Surgical management of congenital coronary artery fistulas.

Congenital fistulas are the most common of the coronary arterial malformations and with the widespread use of selective coronary arteriography are being recognized with increasing frequency. Twenty-eight patients with congenital coronary fistulas have been evaluated at the Duke University Medical Center between 1960 and 1981. An additional 258 patients have previously been reported in the literature, making a total of 286 available for review. The right coronary artery is most commonly involved, and the fistulous communication is most often to the right ventricle, right atrium or pulmonary artery. Slightly more than half of the patients with coronary fistulas are symptomatic at the time the diagnosis is made. Surgical correction is strongly recommended to prevent the development of congestive heart failure, angina, subacute bacterial endocarditis, myocardial infarction, and pulmonary hypertension, as well as coronary aneurysm formation, with subsequent rupture or embolization. There were no operative or late deaths in the patients who underwent operations. Moreover, there have been no recurrent fistulas during a mean follow-up period of ten years. The risks of operative correction appear to be considerably less than the potential for development of serious and potentially fatal complications, even in asymptomatic patients.     

Left main coronary artery-to-pulmonary artery fistula with severe aneurysmal dilatation

Coronary artery fistulas (CAF) are rare abnormalities that can be symptomatic or asymptomatic. Most drain into the right ventricle or pulmonary artery, though a variety of other drainage sites have been reported. We report the results of the surgical closure of a symptomatic left coronary-to-pulmonary artery fistula associated with a giant 10-cm aneurysm and discuss the management of coronary artery fistulas.     

Surgical management of 56 patients with congenital coronary artery fistulas

Over a 25-year period, 58 patients underwent evaluation or surgical treatment of coronary artery fistulas, or both, at our institution. Twenty-one patients had major associated cardiac defects, either congenital or acquired, and 9 had multiple fistulas. Fistulas to the left heart were unusual. Exertional angina and dyspnea were the most common presenting symptoms, but most young patients were asymptomatic. Seventy percent had audible heart murmurs. No operative deaths occurred in patients with isolated fistulas. Coronary artery fistulas cause symptoms due to "coronary steal" with resulting myocardial ischemia and, more rarely, marked left-to-right or left-to-left shunting. Definitive surgical correction is safe and effective, with good long-term results.     

Congenital coronary artery-cardiac chamber fistula: diagnosis and surgical treatment]

From Nov. 1976 to Dec. 1989, 9 patients with congenital coronary artery-cardial chamber fistula were operated upon in our hospital. In this series right coronary artery-right ventricle fistula was found in 3 patients, right coronary artery-left ventricle fistula in 2, left coronary artery-right ventricle fistula in 2, left circumflex artery-right atrium fistula in 1, and bilateral coronary artery-cardiac chamber fistula in 1. Cardiopulmonary bypass was performed in 8. The fistula was closed by interrupted mattress sutures outside the cardiac chamber plus ligation of terminal portion of the coronary artery near the fistula in 5 patients, and by mattress sutures inside the dilated coronary artery or cardiac chamber in other 4. There was no operative death in the 8 patients with single fistula. One patient with bilateral coronary artery fistula died from hypoxemia postoperatively. Late complications or recurrent fistulas were not found in the follow-up period from 3 months to 12 years. The diagnosis, operative indications and technique of closing fistula were discussed.     

Traumatic coronary artery fistula

Coronary artery fistulas can occur in patients who survive cardiac trauma. We report one such case with development of a right coronary artery-right atrial fistula 2 years after injury. The literature shows that surgical correction should be performed before the development of incapacitating symptoms (angina, pulmonary hypertension, congestive heart failure). Proximal and distal ligation of the affected coronary artery with distal bypass grafting is the recommended surgical procedure. Other procedures have led to recurrence of the fistula.     

成人先天性冠状动脉瘘的诊断和外科治疗

目的　总结成人先天性冠状动脉瘘 (CAF)的临床特点、诊断和外科治疗经验。　方法回顾性分析 1985年 3月～ 2 0 0 2年 4月间我院外科治疗的 14例成人CAF的临床资料。其中男 8例 ,女6例 ;平均年龄 ( 32± 13)岁 ;有症状者 11例。本组患者术前经超声心动图或心导管造影检查明确诊断。右冠状动脉 心腔瘘 10例 ( 71% ) ,其中瘘口位于右心室 6例、左心室 3例、右心房 1例 ;左冠状动脉 心腔瘘 3例 ( 2 1% ) ,其中瘘口位于右心室 2例、左心室 1例 ;双冠状动脉 肺动脉瘘 1例 ( 7% )。瘘口为单个 12例 ,多个 2例 ;瘘口直径 0 30～ 1 80cm ,平均 ( 1 16± 0 49)cm。合并冠状动脉瘤 6例、其他心血管病变 4例。CAF瘘口在体外循环下闭合 10例 ,非体外循环下直接结扎或缝扎 4例。合并病变同期处理。　结果　全组患者无早期死亡。长期随访 12例 ( 85 71% ) ,平均随访时间 ( 3 35± 4 2 8)年。患者无心肌缺血或梗死、残余瘘和晚期死亡。心功能恢复至Ⅰ级 11例、Ⅱ级 1例。　结论　成人CAF已存在明显的血液动力学和心血管形态学改变 ,应尽早手术治疗。合适的手术方法和确实的心肌保护是取得良好外科手术疗效的关键。     

Surgical treatment of congenital coronary artery fistulas: 27 years' experience and a review of the literature

BACKGROUND AND AIM OF THE STUDY: A congenital coronary artery fistula (CAF) is a relatively rare congenital anomaly and is defined as an abnormal direct communication between any coronary artery and any of the cardiac chambers. This article reviews our experience over the past 27 years, as well as other literature, and discusses the surgical indications and methods relating to CAF. METHODS: From 1973, 25 patients aged from 2 to 69 years underwent surgical treatment for congenital CAF. Seventeen patients were diagnosed as isolated CAF. All patients under 19 years of age with isolated CAF were asymptomatic. Twenty fistulas originated from the left coronary artery and 9 from the right. The pulmonary artery was the most dominant drainage site. Four patients among the isolated CAF cases were surgically treated without a cardiopulmonary bypass. RESULTS: All patients were discharged from hospital without any perioperative complications. Postoperative coronary angiography was done on all patients with only one slightly residual CAF flow. The average follow-up time was 9.6 years and all patients were asymptomatic and doing well. CONCLUSIONS: Definitive surgical correction is safe and effective, with good results. Therefore, it should be considered even in asymptomatic patients because of the risk of future complications.     

Surgical treatment of congenital coronary artery fistula.

Six patients with congenital coronary artery fistula underwent successful corrective surgery. Precise diagnosis was established either by retrograde aortography or more recently by selective arteriography. The left coronary artery was involved in four and the right in two cases. The fistula communicated with the right ventricle in three and the right atrium in three subjects. The operative approach is dictated by the site of entry of the vessel into the cardiac chamber. The use of cardiopulmonary bypass for intracardiac repair allows accurate closure of the fistula thereby reducing the chances of recurrence. A follow-up of one to seven years showed that all patients are asymptomatic and leading normal lives.     

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.     

Surgical treatment of coronary artery-pulmonary artery fistula with coronary aneurysm]

We experienced 4 cases of coronary artery-pulmonary artery fistula with coronary aneurysm, three patients had symptoms of chest pain, and 1 patient had cardiac murmur. Coronary arteriography showed that three patients had fistulas from the left coronary artery to the pulmonary artery; and that 1 patient had a fistula from both the right and left coronary arteries to the pulmonary artery. Moreover, 1 patient had 90% diameter stenosis of segment 7. The maximum diameter of the coronary aneurysm ranged from 12 to 20 mm (average: 15.3 mm). One patient underwent closure of the opening of the fistula, 2 patients underwent multiple ligatures of fistulas, and 1 patient underwent multiple ligatures of fistulas with coronary artery bypass grafting. The postoperative course of every patient was uneventful. There have been 42 reports on this abnormality in Japan. We should treat the fistula as early as possible to prevent cardiac complications such as myocardial ischemia and rupture of coronary aneurysm.     

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect

BACKGROUND: The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD). METHODS: We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998. RESULTS: CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized. CONCLUSIONS: The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.     

Surgical correction for sinus of Valsalva aneurysm

BACKGROUND: To evaluate the efficacy of surgical correction of sinuses of Valsalva aneurysms, 24 patients operated on from 1974-1994 were reviewed. Mean age was 42.2+/-16.7 years; 17 were males. METHODS: Right coronary sinus was affected in 13 patients and noncoronary sinus in 8. Intracardiac fistula was detected in 16 patients: into the right atrium in 8; right ventricle in 5; left ventricle in 2; and left atrium in 1. Repair was performed via an aortic approach in 11 patients and a combined aortic and intracardiac approach in 13 patients. Suture closure of the fistula was via the aortic side in 10 cases and the intracardiac side in 6. Ten patients had patch closure via the aortic side. RESULTS: Follow-up was 92% complete at a mean of 8.4 years with 3 late cardiac and 1 noncardiac deaths. Eighty-eight percent of patients are in New York Heart Association functional class I or II; none underwent reoperation. CONCLUSIONS: Sinuses of Valsalva aneurysm repair have low operative mortality and morbidity risks with excellent early and late results. Surgical approach is dependent upon the presenting pathology.     

Surgical repair of coronary sinus type partial anomalous pulmonary venous drainage with intact atrial septum

We experienced a rare form of PAPVD without atrial septal defect. The patient was a 33-year-old male and he was referred to our institute because of mild right pulmonary congestion detected by a routine chest X-P. Enlarged coronary sinus, right atrium and right ventricle were documented by UCG. The Qp/Qs was 1.9 and pulmonary artery pressure was 38/7 mmHg (mean: 17 mmHg) by cardiac catheterization. Selective pulmonary angiogram showed that all right pulmonary veins drained into the coronary sinus without evidence of an atrial septal defect. Enhanced chest CT clearly demonstrated the connection between the right pulmonary vein and the coronary sinus. Intracardiac repair without atrial baffle was carried out under hypothermic cardiopulmonary bypass. Under cardiac arrest with cardioplegia, the common wall between the right pulmonary vein and the left atrium was incised and the connection between the right pulmonary vein and the left atrium was established. The flap made by this incision was brough posterior to close the right pulmonary vein opening to the coronary sinus. The postoperative course was uneventful and the minimum diameter of the right pulmonary vein was found to be 15.5 mm by a postoperative pulmonary artery angiogram. This operative method without an atrial baffle could be an alternative procedure for coronary sinus type PAPVD.     

Coronary Artery Fistula Associated with Mitral Valve Endocarditis

Abstract Coronary artery fistulas are rare, and the further development of mitral valvular insufficiency and endocarditis is even more uncommon. We report a case of endocarditis secondary to a congenital coronary artery fistula arising from the right coronary artery and draining into the left ventricle. Vegetations were found on the mitral valve leaflet. The fistula was successfully treated with surgery, and the endocarditis, with antibiotic therapy. Surgical repair is the optimal treatment for coronary artery fistula, even in asymptomatic patients. (J Card Surg 2012;27:714‐715)     

Aberrant origin of the left main coronary artery arising from the right coronary artery associated with coronary artery disease: a case report

Coronary artery anomalies are not frequent, nevertheless they are associated with increased and potentially lethal cardiac events. Recognition of these anomalies is fundamental in patients undergoing diagnostic or interventional coronary angiography. Most patients presenting with coronary anomalies are asymptomatic, but the risk of myocardial ischemia and sudden death requires the treatment of those patients. Different therapeutic options have been discussed, including surgery, conservative therapy, and interventional approaches. In this report, an aberrant origin of the left main coronary artery arising from the right coronary artery associated with coronary artery atherosclerosis and its surgical correction is described.     

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。     

Anomalous left coronary from the pulmonary artery-clinical profile,operative techniques and patient outcomes

Background Anomalous left coronary artery arising from the pulmonary artery is a rare congenital heart disease. Mortality is in excess of 90% if left untreated during the first year of life. Surgical management has evolved from coronary ligation to anatomical repair by coronary button translocation to the aorta. We report our recent experience with surgical correction of this rare condition. Methods We retrospectively studied the case records of 14 consecutive patients operated in our institute between Jan 1998 to Aug 2004. The mean age was 65 months ±114 (Range 1 to 420). The male to female ratio was 2.5:1. Coronary ligation ws performed in two patients, coronary artery bypass grafting with saphenous vein graft was done in three, the last nine patients underwent coronary button transfer, one patient required concomitant mitral valve replacement. Clinical profile, surgical techniques and operative outcomes as well as long-term results were analyzed. Results Hospital mortality was 28.5% (4/14), Follow up was 100% complete with no late mortality, ranging from 1 months to five years. Ischaemic mitral regurgitation progressed in only one patient, remained the same in one and improved in all the others. All patients except one are in functional class I at last follow up. Conclusion In conclusion, infants presenting with a diagnosis of anomalous left coronary artery arising from pulmonary artery with congestive cardiac failure are a difficult subgroup of patients. Coronary button translocation has emerged as the single most effective surgical modality and is associated with good long-term results.     

Large common left and right coronary artery to coronary sinus fistula

Coronary fistulas are vascular anomalies which in rare cases can cause hemodynamic problems with indication for intervention. We report about a 47-year-old man with a large coronary fistula arising from both, the left and right coronary artery. To our knowledge this is the first case described with this anatomy. The main coronary arteries were united at the crux cordis and drained through the coronary sinus into the right atrium. As a consequence of the longstanding volume overload the coronary arteries were extremely dilated. Also, both ventricles were dilated. Therefore, although the patient was asymptomatic, the obvious compromise of the ventricles due to volume overload was regarded as an indication for surgical intervention.     

Anatomic correction of double-outlet right ventricle with subpulmonary ventricular septal defect (the "Taussig-Bing" anomaly)

Seven patients with double-outlet right ventricle and subpulmonary ventricular septal defect (the Taussig-Bing anomaly) underwent anatomical repair at the arterial level with transfer of the coronary arteries. At the time of operation, patient ages ranged from 6 weeks to 33 months (mean 14.1 months) and weight ranged from 3.7 to 11.5 kg (mean 7.0 kg). Four patients had prior pulmonary artery banding: Two of these four also had coarctation repairs, and one had a Blalock-Hanlon septectomy. Three different patterns of coronary artery distribution were encountered. Five patients had side-to-side great arteries, and two had more or less anteroposterior great arterial relationships. There was one operative death (14.3%: 70% confidence limits 1.9 - 40.7%) resulting from muscular subvalvular right ventricular outflow tract obstruction (RVOTO). There have been no late deaths in the six survivors followed 6 to 31 months postoperatively (mean 14.8 months). One patient required closure of a residual ventricular septal defect (VSD) and infundibular resection for RVOTO 4 months postoperatively. All other survivors are functionally NYHA Class I. Five of the six survivors have undergone postoperative catheterization (mean interval 5.8 months). There was no aortic insufficiency and good ventricular function in all patients. In addition to the patient with the residual VSD, two other asymptomatic patients had mild or moderate RVOTO. Compared with alternative surgical procedures for this anomaly, anatomic correction has the advantages of acceptable operative mortality, use of the left ventricle as the systemic ventricle, no need for extracardiac conduits, and applicability to patients with all variations of coronary artery and great artery anatomy.     

The anatomy of the heart

The normal heart is the size of the patient's closed fist. The venae cavae drain into the right atrium, which bears the fossa ovalis and receives the coronary sinus and the anterior cardiac vein. The atrium empties into the right ventricle through the tricuspid valve. Both ventricles have trabeculated walls (trabeculae carneae), and from some project the papillary muscles, bearing the chordae tendinae attached to the free borders of the tricuspid valve. The same arrangement is seen on the left side. The right ventricle leads to the pulmonary trunk, guarded by its three valve cusps. Oxygenated blood returns to the left atrium via the four pulmonary veins and passes to the left ventricle via the mitral valve. Exit is through the tricuspid aortic valve. The right and left coronary arteries arise above the valves, their orifices lying in the sinuses of Valsava. The right coronary artery lies in the right part of the atrioventricular groove and gives off the posterior interventricular artery. The left coronary arteries divide into the anterior (descending) interventricular branch and the circumflex branch. Major veins accompany the arteries, except for the anterior cardiac vein, which drains directly into the right atrium.