伴左心室流出道梗阻的完全性大动脉转位行动脉调转术后左心室流出道及主动脉瓣功能随访

目的探讨伴左心室流出道梗阻的完全性大动脉转位患者行动脉调转术后,左心室流出道梗阻的改善情况及主动脉瓣功能情况。方法 2002~2013年共549例患儿于阜外心血管病医院行动脉调转术,其中42例患者合并左心室流出道梗阻,其中男31例、女11例,中位月龄12个月(7 d至96个月);中位体重6.5(3.5~26.0)kg,外周经皮血氧饱和度52%~85%;左心室流出道病变类型包括肺动脉瓣异常,瓣下隔膜,隧道样狭窄,肌性狭窄,附属瓣膜组织及复合病变。术中根据病变类型采取不同方法:瓣交界粘连行交界切开,瓣下隔膜予以切除,单纯肌性狭窄则切除肥厚肌束或部分室间隔,环形或隧道样狭窄则切除纤维组织和肥厚肌肉,副瓣样组织或无功能腱索,予以切除,通过室间隔缺损跨越至左心室的腱索,切下重植。结果平均体外循环时间147~344(193.5±73.1)min,主动脉阻断时间139(109~305)min,呼吸机使用时间36(3~960)h,住ICU时间5(1~48)d。体外膜式氧合(ECMO)辅助3例,均成功撤除。早期死亡2例,1例为多器官功能衰竭,1例为严重感染。随访期间死亡1例,原因不明,失访3例,接受随访患者36例,随访时间24(3~116)个月;再发左心室流出道梗阻1例,为瓣下局限增厚纤维组织所致,新主动脉瓣轻度狭窄1例,新主动脉瓣少量反流11例,中量反流2例;随访时中位左心室-主动脉压差4(2~49)mm Hg,较术前[37.2(12.1~70.6)mm Hg]有明显改善(Z=-5.153)。1年时心脏事件免除率为91%±5%,5年时为78%±8%。结论对于合并左心室流出道梗阻的完全性大动脉转位,需结合解剖情况与压差评估梗阻严重程度,指征把握恰当,行动脉调转术可获得满意的中远期效果。     

快速与长期双期大动脉调转术在室间隔完整大动脉转位中应用的对比研究

目的通过比较快速和长期双期大动脉调转术(ASO)的组间特点,总结发生左室退化后室间隔完整大动脉转位(TGA-IVS)的手术治疗经验。方法纳入2007年1月至2019年1月主要诊断为TGA-IVS并行双期ASO的患儿41例,其中男25例、女16例,平均年龄17.9个月(7 d至7岁)。根据双期手术间隔时间,分为快速双期ASO组(19例)和长期双期ASO组(22例)。比较两组患儿临床效果。结果两组患儿的年龄、体重、ASO术前血氧饱和度、训练术前左室舒张期末内径、ASO术前左室后壁厚度差异均有统计学意义(P<0.05)。患儿年龄>1岁是进行长期双期ASO的独立危险因素。结论长期双期ASO对年龄>1岁和左心室退化严重的患儿具有良好的疗效。     

完全性大动脉转位的麻醉处理

完全性大动脉转位(TGA)是比较常见的紫绀型先天性心脏病(简称先心病)之一,约占先心病的7%~9%,自然预后极差。大动脉转换术又叫大动脉调转术(switch术),已广泛应用于完全性大血管转位的纠治,取得较好的手术效果[1,2]。现将我院自1998年5月至2004年11月共8例患儿完全性大动脉转位     

动脉转位术治疗65例新生儿完全性大动脉错位

目的报告美国西雅图儿童医院１９８９年１０月～１９９２年１０月，为完全性大动脉错位新生儿作动脉转位术６５例，其中伴室间隔完整型４１例，伴室间隔缺损型２４例。方法３６例使用深低温停循环方法，２９例使用深低温低流量方法。结果手术死亡４例（６．１５％），死亡原因均与冠状动脉解剖变异有关。结论动脉转位术是矫正新生儿期室间隔完整型和伴室间隔缺损型完全性大动脉错位最好的手术方法。     

影响动脉转位术手术死亡的危险因素分析

目的探讨动脉转位术(arterial switch operation,ASO)手术死亡的危险因素,以提高手术成功率,降低手术死亡率。方法2001年1月1日至2007年12月31日期间在阜外心血管病医院行ASO208例,男157例,女51例;年龄6 h至17岁,中位年龄90 d;体重3～43 kg,中位体重5 kg。大动脉转位(TGA)/室间隔缺损(VSD)127例,TGA/室间隔完整(IVS)或合并VSD直径5 mm81例。冠状动脉分布正常(1LCx2R)151例,单冠状动脉畸形15例,壁内走行6例,冠状动脉分布反位36例。收集所有患者术前、术中和术后的临床资料,建立数据库,行单因素和logistic多因素分析影响ASO手术死亡的危险因素。结果围术期死亡24例(11.54%),死于术后感染合并多器官功能衰竭12例,低心排血量综合征10例,肺动脉高压危象1例,脑部并发症1例。其中2001～2005年手术死亡20例(18.30%),2006～2007年手术死亡4例(4.00%),后者手术死亡率较前者明显降低(P0.05)。单因素分析结果:死亡患者体外循环时间明显长于生存患者(236±93 min vs.198±50 min,P=0.002),冠状动脉分布异常(33.3%vs.6.5%,P=0.000)及术中发生冠状动脉事件(33.3%vs.2.2%,P=0.000)的比率明显高于生存患者;logistic回归多因素分析结果:手术年代早(OR=7.463,P=0.003)、冠状动脉分布异常(OR=6.303,P=0.005)及术中发生冠状动脉事件(OR=17.312,P=0.000)是ASO围术期死亡的主要危险因子。结论ASO有明显的学习曲线,手术年代早、冠状动脉分布异常、术中对冠状动脉处理不当是影响ASO死亡的主要危险因子。     

影响大动脉转位术死亡率的危险因素分析

目的分析大动脉转位术（arterial switch operation，ASO）治疗完全性大动脉错位（complete transposition of the great arteries，TGA）的手术结果，探讨影响死亡率的危险因素。方法收集2003年1月至2004年12月期间，我院对67例TGA患者施行ASO的临床资料，包括住院病历、超声心动图和手术记录。应用x^2检验和logistic多变量回归分析对患者的手术年龄、体重、诊断、冠状动脉分型、体外循环时间、主动脉阻断时间、停循环时间、术后呼吸机辅助时间、延迟关胸等因素进行统计分析，分析影响死亡率的危险因素。结果施行ASO的67例TGA患者中，围手术期死亡5例（7．5％）。单变量分析结果表明，影响ASO死亡率相关的危险因素有：年龄（P=0．004）、体重（P=0．042）、冠状动脉分型（P=0．006）和体外循环时间（P=0．048）。伴有室间隔缺损（ventricular septal defect，VSD）的患者（TGA／VSD）术后住心脏监护室（CICU）时间（P=0．004）和术后住院时间（P=0．007）明显长于室间隔完整（intact ventricular septum，IVS）的患者（TGA／IVS）。logistic多变量回归分析结果表明，患者手术时年龄（P=0．012）、冠状动脉畸形（P=0．001）和较长的体外循环时间（P=0．002）是影响ASO死亡率的危险因素。结论对TGA患者及时施行ASO可获得良好的临床效果，患者手术时年龄、冠状动脉畸形和较长的体外循环时间是影响死亡率的危险因素。     

心房内调转术治疗完全性大动脉错位

目的总结用心房内调转术治疗完全性大动脉错位(TGA)患者的经验。方法自2002年1月至2005年3月共对10例TGA患者行心房内调转术,男6例,女4例,年龄3个月~12岁。TGA伴室间隔缺损、肺动脉高压7例,伴房间隔缺损、室间隔完整2例,伴室间隔缺损、动脉导管未闭1例。采用Senn ing手术4例,M ustard手术6例。结果10例患者无1例死亡,脉搏血氧饱和度由手术前的0.65±0.05增至术后的0.95±0.03。紫绀消失,体质增强。随访4~28个月,复查心脏彩色超声心动图提示心房内转流正常,无梗阻现象,三尖瓣无明显反流。结论对TGA患者施行心房内调转术,可以取得较好的临床效果。     

大动脉调转术患儿术后监护

回顾分析34例行大动脉调转术惠儿死亡原因及并发症.结果术中死亡6例,术后死亡8例,死亡率41. 2%.术后死亡原因为低心排综合征(5例)、心律失常(3例).20例存活患儿术后并发症:出血(9例)、肺部感染/肺不张(8例)、心律失常(3例)、低心排综合征(2例)、肾功能不全(1例)、膈神经麻痹(1例).经对症治疗、护理,除1例膈神经麻痹外,均好转.提示大动脉调转术死亡率高,并发症种类多而且严重,护理人员在监护中除保持高度的警惕性外,应不断总结监护经验,以提高施护水平.     

完全性大动脉转位的外科治疗

对３３例完全性大动脉转位施行了手术治疗，其中全腔静脉肺动脉连接３例、改良Ｆｏｎｔａｎ手术２例、Ｍｕｓｔａｒｄ及大动脉调转术各１例，Ｒａｓｔｅｌｉ手术２６例，包括１５例心内隧道和心外管道，１１例心内、外双管道。这２６例心外管道中，除４例采用同种主动脉管道外，其余为经处理的猪肺动脉人工管道。全组手术死亡１０例，晚期死亡１例。结论：完全性大动脉转位病理解剖变异性大，应按不同类型的病理解剖特征选择手术方法     

姑息性大动脉调转手术的麻醉管理

目的 总结姑息性大动脉调转手术(palliative arterial switch operation,PASO)的麻醉管理. 方法 回顾性总结分析28例于我院行PASO的完全性大动脉转位合并室间隔缺损(transposition of the great arteries with ventricular septal defect,TGA/VSD)或Taussing-Bing综合征患者的临床资料.28例患者年龄中位数为4岁(1月～25岁),体重中位数为12.5 kg(3.6～43kg),术前均诊断为重度肺动脉高压,术前SpO2波动在44％～91％. 结果 所有患者麻醉过程平稳,平均CPB时间为(223±81) min,平均主动脉阻断时间为(153±32) min.平均室间隔补片留孔大小为(5.3±1.5) mm.术后机械辅助通气时间中位数为36 h(7～408 h),ICU停留时间中位数为5.5 d(2～27 d).术后平均SpO2为(96±2)％,与术前比较,差异有统计学意义(P＜0.05).住院死亡5例(18％),余好转出院.出院患者中5例在出院后的1～5年间行介入残余室缺分流堵闭术. 结论 充分的术前准备和评估,平顺的麻醉诱导和维持,围手术期肺动脉高压的处理,早期合理地应用血管活性药物以及出凝血功能的调整,有利于PASO的成功.     

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries

Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO). Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out. Patient population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population. Median time between repair and the coronary angiography was 2.9 ± 1.9 years. A comparison between the eight patients (6.7% out of the entire study population), known to have postoperative coronary obstructions (group I) and the rest of the cohort with angiographic normal coronary vessels (group II) was performed by univariate analysis of variance and logistic regression models. One patient had surgical plasty of the left coronary main stem with subsequent percutaneous angioplasty, three patients had primary coronary stent implantation and four patients had no further intervention at all. In group I, all but one patient denied symptoms of chest pain and echocardiography failed to show any difference between the two groups in terms of left ventricular systolic function (ejection fraction group I 61 ± 2% vs 62 ± 6% of group II, p = 1.0). Results: The association of coronary obstruction with complex native coronary anatomy (Yacoub type B to E) was evident at both univariate (62% of group I vs 22% of group II, p = 0.04) and logistic regression (p = 0.007, odds ratio (OR) 8.1) models. The type of coronary reimplantation (i.e., coronary buttons on punch vs trap-door techniques) was similar between the two groups (punch reimplantation in 25% of patients of group I vs 31% of group II, p = 0.1) as was the relative position of the great vessels (aorta anterior in 100% of patients of group I vs 96% of group II; univariate, p = 0.1). Conclusions: The late outcome in terms of survival and functional status after ASO is excellent. Nevertheless, the risk of a clinically silent late coronary artery obstruction of the reimplanted coronary arteries warrants a prolonged follow-up protocol involving invasive angiographic assessment.     

Early and long term outcome of the arterial switch operation for transposition of the great arteries: predictors and functional evaluation

Objectives: The aims of this report were to study the early and late outcome in terms of mortality, freedom from reoperation, predictors for late pulmonary stenosis (PAS) and insufficiency of the neo-aortic valve (AVI) in patients with transposition of the great arteries (TGA) undergoing arterial switch operation (ASO). Materials and methods: Between January 1990 and December 2001, 134 patients with TGA underwent ASO. The patients were divided in Group I (n=88)-TGA with intact ventricular septum and Group II (n=46)-TGA with ventricular septal defect (VSD). The pulmonary artery was reconstructed employing the direct anastomosis technique (PT-I) in 21 (15.7%) patients, the double-patch technique (PT-II) in 41 (30.6%), single pantaloon patch (partial circumference) (PT-III) in 46 (34%) and single pantaloon patch (total circumference) (PT-IV) in 35 (26%) patients. The mean follow-up was 3.4±1.3 years. Results: The hospital mortality was 17 (12.7%) patients. The mortality in Group I was significantly lower than Group II (P=0.002). The overall actuarial survival at 1, 3 and 5 years follow-up resulted to be 98, 93, and 91.5%, resulting to be significantly higher in Group I (P=0.032). The multivariate analysis revealed the complex TGA (P=0.007), VSD (P=0.032), coronary anomalies (P=0.004), aortic coarctation or hypoplastic aortic arch (P=0.021), left ventricular outflow tract obstruction (LVOTO) or moderate PAS (P=0.041) as strong predictors for poor free-reoperation cumulative survival. A strong inverse correlation was found between the mean trans-pulmonary gradient at follow-up and the age at the operation (r=−0.41, P<0.0001). The univariate analysis revealed the PT-I technique (P=0.002), prior moderate PAS (P=0.0001), and age <1 month (P=0.018) as strong predictors for moderate-to-severe PAS. The neo-AVI incidence was significantly higher in Group II (P=0.011). Predictors for neo-AVI were male sex (P=0.003), preoperative neo-AV Z-score >1 (P<0.001), prior or concomitant operation for aortic coarctation or hypoplastic aortic arch (P=0.001), LV retraining (P=0.003). Conclusion: ASO remains the procedure of choice for the treatment of various forms of TGA with acceptable early and later outcome in terms of overall survival and free reoperation. Strong predictors for poor overall free-reoperation survival are complex TGA, VSD, coronary anomalies, aortic coarctation and LVOTO or moderate PAS. The pulmonary artery reconstruction using a single ‘pantaloon patch’ seems to offer less residual stenosis. Patients with a VSD and a significant mismatch between the neo-aortic root and distal aorta are at a higher risk for developing postoperative neo-AVI.     

Modification of the arterial switch operation for transposition of the great arteries with complex coronary artery patterns

The arterial switch operation has evolved into the treatment of choice for most forms of transposition of the great arteries (TGA). Recently reported operative mortality of the procedure has fallen to the range of 1.1%–6.0%, even for complex forms of TGA. Despite advancement in the technical aspects of the procedure, certain anatomical variations of the coronary arteries, such as a single coronary orifice and/or intramural coronary artery, are still considered surgical risks in many centers. Optimizing the surgical technique for relocating these challenging variations of the coronary anatomy is key to improving the surgical outcomes for the procedure. In this review, the surgical modifications of the arterial switch operation for TGA associated with complex patterns of the coronary arteries are examined. This review was submitted at the invitation of the editorial committee.     

Results of a new technique of arterial switch operation for transposition of great arteries with single coronary artery

Background Transposition of great arteries (TGA) with Single Coronary artery is one of the high risk groups for Arterial Switch Operation (ASO). To eliminate this risk, we have innovated a new technique of ASO with insitu coronary re-allocation. We present our experience with this new technique. Methods From September 1998 to October 2005, ten consecutive cases of TGA with single coronary artery were operated employing this new technique. Their age ranged from 8 days to 9 months; their weight ranged from 2.6 to 5.8 Kgs. ASO was done by transecting the great arteries just above the commissures. For coronary re-allocation, hockey stick shaped incisions were made in the facing sinuses of the proximal aorta and the pulmonary artery. These flaps were sutured in such a way that the coronary ostium was committed to the neo-aorta. Results There was no in-hospital mortality. The follow-up ranged from 4 months to 7 years. All patients had follow-up echocardiogram at regular intervals, which showed no significant right or left ventricular outflow obstruction, no regional wall motion abnormalities and no, neo-aortic or neo-pulmonary regurgitation. Conclusion This new coronary re-allocation technique avoids problems related to coronary translocation such as traction and kinking. It spares the need for dissection of proximal coronary artery and its branches, and thereby eliminates the risk of development of fibrosis and stenosis. The same technique can be used regardless of the sinus of origin of the coronary artery. It is a reliable and reproducible technique. The early results were excellent.     

Midterm follow-up of arterial switch operation for transposition of the great arteries with intact ventricular septum and left-ventricular outflow tract obstruction

Objective: We report the mid-term follow-up of patients, who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) with intact ventricular septum and left-ventricular outflow tract obstruction (LVOTO) over a 10-year period from 2000 to 2009. Methods: Thirteen TGA patients (3.9% of our ASO cohort) with intact ventricular septum and LVOTO underwent ASO. LVOTO was defined as pulmonary valve z-score ≤ −2.0 (n = 3) or peak LVOT gradient ≥40 mmHg with (n = 7) or without (n = 3) anatomic subvalvar stenosis on echocardiography. Median age and weight were 14 days (range, 7–130 days) and 3.2 kg (range, 2.1–4.6 kg). The LVOT abnormalities included fibromuscular narrowing (n = 5) and atrioventricular valve-related findings (n = 5). LVOT clearance was achieved by resection of accessory mitral tissue (n = 2) only. Results: Follow-up was 100% complete. There were no early or late deaths. Freedom from re-operation for neo-aortic valve regurgitation and/or LVOTO was 100% at a median follow-up of 38 months (range, 6–115 months). All patients had functional status appropriate for their age. Three patients had mild aortic regurgitation. The median Doppler estimated LVOT systolic gradient was 12 mmHg (range, 0–18 mmHg) for the entire cohort at the latest follow-up. Conclusions: Mid-term outcomes of ASO for a highly selected group of patients with pulmonary valve annulus z-score ≤ −2.0 ≥ −0.4, resectable organic LVOTO, and dynamic peak LVOT gradient ≥40 mmHg remain satisfactory, with a need for long-term follow-up.     

快速二期动脉转位术纠治新生儿完全型大动脉转位

目的总结快速二期动脉转位术的临床应用效果.方法 2002年9月至2003年5月,5例完全型大动脉转位患者行快速二期动脉转位术.手术平均年龄83.0±72.2 天,平均体重4.7±0.9 kg.由于求诊时已经超过最佳手术年龄,左心室退化,不能承受体循环压力,则先行肺动脉环缩和体肺动脉分流术,术后6～9天行第二期动脉转位术.结果一期手术中,1例术后32小时发生室上性心动过速,少尿,经腹膜透析和药物处理无效死亡;其余4例分别在术后6～9天行二期动脉转位术,无死亡.术后随访2～10个月,生长发育良好,超声心动图检查显示心内无残余分流,主动脉和肺动脉干吻合口通畅,左心室射血分数(EF)0.68～0.77,短轴缩短率(FS)0.24～0.37,1例主动脉瓣轻度反流.结论对失去最佳手术时间的新生儿完全型大动脉转位,行快速二期动脉转位术是最佳的选择.     

169例动脉调转手术治疗大动脉转位的早、中期结果

目的 探讨大动脉调转手术(ASO)治疗完全性大动脉转位(TGA)的早、中期结果,分析其随访死亡、术后主动脉瓣反流及肺动脉狭窄的危险因素.方法 2004年1月至2007年12月,169例行动脉调转术病儿入选,其中男129例,女40例,平均年龄(11.7±26.3)个月.病儿分两组:Ⅰ组为室间隔完整组(56例),Ⅱ组为室间隔缺损组(113例).所有术后生存病儿均进行超声随访,平均随访时间(27.7±14.6)个月.危险因素采用Logistic回归模型分析.结果 全组住院死亡19例(11.24%),两组间差异无统计学意义.随着整体治疗水平的提高,住院病死率由2004年的16.67%下降到2007年的3.92%.1、3及5年生存率分别为94.00%、91.33%及91.33%,两组间差异无统计学意义.Logistic回归分析发现,ASO术后随访死亡的危险因素为手术年龄大于6个月;术后主动脉瓣反流的危险因素为合并室间隔缺损、年龄大于6个月、术后新主动脉瓣Z值>1;术后肺动脉狭窄的危险因素为手术年龄小于1个月和肺动脉采用补片成形.结论 ASO手术具有良好的早、中期结果,是治疗完全性大动脉转位的理想术式.TGA病儿应该尽早手术治疗,手术年龄大于6个月是随访死亡及主动脉瓣反流的重要危险因素;新主动脉根部与主动脉远端不匹配是术后主动脉瓣反流的危险因素;病儿的生长发育与肺动脉成形材料无生长特性的矛盾是导致术后肺动脉狭窄的危险因素.     

大动脉转换术113例的手术结果分析

目的回顾性分析大动脉转换术的手术疗效。方法2001年1月至2005年12月,采用大动脉转换术纠治完全性大动脉错位113例,其中室间隔完整型大动脉错位（TGA／IVS）60例,伴室间隔缺损大动脉错位（TGA／VSD）53例。患儿体重最轻2,3kg,年龄最小出生后6h。在深低温停循环和低流量下行大动脉转换术。结果手术总死亡率9．7％,其中TGA／IVS死亡5例,手术死亡率8．3％,TGA／VSD死亡6例,手术死亡率11.3％。随着手术方法的不断改进和围手术期以及体外循环转流技术的提高,其手术死亡率不断下降,从早期的手术死亡率16.6％降至目前的5．6％。结论冠状动脉畸形是导致大动脉转换术死亡的关键,TGA／VSD的冠状动脉畸形发生率较TGA／IVS要高。主动脉和肺动脉的位置并不影响手术成功率。左心室与右心室压力比例〈0．6时,不能行大动脉转换术,否则术后将出现严重左心室低心排。