冠状动脉畸形的外科治疗

目的 总结先天性冠状动脉畸形的手术治疗经验。方法 11例冠状动脉畸形患者中冠状动脉窦8例, 冠状动脉起源异常3例,合并法洛四联症1例,风湿性心瓣膜病2例。右冠状动脉瘘均在心肺转流术下经心腔修补内瘘口或经扩张的冠状动脉修补外瘘口或同时修补内,外瘘口;左冠状动脉－肺动脉瘘支动脉结扎术或在心肺转流术心脏不停跳下经肺动脉修补;左冠状动脉－左心室瘘伴主动脉瓣关闭不全经左冠状动脉修补外瘘口及主动脉瓣成形术。左冠状动脉起源于肺动脉畸形行左冠状动脉根部结扎或冠状动脉旁路移植术。合并的心脏疾病的同时纠治。结果 本组无手术死亡,术后心脏杂音均消失,10例术后症状缓解。结论 先天性冠状动脉畸形一旦确诊,主张手术治疗。     

一期纠治主肺动脉窗或右肺动脉起源于升主动脉

目的总结主肺动脉窗或右肺动脉起源于升主动脉的外科纠治经验。方法17例病儿中单纯主肺动脉窗5例,右肺动脉起源于升主动脉4例,主肺动脉窗伴主动脉弓中断、法洛四联症、房室间隔缺损、二尖瓣反流和气管狭窄等8例。1例无手术指征,16例行解剖纠治,平均手术年龄1．7岁;平均体重8．5 kg。结果手术无死亡。2例延迟关胸;1例再进胸止血。随访无死亡,无主动脉瓣上狭窄及肺动脉狭窄。结论主肺动脉窗及右肺动脉起源于升主动脉易早期产生肺动脉高压,一经诊断需立即手术。手术方式首选与伴发畸形一期纠治。主肺动脉窗及右肺动脉起源于升主动脉尽管多有伴发畸形,但早期手术纠治可获得较佳中、长期疗效。     

冠状动脉畸形对复杂先心病右室流出道重建的影响

目的探讨冠状动脉畸形对复杂先心病右室流出道（RVOT）重建的影响。方法自1989年4月至2004年5月治疗18例合并冠状动脉畸形的法洛四联症（14例）和右室双出口（4例）患者。右冠状动脉开口缺如7例,左冠状动脉开口缺如4例,左前降支起自右冠状动脉6例,左右冠状动脉交通支形成蔓状血管丛1例。均在全身麻醉低温体外循环下手术,1例行姑息手术,17例行根治术,RVOT重建的方法：游离畸形冠状动脉从其下方做切口,RVOT心包补片扩大术4例;3例在异常交通支下方平行切口修补室间隔缺损（VSD）,交通支上方纵切口加宽RVOT和肺动脉瓣环;4例行右心房切口修补VSD,RVOT小切口和肺动脉切口心包补片扩大术;3例肺动脉主干与RVOT直接吻合;3例同种带瓣主动脉重建RVOT。结果术后早期死于顽固性低心排血量综合征1例,无严重出血并发症和呼吸道并发症,姑息手术1例,血氧饱和度由术前的68．0％升至82.0％。16例随访10个月～8年,1例术后1年不明原因猝死;超声心动图示轻度残余漏3例,残余梗阻3例。结论复杂先天性心病并冠状动脉畸形RVOT重建时,应根据冠状动脉畸形特点选择手术方式。     

左冠状动脉异常起源于肺动脉的临床治疗

目的总结左冠状动脉异常起源于肺动脉的手术治疗经验。方法回顾性研究总结近年9例左冠状动脉异常起源于肺动脉的患者,对4例于肺动脉起始部结扎左冠状动脉,5例于体外循环下行肺动脉内隧道成形术(Takeuchi术),合并的心脏畸形同时予以纠治。结果全组9例术后无心肌缺血或梗死、残余分流和晚期死亡,心功能恢复到I级。结论左冠状动脉异常起源于肺动脉畸形一旦确诊,主张手术治疗。     

合并异常冠状动脉法洛四联症和右心室双出口一期根治术

目的 总结合并异常冠状动脉的法洛四联症和右心室双出口一期根治术的经验，探讨有关外科技术的改进。方法1995年6月至2002年6月完成该类一期根治术12例，其中2例采取了肺动脉移位、3例在游离的左前降支下加宽右室流出道、5例改变右室切口并在冠状动脉下缝合、2例经肺动脉和右房疏通流出道。结果无手术死亡。随访3个月～6年，无晚期死亡和并发症发生。结论合并异常冠状动脉的法洛四联症和右心室双出口的一期根治手术是可行的，且效果良好，但必须采用适当的外科技术以保护异常的冠状动脉。     

一期手术纠治主-肺动脉窗及伴发畸形

目的总结一期手术纠治主－肺动脉窗及伴发畸形的临床经验，以提高手术疗效。方法我科共收治26例主－肺动脉窗患者．男14例．女12例；年龄1．4±1．6岁；体重7．8±3．8kg。其中单纯主－肺动脉窗8例，合并主动脉弓中断、右肺动脉异常起源于主动脉、法洛四联症、房间隔缺损、室间隔缺损、二尖瓣反流和气管狭窄等18例。25例患者一期手术经主动脉切口补片关闭瘘口纠治主－肺动脉窗，同时纠治伴发的畸形；1例放弃治疗。结果全组无手术死亡。2例伴主动脉弓中断、动脉导管未闭和右肺动脉起源于升主动脉的新生儿术后3d延迟关胸；1例术后出血，3h后再次开胸止血。术后随访22例．随访时间1个月～4年。所有患者无明显的主动脉瓣上狭窄和肺动脉分支狭窄，2例残留轻度二尖瓣反流，1例残留轻至中度二尖瓣反流。结论主－肺动脉窗患者早期易发生肺动脉高压，一经诊断应立即手术。手术方式首选修补主－肺动脉窗及一期纠治伴发畸形。尽管主－肺动脉窗可合并各种心内外畸形，但早期手术纠治可获得较好的中长期疗效。     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

右室双出口的外科治疗

目的：总结手术纠治右室双出口６７ 例的经验。资料与结果：手术年龄４ 个月～１２ 岁,平均（４ ．９８ ±２ ．９６） 岁;体重４ ．１ ～３６ ．０ｋｇ,平均（１５ ．１３ ±５ ．５４）ｋｇ 。其中伴右室流出道梗阻４８ 例,肺动脉高压１８例,肺动脉瓣闭锁１ 例。１３ 例行姑息手术者无死亡。５４ 例行根治术,其中４ 例又行ＩＩ期根治,６ 例行Ｆｏｎｔａｎ 纠治术;根治手术死亡５ 例,病死率８ ．６２ ％ 。本组总病死率７ ．４６ ％ 。结论：右室双出口伴肺动脉高压者,必须早期手术,防止肺血管病变发生。对肺动脉瓣下型室缺的手术纠治较困难,死亡率高。心内隧道补片方法,术后需定期随访,及时发现左室流出道梗阻,必要时需再次手术解除。     

肺动脉右心室直接吻合纠治复杂先天性心脏病

目的 探讨肺动脉右心室直接吻合纠治复杂先天性心脏病的效果。方法 2001年3月至2004年6月，对17例复杂先天性心脏病病儿采用肺动脉右心室直接吻合方法予以纠治，其中3周岁以下者11例。肺动脉闭锁合并室间隔缺损6例，完全性大动脉错位合并室间隔缺损和肺动脉狭窄4例，永存动脉干4例，法洛四联症合并冠状动脉横跨右心室流出道3例。12例一期根治，5例分期纠治。结果 手术死亡1例。5例分期手术中的3例已成功二期根治。随访1～40个月，所有病儿生长发育良好，心功能0～Ⅰ级。结论 肺动脉右心室直接吻合应用于纠治某些复杂先天性心脏病可取得满意效果。对无同种带瓣管道可选用的婴幼儿病例，此术式尤为适用。     

肺动脉发育不良型法洛四联症根治术前姑息手术和介入技术的应用

目的 总结姑息手术和介入技术治疗合并肺动脉发育不良的重症法洛四联症的临床经验.方法 2002年12月至2009年12月,1586例患儿行法洛四联症根治术中18例(男12例、女6例)在根治手术前进行了姑息手术和介入技术相结合的复合治疗.合并心血管畸形包括:房间隔缺损3例,左肺动脉缺如2例,左肺动脉起自主动脉1例,永存左上腔静脉1例,合并粗大体肺侧支血管7例.根治手术前行一次姑息手术者13例,两次者4例,三次者1例.手术术式包括改良Blalock-Taussig分流术14例次,Waterston分流术4例次,右室流出道重建术3例次,肺动脉瓣球囊扩张术3例次,肺动脉环缩1例,行侧支血管结扎6例(16支),侧支血管融合1例(2支),侧支血管介入封堵2例(6支).结果 全组无死亡,1例因人工血管堵塞在术后第1天再次行体肺分流术,患儿根治手术前Nakata指数和McGoon比值[(200±81)和(1.77±0.51)]均较姑息手术前[(84±40)和(1.14±0.33)]有明显增加(P＜0.001),末梢血氧饱和度和血红蛋白浓度[(0.71±0.09)和(175±46) g/L]均显著改善[(0.86±0.05)和(149±15) g/L,P＜0.05].所有18例患儿均完成了最终的根治手术.结论 采用姑息手术和介入技术相结合的复合治疗措施能有效改善肺动脉发育,为合并肺动脉发育不良的重症法洛四联症根治手术创造条件.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

Internal Mammary–Anomalous Left Anterior Descending Coronary Artery Graft in 16-Month-Old Infant with Tetralogy of Fallot: 30-Month Follow-up

Congenital anomalies of the coronary arteries can occur in conjunction with tetralogy of Fallot. We describe a case of unsuspected anomalous origin of the left anterior descending coronary artery arising from the right coronary artery that was discovered at the time of operation in a 16-month-old infant with tetralogy of Fallot. Successful direct vascular repair of the intracardiac anomalies was performed, including left internal mammary–coronary artery anastomosis. The patient, whom we believe to be the youngest to undergo this technique, underwent cardiac catheterization 21 months after operation. The successful results were confirmed at that time.     

Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options

Anomalous origin of the left anterior descending coronary artery from the right coronary artery can interfere with the location of the usual ventriculotomy during repair of tetralogy of Fallot. The purpose of this study was to compare the results of two operative techniques: (1) a "tailored" right ventricular incision and outflow patch reconstruction and (2) placement of a conduit from the right ventricle to the main pulmonary artery. We reviewed the records of 416 patients who had complete repair of tetralogy of Fallot at the Mayo Clinic from 1973 through 1984. Twenty (5%) (median age 6.5 years) had anomalous origin of the left anterior descending coronary artery from the right coronary artery. Twelve of these patients had right ventricular outflow patch reconstruction, and eight had placement of a conduit from the right ventricle to the pulmonary artery. Three deaths occurred, all during hospitalization, two in the patch reconstruction group and one in the conduit group. The average reduction in right ventricular pressure postoperatively was slightly but not significantly greater for the conduit group. These data indicate that correction of tetralogy of Fallot with anomalous origin of the left anterior descending coronary artery can be done with either patch reconstruction or conduit placement. Selection of the more appropriate procedure depends on the exact location and degree of tortuosity of the anomalous artery and the level and severity of right ventricular outflow obstruction.     

Repair of divided anomalous anterior descending coronary artery in tetralogy of Fallot.

Coronary artery anomalies in tetralogy of Fallot have often compromised surgical repair and imposed an increased mortality rate. Thus accidental division of the anterior descending coronary artery crossing the right outflow tract has previously usually resulted in death. The case is presented of a 13-year-old boy with tetralogy of Fallot in whom the anomalous left anterior descending coronary artery was divided. The blood supply to the left anterior descending artery was successfully restored using a reversed saphenous vein graft.     

Characteristics of the radical correction of Fallot's tetralogy in anomalous distribution of the coronary arteries

The variants of distribution of the coronary arteries were studied on 41 specimens of the heart with Fallot's tetralogy. The typical topography of the coronary arteries in this cardiac defect is characterized by counterclockwise rotation of the coronary sinuses, dominant system of the right coronary artery with a large infundibular branch, and right type of heart blood supply. There were 6 variants of anomalies of the coronary arteries. The most characteristic of Fallot's tetralogy were: a single coronary artery, origin of the large infundibular artery by an independent orifice, and anomalous origin of the anterior interventricular branch from the right coronary artery. The peculiarities of radical correction of the tetralogy in atypical distribution of the coronary arteries were discussed on the basis of analysis of 27 operations conducted with the use of non-traditional methods of correction.     

Anomalous origin and distribution of coronary arteries. Review of 38 patients who underwent operation.

Between October 1961 and December 1973, 38 patients with an anomaly in origin (15 patients) or distribution (23 patients) of the main coronary artery or one of its branches underwent operation at the Texas Heart Institute. The left coronary artery originating from the pulmonary artery occurred most frequently-in 13 of 15 patients. An aortocoronary artery bypass was performed in 12 patients with the saphenous vein used in ten of the 12, initially in 1965; and a Dacron tube graft in the other two. Of the 15 patients, only one died during the early period after operation. A follow-up of ten years revealed 11 asymptomatic patients; to date the longest period of patency of a saphenous vein graft is seven years in an 11-year-old girl. Of 23 patients with an unusual coronary artery distribution, 22 had tetralogy of Fallot, 20 of whom underwent total correction. In 21 of the 23 patients the left anterior descending coronary artery originated from the right coronary artery and crossed the right ventricular outflow tract. In two patients this abnormally distributed artery was injured through a vertical right ventriculotomy; both patients died from myocardial failure during the early postoperative period. Subsequently a transverse right ventriculotomy, either alone or combined with a right ventricular outflow and/or pulmonary artery patch enlargement was performed in 16 patients, and a double outlet right ventricle was created through insertion of a Dacron tube graft in two patients. With this method injury to the abnormal left anterior descending coronary artery was avoided and all 18 patients survived the operation. On the basis of our experience and today's advanced techniques, it is believed that most patients, including some under two years of age, can undergo correction of a left coronary artery originating from the pulmonary artery through insertion of a saphenous vein graft between the aorta and left coronary artery. During the surgical correction of cardiac anomalies necessitating a right ventriculotomy, a transverse or double incision in the right ventricular outflow tract in most patients will prevent injury to an abnormally distributed coronary artery branch; sometimes insertion of a Dacron tube graft between the right ventricular outflow tract and pulmonary artery is necessary.     

连续115例小儿法乐四联症外科治疗无手术死亡经验

目的：总结连续１１５ 例小儿法乐四联症根治术无手术死亡经验。方法：１１５ 例法乐四联症病儿,９ 月龄～１３ 岁,平均（４ ．６ ±２ ．７） 岁,３ 岁以下５５ 例,合并有肺总动脉或一侧肺动脉闭锁４ 例,肺动脉瓣缺如１ 例,合并房间隔缺损和动脉导管未闭１１ 例。全部病儿均施行根治手术,用ｄａｃｒｏｎ 补片修补室间隔缺损,用自体心包作右室流出道及肺动脉扩大,１０２ 例（８９ ％ ） 作了跨瓣环补片。结果：全组无手术死亡。随访２ ～４８ 个月,术后３ 个月１ 例死于心律紊乱,其余均恢复良好。结论：提高小儿法乐四联症根治术成功率的关键是：改进手术方法,彻底解除右室流出道及肺动脉远端梗阻,选用适当的转流技术和重视术后监测。     

心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。