(48.8%)经历了快速2期ASO。
    长期2期组在ASO时年龄较大(3.5vs25个月;P<.001)。结果在长期组优于快速组重症监护病房时间(P=0.004),机械通气时间(P=.004),和停留时间(P=0.007)。长期组没有发生院内死亡,长期组的术后病程比快速组更易于管理。然而,快速组显著的新主动脉瓣反流风险较低,也有较好的左心室射血分数。
    长期组取得了比快速组更好的早期结果。然而,我们还注意到新主动脉瓣反流和心肌功能障碍的高风险.
    This study investigated a 2-stage arterial switch operation (ASO) to treat transposition of the great arteries (TGA) with intact ventricular septum (TGA-IVS) in late referral patients.
    We retrospectively analyzed patients with TGA-IVS or TGA with restricted ventricular septal defects who had undergone 2-stage ASO at our institution from February 2007 to August 2018. Included were 41 patients: 21 (51.2%) who had undergone long-term 2-stage ASO and 20 (48.8%) who had undergone rapid 2-stage ASO.
    The long-term 2-stage group was older at ASO (3.5 vs 25 months; P < .001). Results were more satisfactory in the long-term group than in the rapid group for intensive care unit time (P = .004), mechanical ventilation time (P = .004), and length of stay (P = .007). No in-hospital death occurred in the long-term group, and the postoperative course was more manageable in the long-term group than in the rapid group. However, the risk of significant neoaortic regurgitation was lower in the rapid group, which also had a better left ventricular ejection fraction.
    The long-term group achieved better early-term outcomes than the rapid group. However, a high risk of neoaortic regurgitation and myocardial dysfunction was also noted.
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  • 文章类型: Journal Article
    回顾在我们中心接受动脉转换手术(ASO)的大动脉转位(TGA)和壁内冠状动脉(IMCA)患者的早期和中期结局。在2010年至2018年间接受ASO的450例TGA患者中,有26例(5.8%)患者被确定为IMCA。26例中有21例患者的左冠状动脉位于壁内。我们对所有26例患者都采用了双冠状动脉按钮和无顶壁内病程的冠状动脉转移。患有IMCA的患者的早期死亡率为26人中的3人(11.5%),而没有IMCA的患者为424人中的10人(2.4%)(p=0.007)。6例患者发生重大不良事件,包括3例患者的体外膜氧合支持,6例延迟胸骨闭合。所有23名幸存者都可以进行随访,平均随访73.5±28.7个月。没有晚期死亡和再干预,所有患者在末次随访时无症状。一名患者表现出中度新肺返流,1例患者出现右肺动脉远端狭窄。对于患有TGA和IMCA的患者,使用双冠状动脉按钮和无顶壁内路线的冠状动脉转移是一个不错的选择。有了这项技术,ASO可以以最佳的早期和中期结果进行。
    To review the early and intermediate outcomes of patients with transposition of the great arteries (TGA) and intramural coronary artery (IMCA) who underwent arterial switch operation (ASO) at our center. Among 450 patients with TGA who underwent an ASO between 2010 and 2018, 26 (5.8%) patients were identified with IMCA. The left coronary artery was intramural in 21 of 26 patients. We adopted coronary transfer using double coronary buttons with unroofed intramural course for all 26 patients. Early mortality for patients with IMCA was 3 of 26 (11.5%) compared with 10 of 424 (2.4%) for those without IMCA (p = 0.007). Six patients suffered major adverse events, including extracorporeal membrane oxygenation support in 3 patients, delayed sternal closure in 6 patients. The follow-up was available for all 23 survivors, with the mean follow-up period of 73.5 ± 28.7 months. There was no late death and reinterventions, and all patients were asymptomatic at last follow-up. One patient exhibited moderate neopulmonary regurgitation, and 1 patient presented with distal stenosis of the right pulmonary artery. Coronary transfer using double coronary buttons with unroofed intramural course was a good option for patients with TGA and IMCA. With this technique, ASO could be performed with optimal early and intermediate outcomes.
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  • 文章类型: Journal Article
    这项研究的目的是评估和比较改良的脑室(REV)和Rastelli手术治疗大动脉转位(TGA)的结果,室间隔缺损(VSD),和肺动脉狭窄(PS)。记录了38例接受改良REV(n=16)或Rastelli手术(n=22)治疗TGA的患者,VSD,并对2010年至2019年的PS进行了审查。中位年龄为2.2岁(范围0.6-8.0岁),中位体重为11.3kg(范围6.4-22.0kg)。没有发生院内死亡,有4例早期再次手术(每组2例)。10年总生存率为97.4%(改良REV组为100%,Rastelli组为95.5%,P=0.39)。两组的左心室流出道(LVOT)再手术自由度均为100%。改良REV组无右室流出道(RVOT)再手术率为100%,Rastelli组为75.4%(P=0.073)。改良REV组无事件生存率为100%,Rastelli组为72.0%(P=0.048)。最近的超声心动图显示,所有患者的LVOT峰值梯度均小于10mmHg。在ModifiedREV组中,30.8%的患者(4/13)患有RVOT梗阻(RVOT峰值梯度超过40mmHg)或中度或重度肺功能不全,而Rastelli组有25.0%(3/12)的患者发现导管狭窄(峰值梯度大于40mmHg)。改良的REV和Rastelli手术提供了令人满意的早期结果,以及长期生存和LVOT性能。然而,改进的REV具有更好的RVOT性能。
    The objective of this study was to evaluate and compare the results of the modified réparation à l\'ètage ventriculaire (REV) and the Rastelli operation for the treatment of transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS). Records of 38 patients who underwent the modified REV (n = 16) or the Rastelli operation (n = 22) for the treatment of TGA, VSD, and PS between 2010 and 2019 were reviewed. The median age was 2.2 years (range 0.6-8.0 years) and the median weight was 11.3 kg (range 6.4-22.0 kg). No in-hospital death occurred and there were 4 early reoperations (two in each group). Overall survival at 10 years was 97.4% (100% in Modified REV group and 95.5% in Rastelli group, P = 0.39). Freedom from left ventricular outflow tract (LVOT) reoperation was 100% in both groups. Freedom from right ventricular outflow tract (RVOT) reoperation was 100% in Modified REV group and 75.4% in Rastelli group (P = 0.073). Event-free survival was 100% in Modified REV group and 72.0% in Rastelli group (P = 0.048). The most recent echocardiography showed that LVOT peak gradient was less than 10 mmHg in all patients. In Modified REV group, 30.8% of patients (4/13) had either RVOT obstruction (RVOT peak gradient more than 40 mmHg) or moderate or severe pulmonary insufficiency, while conduit stenosis (peak gradient more than 40 mmHg) was found in 25.0% of patients (3/12) in Rastelli group. The modified REV and the Rastelli operation provide satisfactory early results, as well as long-term survival and LVOT performance. However, the modified REV has better RVOT performance.
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  • 文章类型: Case Reports
    大动脉转位(TGA)和主动脉弓中断(IAA)是罕见的先天性心脏病。TGA和IAA之间的关联很少。本研究的目的是提出一个TGA和IAA组合的病例,他们接受了一期修复,并回顾了类似病例的文献。1个月大的患者因呼吸急促和紫癜入院。由于缺乏产前检查,导致诊断延迟。超声心动图和计算机断层扫描血管造影证实TGA伴有前-后定向大动脉,宽动脉导管未闭,B型IAA,室间隔缺损(VSD)和肺动脉高压。患者接受了导致VSD闭合的单阶段初级手术修复过程,2019年10月主动脉弓和动脉转换手术的重建。患者在术后3个月的随访中表现良好。超声心动图提示心室的收缩功能正常,主动脉瓣和肺动脉瓣的反流轻微。结论:采用VSD闭合的单阶段修复,主动脉弓重建和动脉转换手术可能是大多数TGA和IAA合并患者的适用方法。需要长期随访,因为复发性缩窄的再干预率高,主动脉瓣上狭窄,新主动脉瓣反流,据报道,右心系统阻塞和冠状动脉狭窄。
    Transposition of the great arteries (TGA) and interruption of the aortic arch (IAA) are uncommon congenital heart diseases. The association between TGA and IAA is rare. The aim of this study is to present a case with combined TGA and IAA, who underwent the primary repair and review the literature with similar cases. The one-month-old patient was admitted with tachypnea and cyanosis. Delayed diagnosis was caused due to the absence of prenatal examination. Echocardiography and computed tomography angiography confirmed TGA with anterior-posterior-oriented great arteries, wide patent ductus arteriosus, type B IAA, ventricular septal defect (VSD) and pulmonary arterial hypertension. The patient underwent a single-stage primary surgical repair process leading to VSD closure, reconstruction of the aortic arch and arterial switch operation in October 2019. The patient is doing well at a 3-month follow-up post-surgery. The echocardiogram suggests a normal systolic function of the ventricles and trivial regurgitation for both aortic and pulmonary valves. CONCLUSIONS: The single-stage repair with VSD closure, reconstruction of aortic arch and arterial switch operation might be an applicable approach for most of the patients with combined TGA and IAA. Long term follow-up is required as a high re-intervention rate for recurrent coarctation, supravalvular aortic stenosis, neoaortic valve regurgitation, obstruction of the right heart system and coronary stenosis has been reported.
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