52例先天性冠状动脉瘘的外科治疗

目的　总结 5 2例先天性冠状动脉瘘的外科治疗效果。方法　全组 5 2例中 ,年龄 9个月～ 5 8岁 ,平均 (15 7± 16 4)岁。 36例为单纯的先天性冠状动脉瘘 ,16例合并有其他心脏外科疾病。5 2例中 ,右冠状动脉瘘占 71 2 % ,左冠状动脉瘘占 2 8 8%。瘘口于右室 ,右房 ,左室 ,左房及肺动脉分别为 42 3% ,30 8% ,11 5 % ,5 8%和 9 6 %。结果　手术均在体外循环下进行 ,10例切开冠状动脉闭合瘘口 ,2 6例经心腔闭合瘘口 ,16例分别闭合在冠状动脉内和在心腔内的瘘口 ;16例合并其他心脏病变同期矫治。心腔内发现二个瘘口者有 6例 ,三个瘘口者 3例 ,余 43例为单一瘘口。全组无死亡及残余漏。 37例随防 1个月～ 8年 ,平均 3 5年 ,均无症状。一例有ST T改变。结论　外科治疗先天性冠状动脉瘘是安全和有效的方法 ,合并有其他心脏外科疾病应同时矫治。准确判断瘘口的位置和确切可靠的缝合技术是防止残余漏的关键。     

双主动脉弓合并复杂心内畸形患者的同期矫治

目的 探讨双主动脉弓合并心内畸形患者的临床特点和同期矫治手术方法.方法 回顾性分析4例双主动脉弓合并心内畸形患者的临床资料,着重分析手术方法和手术结果.结果 4例双主动脉弓合并复杂心内畸形的患者中,2例合并法洛四联症,2例合并右心室双出口.手术经正中开胸,在锁骨下动脉远端切断一侧小弓,同时切断动脉导管或动脉韧带,松解气管食管周围组织,同期行心内畸形矫治.1例术后因呕吐误吸导致病情恶化死亡,其余3例存活,未出现与双弓矫治相关的并发症.结论 双主动脉弓手术治疗可取得良好效果,对于合并心内畸形的双主动脉弓患者,可经胸骨正中切口同期矫治.     

冠状动脉瘘的诊断及治疗方法探讨

目的探讨冠状动脉瘘(CAF)的诊断及治疗方法。方法16例冠状动脉瘘患者，经彩色多普勒超声检查14例，确诊12例，升主动脉及冠状动脉造影检查4例均确诊。16例患者均行手术治疗．其中非体外循环下行缝扎瘘口1例，体外循环下经心腔内闭合瘘口12例，切开冠状动脉闭合瘘口1例，分别闭合冠状动脉内瘘和心腔内瘘口2例；同期行冠状动脉旁路移植术1例，室间隔缺损修补1例。结果手术发现单一瘘口11例，两个瘘口5例。全组无死亡病例及并发症发生。术后随诊无残余瘘发生。结论选择性冠状动脉造影检查是诊断CAF的金标准；CAF自行闭合性小，一旦确诊，即使无症状也应及早手术治疗。手术宜在体外循环下进行，准确判断漏口位置和精确缝合是手术成功的关键。     

双主动脉弓合并复杂心内畸形患者的同期矫治

目的探讨双主动脉弓合并心内畸形患者的临床特点和同期矫治手术方法。方法回顾性分析4例双主动脉弓合并心内畸形患者的临床资料，着重分析手术方法和手术结果。结果4例双主动脉弓合并复杂心内畸形的患者中．2例合并法洛四联症，2例合并右心室双出口。手术经正中开胸，在锁骨下动脉远端切断一侧小弓．同时切断动脉导管或动脉韧带，松解气管食管周围组织，同期行心内畸形矫治。1例术后因呕吐误吸导致病情恶化死亡，其余3例存活，未出现与双弓矫治相关的并发症。结论双主动脉弓手术治疗可取得良好效果，对于合并心内畸形的双主动脉弓患者，可经胸骨正中切口同期矫治。     

先天性冠状动脉瘘的诊断与治疗(附34例报告)

目的:总结分析34例先天性冠状动脉瘘(CAF)的临床诊断治疗与外科手术方法。方法:回顾性分析应用超声心动图、64层螺旋CT、选择性冠状动脉造影等检查确诊的34例CAF患者的临床资料,其中24例为单纯性CAF,10例合并其他心脏外科疾病。结果:34例中5例直接行动脉瘘切线缝合术,其余29例均在体外循环直视下行瘘口修补并同时矫治合并心脏疾病。心腔内发现2瘘口1例,4瘘口1例,其他均为单一瘘口。所有患者术毕效果明显,无一例死亡,超声心动图复查均无残余瘘存在。22例随访0.5~3年无死亡及并发症发生。结论:64层螺旋CT检查可准确显示各种CAF的起源、行程、引流部位及并发异常,显示冠状动脉近段管腔的形态及一些较大冠状动脉瘘,是一种较为理想的无创性诊断CAF的影像方法。CAF明确诊断后,及时行外科手术治疗安全可靠,少数患者可行介入治疗。     

先天性冠状动脉瘘5例治疗体会

总结先天性冠状动脉瘘５例手术治疗体会。作者认为：冠状动脉瘘虽为罕见先天性心脏畸形,但手术安全,效果良好。非手术治疗远期可发生心肌缺血、心肌梗死、冠状动脉瘤破裂等并发症。故一经确诊,均宜手术治疗。手术方法以体外循环下切开瘘口所在心腔或冠状动脉缝闭瘘口为宜。冠状动脉造影可确定瘘口位置,具有确诊价值。     

先天性冠状动脉畸形11例报告

报告经冠状动脉造影或手术证实的单纯性先天性冠状动脉畸形11例,约占同期580例冠状动脉造影的1.6％。本组冠状动脉畸形病变分两大类:①冠状动脉瘘8例,含右冠状动脉右室瘘6例;②冠状动脉起源异常3例。指出冠状动脉瘘需与其他先天性心脏病鉴别,心脏B超对诊断有一定的帮助,冠状动脉造影为确定诊断的重要手段;对大的冠状动脉瘘应及时手术治疗。     

双腔右心室11例报告

本文报告11例双腔右心室(DCRV),共中9例合并室缺,仅2例于术前通过造影确诊。经在体外循环下行心内畸形矫治手术,术后随访10个月至10年,效果良好。作者结合文献对DCRV的发生机制、临床特征及手术有关问题进行了讨论。     

先天性冠状动脉瘘的外科治疗

目的总结外科手术治疗冠状动脉瘘的临床经验及治疗方法，以提高对该类疾病的治疗效果。方法对2001年2月至2011年11月收治的104例先天性冠状动脉瘘患者的临床资料进行回顾性分析，其中男性48例，女性56例，年龄5个月至71岁，平均年龄34岁。104例中有56例患者在非体外循环下行直接结扎及缝扎，其中微创小切口14例；48例合并其他先天畸形及心脏疾病者在体外循环下施行手术，直接结扎及缝扎瘘口，对合并的心脏畸形或疾病同期做相应的矫治。结果瘘口发生于右冠状动脉63例，发生于左冠状动脉41例，瘘人右心房21例，瘘人右心室40例，瘘人肺动脉30例，瘘人冠状静脉1例，瘘入上腔静脉1例。除1例因其他疾病死亡外，全组无手术死亡。随访2-10年，复查超声均无异常。结论先天性冠状动脉瘘可采用直接结扎及缝扎方法，部分病例可采用小切口治疗，手术效果安全有效。     

婴幼儿主动脉缩窄合并心内复杂畸形的一期手术治疗

目的:探讨婴幼儿主动脉缩窄合并心内复杂畸形一期手术的治疗方法及手术效果.方法:回顾分析2002-11至2008-09收治的婴幼儿主动脉缩窄合并心内复杂畸形一期手术治疗的临床病例.共计18例,其中男12例,女6例.年龄平均(7.7±8.8)个月,体重(6.1±2.4)kg.主动脉缩窄包括合并主动脉弓发育不良5例,单纯主动脉缩窄13例.不合并主动脉弓发育不良者,切除缩窄段,端端吻合降主动脉;合并主动脉弓发育不良者,切除缩窄段,用自体心包或自体肺动脉片加宽主动脉弓.心内复杂畸形的矫治包括完全性大动脉转位大动脉调转术8例,右心室双出口根治术6例,右心室双出口陶西平畸形大动脉调转术3例,完全性心内膜垫缺损矫治术1例.结果:所有患儿出院前超声心动图检查均显示主动脉弓降部缩窄解除,吻合口通畅,血流正常.无中枢神经系统并发症.术后早期上、下肢收缩压基本相等者4例,上肢高于下肢15～20mmHg者8例,上肢低于下肢收缩压5～10mmHg者6例.死亡3例,死亡率16.7%.1例术后当天死于低心排综合征,1例术后9天死于肺部感染,1例术后7天死于突发恶性心律失常.术后另3例出现右侧膈肌麻痹行膈肌折叠术.结论:婴幼儿主动脉缩窄合并心内复杂畸形经胸骨正中切口一期手术治疗,主动脉弓及降主动脉上段显露良好.同期矫治心内复杂畸形手术效果满意.深低温停循环区域性脑灌注可有效降低神经系统并发症的发生.     

冠状动脉瘘封堵技巧与效果

目的:冠状动脉瘘（coronary artery fistula,CAF）出口多变,形态多样,本研究探讨不同冠状动脉瘘的封堵技巧与封堵术治疗效果。方法:纳入1999年1月~2012年12月所有试图实施CAF封堵术的患者,在除外其它心脏畸形的基础上,根据主动脉或者冠状动脉造影观测CAF解剖形态,选择封堵术径路、封堵器类型和大小,封堵术后定期随访。结果:共纳入36例患者（男性17例）,年龄3至74 岁(中位数21岁)。CAF起源于左冠状动脉13例（36%）,右冠状动脉18例（50%）,双侧冠状动脉5例（14%）,引流至左心室7例（19%）,右心系统29例（81%）,包括右心房7例,右心室14例和肺动脉8例。成功封堵25例,成功率69%。经静脉途径封堵9例,CAF出口分别为右心房（n=5）,右心室（n=3）和肺动脉（n=1）;经动脉途径封堵16例,出口分别为左心室（n=3）, 右心房（n=1）,右心室(n=10)和肺动脉（n=2）。术后出现短暂胸痛2例,心电图ST T改变6例和再通1例。结论:介入方式治疗CAF安全、可靠,但具体采用何种径路和封堵器,需要根据其解剖形态确定。     

常温非体外循环下手术治疗先天性冠状动脉瘘

目的:探讨常温非体外循环下,手术治疗先天性冠状动脉瘘(congenital coronary arterial fistula,CAF)的手术方法和远期效果。方法:2001年8月至2009年8月在阜外心血管病医院行常温非体外循环下手术治疗CAF21例。全组年龄12d～60岁,平均(20.6±20.0)岁;20例为单纯的CAF,1例合并冠心病3支病变。21例中瘘口起源于右冠状动脉5例(23.8%),左冠状动脉12例(57.1%),双冠状动脉4例(19.1%);瘘口入右心房,右心室,肺动脉及左心室分别为5例(23.8%),8例(38.1%),7例(33.3%)和1例(4.8%);结果:21例均在常温非体外循环下行冠状动脉瘘结扎或褥式带垫片缝扎,其中1例同时行常温不停跳冠状动脉搭桥术,无手术死亡。术后残余瘘1例,其余术后无心肌缺血及残余瘘等并发症。19例随访0.5～5.5年,平均(2.1±1.7)年,无远期死亡。1例残余瘘,其余无心肌缺血及残余瘘等并发症。结论:常温非体外循环下手术治疗CAF安全、有效,远期效果良好。     

Coronary artery fistula: Comparison of diagnostic accuracy by echocardiography versus coronary arteriography and surgery in 63 patients studied between 2002 and 2012 in a single medical center in China

Background

Coronary artery fistula (CAF) is a rare congenital anomaly, which is conventionally diagnosed by coronary arteriography; however, the relation of the coronary artery fistulas to other structures, their origin and course may not be always apparent.

Methods

The echocardiograms of 63 patients with coronary artery fistulas, who had undergone coronary arteriography and/or surgery from June 2002 to December 2012 at the Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, were analyzed retrospectively, and the results were compared with findings by coronary arteriography and at surgery.

Results

Right CAFs were detected in 33 of the 63 patients (52.4%); 11 had drainage to the right atrium, 10 to the right ventricle, 2 to the left ventricle, 9 to the main pulmonary artery, and 1 to the coronary sinus. Left CAFs were detected in 29 patients (46.0%); 6 had drainage to the right atrium, 12 to the right ventricle, 1 to the left atrium, 2 to the left ventricle, 7 to the main pulmonary artery, and 1 to the coronary sinus. One patient (1.6%) had the origin of the fistula in both coronary arteries. The entry point of the fistula was most often a single orifice (96.8%) and rarely multi-orificial (3.2%). 57 patients (90.5%) had isolated coronary fistulas (90.5%); 6 patients (9.5%) had other congenital cardiac malformations. The ultrasonic diagnosis of 60 patients was in line with findings at surgery and/or coronary arteriography. The diagnostic accuracy rate for coronary artery fistula was 95.2%. Preoperative transthoracic echocardiography missed the diagnosis of coronary artery fistula in three patients (4.8%). There is no difference (P > 0.05) in diagnostic accuracy between echocardiography and coronary arteriography and/or surgery.

Conclusions

Transthoracic echocardiography, in comparison with coronary arteriography and/or surgery, is much simpler, easier, less expensive, safer, readily repeatable, and more convenient with equal accuracy, and should be the first-line method for the diagnosis of congenital coronary artery fistula.     

Right coronary artery fistula to left ventricle complicated with huge coronary artery aneurysm

Congenital coronary artery fistula (CAF) with huge coronary artery aneurysm is a very rare condition. In this paper, we describe a 26-year-old asymptomatic male patient with right coronary artery (RCA) to the left ventricle fistula with a huge coronary artery aneurysm which was diagnosed by multidetector computed tomography and coronary angiography. The patient received surgical treatment for coronary artery after diagnosis. Both RCA and a giant aneurysm were excised; surgical closure of CAF and coronary artery bypass grafting were performed on this patient. Two months after surgery, the enlarged left ventricle returned to normal as evaluated by echocardiography.     

Transcatheter closure of right coronary artery fistula to the right ventricle

Coronary artery fistula (CAF) is an uncommon anomaly that is usually congenital but can be acquired. Although most patients are asymptomatic, some may present with congestive heart failure, infective endocarditis, myocardial ischemia or rupture. In the past, surgical ligation was the only option in the management of CAF, but since 1983, transcatheter closure of CAF has been increasing as an alternative to surgery. We report a 3-year-old boy, presented in Queen Alia Heart Institute, who underwent successful transcatheter closure of a large fistula communicating the distal part of the right coronary artery to the right ventricle. Our case differs from other CAFs in that the fistula was communicating the right coronary artery itself to the right ventricle.     

冠状动脉瘘的临床分析

目的探讨11例先天性冠状动脉瘘(CAF)的临床特点及治疗方法。方法2003年至2005年,应用超声心动图、升主动脉及选择性冠状动脉造影检查确诊CAF 11例,其中单支瘘管起源于左冠状动脉5例,右冠状动脉2例,双侧起源4例。6例行外科手术治疗,2例患者行导管弹簧栓堵闭术,另3例未进行有创治疗,以药物保守治疗。结果8例手术及介入治疗均成功,无死亡及并发症发生。所有患者随访6～36个月,均无残余瘘发生。结论超声心动图及选择性冠状动脉造影是CAF的主要确诊手段。外科手术和经导管栓堵术闭合CAF均安全有效,应在临床症状出现之前进行根治性治疗。     

Unusual presentation of a huge right coronary artery fistula; Technical issues with device closure

Coronary artery fistula (CAF) is considered an embryologic persistence of primitive intra-trabecular spaces which allow the developing coronary artery to communicate with the other cardiac chambers or vascular structures. It is observed in 0.05–0.25% of coronary angiographic studies, most of which drain into a right heart chamber or into the pulmonary artery, while a congenital right coronary artery (RCA) into a left heart chamber is less frequent.⁵ In this study, we describe an unusual case treated by closure device in the right coronary artery fistula to the left ventricle, and associated literature is reviewed. A 40-year-old female presented with chronic cough, otherwise asymptomatic. Echocardiogram revealed unusual flow into the LV with mild LV dilatation. A 64 multi-slice CT scan confirmed the presence of a huge right coronary opening with a fistula into the left ventricle. The decision was to close this fistula with device through the RCA into the left ventricle. The management of this unusually large fistula is described with focus on technical issues with device closure.