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。     

动脉转位术的临床应用

目的 总结动脉转位术(arterial switch operation，ASO)治疗完全型大动脉转位(transposition of the great arteries，TGA)和右心室双出口伴肺动脉瓣下室间隔缺损(VSD)的临床经验。方法 采用ASO治疗小儿先天性心脏病32例，其中TGA22例，伴室间隔完整型(intact ventricular septum，IVS)9例，伴VSDl3例；右心室双出口伴肺动脉瓣下VSD(Taussig—Bing)10例。结果TGA／IVS9例中死亡1例，TGA／VSD13例中死亡4例，Taussig-Bing10例死亡3例，总手术死亡率25％(8／32)。术后随访3个月～2年，所有患者紫绀消失，活动能力明显增强。1例Taussig—Bing术前二尖瓣轻-中度反流，术后仍为中度反流；2例TGA主动脉和肺动脉瓣上狭窄，压差40mmHg(1kPa=7．5mmHg)，1例肺动脉瓣下狭窄和残余VSD，3个月后再次手术治愈。结论 ASO已广泛应用于TGA的纠治，手术效果满意；应用于右心室双出口肺动脉瓣下VSD的早期纠治，不但可防止发生肺血管阻塞性病变，而且避免了心内修补左心室流出道梗阻的远期并发症。     

利用“多余”冠状动脉纽片作主动脉根部成形在大动脉转位术中的应用

目的总结大动脉转位术(ASO)中利用"多余"的冠状动脉纽片作新主动脉根部成形的临床经验,探讨保持主动脉瓣窦的解剖形态对改善术后冠状动脉血流的临床意义。方法选取2003年1月至2009年6月,我院收治的室间隔缺损型大血管错位和Taussig-Bing畸形患者110例,手术年龄为出生2d～2岁,平均年龄91.1d;体重1.79～9.50kg,平均体重4.70kg。根据不同的外科处理技术,将患者分为两组,A组:78例,术中采用多余的冠状动脉纽片作新的主动脉根部成形,减小新主动脉根部近心端开口的直径;B组:32例,术中剪除多余的冠状动脉纽片,主动脉吻合口近心端与远心端直径不匹配。采用logistic逐步回归筛选结果中影响住院死亡的危险因素。结果术后早期死亡12例,总病死率为10.9%(12/110),A组病死率明显低于B组[6.4%(5/78)vs.21.9%(7/32),P=0.019]。随访72例,随访时间为术后3个月～5年。后期死亡3例,A组1例,B组2例;5例患者再次手术。单因素logistic回归分析结果显示:影响术后早期死亡的危险因子包括Taussig-Bing畸形(χ2=4.011,P=0.046)、合并主动脉弓病变(χ2=4.437,P=0.036)、单支冠状动脉(χ2=5.071,P=0.025)和B组患者(χ2=5.584,P=0.019)。多因素logistic回归分析结果显示:合并主动脉弓病变(χ2=5.681,P=0.010)和B组患者(χ2=3.987,P=0.047)是构成影响术后早期死亡的独立危险因子。结论利用"多余"的冠状动脉纽片作新的主动脉根部成形能够较好地保持新主动脉瓣窦形态,而主动脉根部的特殊解剖形态与冠状动脉灌注有重要的关系,手术病死率降低可能与术后冠状动脉灌注得到改善有关。     

单中心11年大动脉调转术早期死亡及远期再手术分析

目的通过对大动脉调转术(ASO)患儿术后早期死亡和远期再手术分析, 探讨ASO的手术风险和远期再手术原因。方法回顾性分析2010年1月至2020年12月在上海儿童医学中心接受ASO手术治疗的患儿的临床资料及在该时间段内的随访资料, 分为室间隔完整型的大动脉转位(TGA/IVS)、大动脉转位合并室间隔缺损(TGA/VSD)、Taussig-Bing畸形(TBA)及二期ASO(Ⅱ-ASO)4组。采用χ2检验分析不同组别ASO术后的早期死亡比例、远期再手术率。结果本研究共纳入861例ASO手术患儿, 术后早期死亡108例(12.5%)。753例术后随访, 失访102例(13.5%, 102/753)。651例完成随访, 男352例, 女299例, 中位随访7.23(4.74, 9.37)年。66例(10.1%, 66/651)远期再手术治疗, 4例(6%, 4/66)再手术死亡。TGA/IVS 241例, 再手术24例(10%);TGA/VSD 256例, 再手术23例(9%);TBA 126例, 再手术18例(14.3%);Ⅱ-ASO 28例, 再手术1例(3.6%)。再手术原因包括:肺动...     

动脉调转术治疗心室大动脉连接异常的先天性心脏病

目的总结动脉调转术（ASO）治疗心室大动脉连接异常的先天性心脏病（先心病）手术疗效。方法2000年1月至2004年8月，60例病儿实施ASO，早年（2000．1—2003．5）42例，近期（2003．6—2004．8）18例；其中完全性大动脉转位（TGA）49例、Taussig-Bing畸形7例、矫正性大动脉转位（ccTGA）4例；年龄1—6个月15例、7～12个月14例、1-3岁6例、〉3岁6例，其中〉6月龄的TGA/VSD或TGA/PDA18例。行大动脉调转术，同期矫治合并畸形；ccTGA病儿先行心房转流术，后行ASO。结果全组手术死亡10例（16．7％），其中早年9例（21．4％）、近期1例（5．6％），死亡率明显下降（P〈0．05）。生存病儿随访0．5—56．0个月，心功能恢复良好，无死亡及并发症。结论ASO应用于TGA、Taussig-Bing畸形以及ccTGA能取得良好的手术结果。针对国内TGA/VSD或TGA/PDA病儿就诊较晚、年龄较大，肺动脉压力较高等特点，如心导管检查显示肺小动脉阻力不高，ASO仍可获得满意的疗效。     

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.     

动脉调转术不同年龄手术效果的对比——附142例错过最佳手术年龄者治疗经验

目的 探讨大动脉转位(TGA)病儿接受动脉调转手术(ASO)时年龄对手术效果的影响.方法 2000年5月至2008年9月,264例rIGA病儿行ASO.男194例,女70例;年龄·l d一19岁.其中室隔完整型TCA('rcAJrvs)84例;年龄≤2周28例,>2周56例.伴室隔缺损TCA(TCAJVSO)130例,Tams-ing-B~畸形(TBA)50例,其中≤6月94例,>6月86例.符合最佳手术年龄(≤2周TCA/IVS,≤6个月TGA/IVSD、TBA)者122例,错过最佳手术年龄者(>2周TCA/IVS,>6个月TGA/VSD、TBA)142例.手术方法:18例>2周TGA/IVS先期行左室训练,4例>6个月TGA/VSD先期行肺动脉环缩,余者均一期行ASO,同期矫治合并畸形.结果 全组手术死亡26例,病死率9.85%.平均随访(29.5±7.6)个月,2例分别于术后2个月和1.5年猝死,l例术后1.5年因肺动脉吻合口狭窄再次手术,余者心功能明显改善.无死亡和远期并发症发生.符合最佳手术年龄者病死率13.1l%;错过最佳手术年龄者病死率7.04%.2006年后(共154例),TGA病儿病死率由19.09%降至3.25%(P<0.05),符合最佳手术年龄者由22.64%降至5.80%(P<0.05),错过最佳手术年龄者由15.79%降至1.18%(P<0.05).结论 ASO应用于错过最佳手术年龄TCG者亦可取得满意效果.     

73例大动脉调转术非典型冠状动脉解剖类型分析

目的 分析73例大动脉调转术非典型冠状动脉解剖类型,从临床角度探讨其分类和描述.方法 2001年1月至2008年12月,采用大动脉调转术治疗完全性大动脉转位伴室间隔缺损型(TGA/VSD)38例,完全性大动脉转位伴室间隔完整型(TGA/IVS)35例.根据术中所见冠状动脉解剖,采用Leiden分类标准进行分类,图例参考Marie-Lannelongue医院绘制图例制作.结果 非典型冠状动脉17例,发生率约23%,其中TGA/VSD 10例,TGA/IVS 7例.根据Leiden分类标准可分9类,但有12种描述,7例Leiden标准尚不能准确的分类或全面的描述.结论 TGA 病儿非典型冠状动脉的发生率较高,类型多样,其中有较多的解剖类型Leiden标准尚不能准确描述,为便于临床手术操作,对于非典型冠状动脉的解剖最好足Leiden分类结合补充描述的方法.     

影响动脉调转术后新主动脉瓣反流的危险因素分析

目的 旨在探讨完全性大动脉转位(transposition of the great arteries,TGA)患者动脉调转术(arterial switch operation,ASO)后出现新主动脉瓣反流(neoaortic regurgitation,NAR)的危险因素。方法 回顾性分析2008~2013年1月阜外医院行ASO 229例TGA患者的临床资料,其中男173例、女56例,年龄3 d~93.9(7.8±15.9)个月(中位年龄47 d),体质量4~18(6.3±4.2)kg(中位体质量2.4 kg)。结果 平均随访(62.5±31.1)个月(最短随访36个月),ASO术后有28例(12.2%)患者出现了中重度的NAR。Kaplan-Meier生存曲线显示ASO术后1年、2年、3年、5年免除中重度NAR的概率分别为100.0%、100.0%、99.6%及95.3%。单因素分析结果显示出现中重度NAR的患者ASO时的体质量、术前伴有肺动脉高压的比例和前期肺动脉环缩术的比例均明显大于无或微少量NAR的患者[(8.3±5.6)kg vs.(5.8±4.3)kg,P=0.006;50.0%vs.20.4%,P=0.001;28.6%vs.10.4%,P=0.013)]。多因素分析结果显示前期肺动脉环缩术(HR=3.8,P=0.005)以及术前伴有肺动脉高压(HR=16.5,P0.001)是术后中重度NAR的危险因素。结论 ASO术后的NAR发生率较为满意,术前伴有肺动脉高压以及前期肺动脉环缩术和NAR有关。     

双活瓣延长技术在复杂动脉转位术中的应用

目的总结在动脉转位术(arterial switch operation,ASO)中采用双活瓣延长技术做冠状动脉移植治疗完全型大动脉转位(D-transposition of the great arteries,D-TGA)和Taussig-Bing畸形的临床经验,探讨该技术的手术方法和手术适应证。方法回顾性分析2006年1月至2011年6月上海交通大学医学院附属上海儿童医学中心21例合并复杂冠状动脉畸形的D-TGA、L-TGA或Taussig-Bing患者[男13例,女8例;年龄(110.0±84.5)d;体重(5.4±4.2)kg]行ASO治疗,术中采用双活瓣延长技术做冠状动脉移植的临床资料。所有患者均为右冠状动脉主干或粗大右心室圆锥支发自主动脉左侧或右侧瓣窦,并异常前绕于主动脉根部行走。双活瓣延长技术包括:从主动脉剪取较长的冠状动脉纽片做为活瓣;剪取肺动脉(新主动脉)另一带蒂活瓣向冠状动脉纽片方向做等距离延长;活瓣与纽片的边缘互相缝合形成冠状动脉的延长管道。结果无住院死亡,术后呼吸机使用时间(101.6±53.6)h,监护时间(9.5±4.9)d。术后发生低心排血量9例,肺动脉高压危象2例,肺部感染6例,急性肾功能衰竭2例,均经相应的治疗治愈出院。11例患者术后延迟关胸。随访17例,随访时间2个月～5年,随访期间患者生长发育均明显改善,无缺血性心电图改变。1例肺动脉瓣上狭窄患者于ASO术后2年再次行手术修补。结论双活瓣延长技术做冠状动脉移植在复杂ASO中能有效减少因冠状动脉畸形导致的手术死亡,尤其适合于二期ASO以及右冠状动脉主干或粗大圆锥支发自左侧或右侧瓣窦,并沿主动脉异常前绕的患者。     

Arterial switch operation for transposition of the great arteries without use of prosthetic material]

For transposition of the great arteries (TGA), arterial switch operation (ASO) is theoretically preferable to atrial switch operation, since the left ventricle is established as the systemic ventricle and sinus node function is maintained. However, ASO is a delicate operation requiring the transfer of the coronary arteries. Use of prosthetic material for reconstruction of the neo-pulmonary artery causes postoperative supra-valvular pulmonary arterial stenosis. Five neonates and young infants with TGA underwent ASO without the use of prosthetic material by the technique reported by Pacifico et al. Three patients with simple TGA ranged in age from 10 to 27 days, and in weight from 2.9 to 3.9 kg. Two patients with TGA and ventricular septal defect ranged in age from 41 to 63 days, and in weight from 2.8 to 4.2 kg. There were no deaths, either early or late, and all patients are well 7 to 21 months after surgery. Catheterization and Doppler echocardiographic studies performed in all patients showed that pressure gradient between the right ventricle and pulmonary artery decreased significantly during mean follow-up period of 14 months. These results suggest excellent growth of the pulmonary artery after surgery.     

Mid-term results of open aortic valvotomy for infants with critical aortic stenosis: seven-year experience including delayed Ross strategy.

OBJECTIVES: The purpose of this study is to provide short- and mid-term results of open aortic valvotomy (OAV) for patients with critical aortic stenosis (AS). METHODS: Between December 1993 and June 1996, 6 patients with critical AS underwent an OAV in our unit. Their ages and body weights at operation ranged from 1 to 65 days (median age, 9 days) and from 2.4 to 5.7 kg (median weight, 3.3 kg), respectively. Peak pressure gradient and diameter of the aortic valve ranged from 25 to 111 mmHg (mean value, 79 mmHg) and from 4.6 to 7.5 mm (mean diameter, 6.1 mm), respectively. OAV comprised the valvular commissurotomy and excision of the myxomatous nodules with cardiopulmonary bypass. RESULTS: No early or late death occurred. Mean peak pressure gradient across the aortic valve was reduced to 33 mmHg (from 15 to 44 mmHg) with no aortic insufficiency in 2 patients and trivial insufficiency in 4. During the follow-up period of 6 to 9 years, 3 out of 6 patients required no reintervention. The other 3 patients required repeated valvotomy for recurrent stenosis within 0.2 to 1.3 years after the operation. Of these, 2 patients required the Ross procedure at 7 years of age or older, and another at 6 years of age awaits the Ross procedure. CONCLUSION: OAV for critical AS was effective without causing mortality or significant aortic insufficiency. Our current strategy comprising the initial OAV and "delayed Ross procedure" for recurrent stenosis with or without insufficiency is a promising therapeutic option for infants with critical AS.     

快速二期大动脉转位术早期死亡危险因素

目的 探讨影响快速二期大动脉转位术早期死亡的危险因素.方法 回顾性研究2002年9月至2007年9月期间,21例快速二期大动脉转位术病婴临床资料.采用Logistic多元线形回归分析模型对病婴的人口统计学资料,术前诊断资料,左心功能锻炼期资料,以及二期大动脉转位术术中和术后数据进行统计分析,探讨影响快速二期大动脉转位术的危险因素.结果 该手术初期死亡率较高,呈逐年下降趋势.与左心功能锻炼术后死亡相关的为B-T分流自径(P=0.003);与快速二期大动脉转位术后死亡相关的为女性(P=0.006)和术前pLV/RV(P＜0.001).结论 快速二期大动脉转位术是目前治疗错过最佳手术时机的室隔完整型D-TGA病婴的最佳手术方式;使已退化的左心功能得到足够锻炼,是决定二期大动脉转位术成功的关键所在.

Abstract：

Objective To investigate the risk factors associated with early mortality of the rapid two-stage arerial switch operation, which has a significantly higher overall mortality than that of ASO procedure for D-TGA with a intact ventricular septun. Methods The data we reviewed involving patients who underwent rapid two-stage switch operations from September,2002 to September, 2007 in our center, 13 patients were male and 8 were female, their age at operation ranged from 29 to 250 days afer birth, and the body weight was 3.5 to 7 kg. Chi-squared test and multivariant logistic regression methods were used for the analysis of demographic data, pre-diagnosis information, operation data, interval data combined with the operation time,left ventricle training condition, and the early post-operative outcomes. Results The operative mortality was high at initial stage, and then decreased gradually. The logistic multivariant regression analysis indicated that the mortality of left ventricular training operation was associated with the diameters of BT shunt ( P =0.003 ); the mortality of two-stage switch was associated with feminie ( P = 0.006 ) and pre-operative p. LV/RV ( P ＜ 0.001 ). Conclusion Patients with transposition of the great arteries and intact ventricular septum who missed the optimal time for switch operation should receive rapid two-stage switch operations, which provide an opportunity for the correction of the deformity. The key factor associated with the success for operation was good in heart function after left ventricular training.     

Long-term result of arterial switch operation for corrected transposition of the great arteries or double inlet left ventricle

The purpose of this study is to analyze the operative maneuver and long term outcome of the arterial switch operation (ASO) for congenitally corrected transposition of the great arteries (c-TGA) or double inlet left ventricle (DILV). Since October 1977, 221 patients had undergone ASO in National Cardiovascular Center, Japan. Of these, 8 patients underwent ASO as a part of double switch operation (DSO) for c-TGA, and 1 patient underwent ASO and ventricular septation for the DILV with a rudimentary right ventricle simultaneously. We retrospectively reviewed these 9 patients. Six patients had a past history of the pulmonary artery banding. Age at the time of ASO ranged from 6 months to 5 years (median 3 year). As a reconstruction of the pulmonary artery at the time of ASO, Lecompte maneuver was performed in 7 patients, and original Jatene procedure was performed in 2. Coronary transfer was done as usual in all patients. There was no early death, and 1 patient died 1 year after the operation due to chronic heart failure. Late complication related to the ASO was pulmonary artery stenosis (1 patient after DSO) and aortic regurgitation (1 patient after ventricular septation).     

室间隔完整的超龄大动脉转位病儿最佳手术方式的选择

目的 探讨超龄室间隔完整的大动脉转位(TGA-IVS)病儿手术方式的选择和治疗的早、中期结果.方法 2000年3月至2007年6月,收治年龄超过3周的TGA-IVS病儿36例,占同期TGA行动脉调转术(ASO)病儿的23.9%.男26例,女10例;年龄22～2190 d,其中小于3个月20例,大于1岁3例;体重3.5-19.0 kg,平均(5.4±2.9)kg.依据手术方式分为一期手术组(A组)21例和分期手术组(B组)15例.B组先进行左心室功能锻炼,二期再进行ASO,其中快速二期手术14例,长期二期手术1例.两组的平均年龄和术前左、右心室压力比值(LVP/RVP)差异有统计学意义.32例随访2-74个月,平均(20.3±19.1)个月.结果 围术期死亡2例,分别死于肺部感染和肾功能衰竭,巨细胞病毒感染引起的肝肾功能衰竭.术后3-6个月死亡3例,3年生存率为88.8%.生存病儿生活质量和生长发育良好,左心室收缩功能正常.结论 错过手术最佳时机的TGA-IVS病儿,参考术前超声和术中测压情况合理选择一期或分期ASO,并加强术后管理,治疗效果满意.     

Recurrent obstruction after subclavian flap repair of coarctation of the aorta in infants. Can it be predicted or prevented?

Recoarctation is a problem in some patients after subclavian flap aortoplasty. To investigate the reason for recoarctation, we reviewed the records of 26 infants who underwent subclavian flap repair for symptomatic coarctation of the aorta at less than 3 months of age between June, 1979, and December, 1983. Age at repair ranged from 2 to 65 days (median 16 days) and weight from 2.1 to 4.9 kg (median 3.4 kg). In 14 patients the coarctation was associated with significant intracardiac defects (complex in six). There were two intraoperative deaths and one early death (surgical mortality 12%). The survivors were followed from 6 weeks to 66 months (median 12 months). Five survivors (22%), all operated on at less than 14 days of age, developed severe recoarctation 6 weeks to 6 months (median 5 months) after repair. The obstruction appeared to be due to lumen obliteration by shelf-life posterior wall tissue. Morphometric analysis of preoperative angiograms showed no correlation between recoarctation and distance between the left subclavian artery and the site of coarctation, length of the isthmus, diameter of the isthmus, combined cross-sectional area of the left subclavian artery and isthmus, or the ratio of the combined cross-sectional area of the left subclavian artery and isthmus to the cross-sectional area of the descending thoracic aorta. Recoarctation did not correlate with weight at operation, but it correlated significantly with age at aortoplasty (p = 0.02). The results suggest that intrinsic abnormalities of the periductal aortic wall are responsible for recoarctation after subclavian flap aortoplasty. Particular attention to this abnormal tissue at repair may prevent early recurrence in young infants.     

Short-term outcome of extracardiac lateral tunnel using the atrial free wall and autologous pericardium

BACKGROUND: Several modifications to the original Fontan procedure have been proposed in order to decrease postoperative morbidity. Lateral tunnel and extracardiac total cavo-pulmonary connection are 2 such modifications. PATIENTS: Between August 2005 and December 2005, the extracardiac lateral tunnel procedure was performed in 5 patients. The age at operation ranged from 19 to 59 months (median 24 months) and the weight ranged from 9.2 to 16.1 kg (median 11.4 kg). RESULTS: There was no mortality. The mean operation time was 466 +/-118 minutes. The mean cardiopulmonary bypass time was 198 +/- 61 minutes. The mean durations of intubation, intensive care unit stay, drainage tube use, and hospital stay were 1 +/- 1, 7 +/- 3, 12 +/- 5 and 30 +/- 2 days, respectively. Postoperative catheterization findings demonstrated that the mean superior venous caval pressure, inferior venous caval pressure, ventricular volume and ventricular ejection fraction were 10.0 +/- 1.4 mmHg, 11.0 +/- 2.4 mmHg, 140 +/- 47% of normal and 58.0 +/- 6.8% , respectively. CONCLUSIONS: The short-term results of the extracardiac lateral tunnel compared favorably with the results of different types of Fontan operation. In addition this procedure has the potential for growth and anticoagulation therapy is unnecessary.     

Surgical management of aortopulmonary window.

OBJECTIVE: Aortopulmonary window is a rare congenital malformation involving a window-like communication between the ascending aorta and the pulmonary artery. Here, we present our experience regarding the surgical repair of an aortopulmonary window, and also assess the long-term outcome. METHODS: Thirteen children with an aortopulmonary window associated with various congenital lesions underwent a repair of the defect. The age at operation ranged from 3 days to 1 year (median age, 19 days). The patient's weight ranged from 2.1 to 7.0 kg (mean weight, 3.6 kg). The associated lesions included an interrupted aortic arch (5 patients), a ventricular septal defect (2), an atrial septal defect (1), mitral valve regurgitation (1), and tricuspid atresia [Ic] with mitral valve regurgitation (1). The aortopulmonary window was repaired with a cardiopulmonary bypass in 11 patients, and 2 patients were ligated without a cardiopulmonary bypass. RESULTS: One patient associated with tricuspid atresia died (mortality rate of 7.7%). There has been no late death during a mean follow-up of 7 years and 3 months. CONCLUSIONS: The surgical results for an aortopulmonary window are encouraging, even if such patients are associated with major cardiac anomalies and an interrupted aortic arch. Most have shown a good long-term outcome.