Coronary vessel anomaly: fistula between the right coronary artery and the left ventricle clinically presenting as aortic valve insufficiency (author's transl)]

Under more than 200 cases of coronary vessel anomalies only 33 are fistulas from the right or left coronary artery into the left ventricle. The case presented here of a fistula from the right coronary artery into the left ventricle featured the clinical picture of an aortic valve insufficiency. The histologic findings support the theory that these fistulas are embryological anomalies: transient communications between the ventricular trabeculas and the coronary vessels may be lined with endothelium and become then persisting as fistulas.     

Coronary fistula to the left ventricle: assessed by computed tomography

Coronary artery fistulas are rare and are most often diagnosed by echocardiography or by cine-angiocardiography. However, the computed tomography angiography (CTA) of coronary arteries has been gaining ground. The incidence of this disease is very low, with a more frequent occurrence of fistulas originating in the right coronary artery. There is a higher incidence of coronary artery fistulas to right heart chambers, with coronary artery fistulas to the left ventricle (LV) being rare. Treatment can be surgical or percutaneous. This report describes a case of coronary fistula to left ventricle diagnosed by CT angiography of coronary arteries in a hypertensive and asymptomatic 46-year-old male, who was tested positive for ischemia in an exercise test. The CT angiography ruled out coronary obstructive disease, but it revealed a coronary fistula to the left ventricular cavity.     

Transcatheter closure of coronary artery fistulas.

Transcatheter closure of a coronary artery fistula was undertaken in nine patients. There were three fistulas from the left circumflex coronary artery to the coronary sinus, three from the left anterior descending coronary artery to the right ventricular apex, two from the right coronary artery to the superior vena cava/right atrial junction and one fistula from the left circumflex artery to the pulmonary artery. The fistula was closed with Gianturco coils in six patients, a double-umbrella device in two and a combination of an umbrella and coils in one patient. All fistulas are completely occluded. Complications consisted of migration of two coils, one of which was retrieved, and a transient junctional tachycardia in one patient. In an additional three patients with multiple coronary artery fistulas, transcatheter occlusion was not attempted.     

Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

BACKGROUND. Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses. METHODS AND RESULTS. Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction. CONCLUSIONS. 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.     

Transcatheter coil embolization of iatrogenic coronary artery–left ventricle fistula after mitral valve replacement

Acquired causes of coronary fistulas are rare disorders and may develop following coronary atherosclerosis, infection, or trauma (iatrogenic). Iatrogenic coronary fistulas may be acquired secondary to surgical or nonsurgical interventions. We describe a case of an iatrogenic coronary artery–left ventricle fistula following mitral valve replacement surgery, presented with ventricular arrhythmia and heart failure. In a unique technique, we implanted three coils with the aid of a Scepter C balloon with inflation at the ostial portion of the left circumflex artery.     

Exercise‐induced ventricular arrhythmia due to bilateral coronary to pulmonary artery fistulas

Ischemic polymorphic ventricular ectopy was documented during exercise testing in a 65‐year‐old Caucasian male patient. Coronary angiogram revealed four coronary to pulmonary artery fistulas (CPAFs) originating from the right and left coronary artery, leading to myocardial ischemia due to steal phenomenon. The three dominant fistulas were coiled percutaneously, while one small fistula was left untreated. During follow‐up, no significant residual ventricular arrhythmia was detected.     

Coronary artery fistulas in adults: Incidence,angiographic characteristics,natural history

The incidence, angiographic characteristics, and natural history of coronary artery fistulas in patients undergoing diagnostic cardiac catheterization have not been well defined. Of 33,600 patients who had diagnostic cardiac catheterization, 34 (0.1%) had coronary artery fistula. Nineteen fistulas originated from the right, 11 from the left anterior descending, and 4 from the circumflex coronary arteries, respectively. The mean ratio of pulmonary to systemic flow was 1.19 ± 0.33. Only one patient with coexistent atrial septal defect had a pulmonic to systemic flow ratio >1.5. Right and left heart pressures, with the exception of three patients in whom left ventricular end-diastolic pressure was >12 mm Hg, were within normal limits. During a mean follow-up period of 6.3 years (range 2–14 years), there were no complications related to coronary artery fistula. It was concluded that the incidence of coronary artery fistulas detected during diagnostic coronary angiography is very low. Coronary artery fistulas originate predominantly from the right coronary artery and are not associated with hemodynamic abnormalities or other congenital heart diseases. The prognosis of coronary artery fistulas in adults is good. © 1995 Wiley-Liss, Inc.     

Coronary ventricular fistulas in patients with heart transplants

In detailed angiographic follow-up examinations in patients after heart transplantation, coronary artery-ventricular fistulas were observed in two of ten patients. In one patient, in the first postoperative year, 19 right ventricular biopsy procedures were performed obtaining a total of 71 tissue specimens and one year after transplantation, additionally, three specimens were obtained from the left ventricle. Coronary angiography demonstrated a fistula from the first anterior ventricular branch of the right coronary artery into the right ventricle (Figure 1) as well as a second smaller fistula between from a septal perforator of the left anterior descending artery into the left ventricle. At follow-up angiography two months later, the fistulas were unchanged in site and extent (Figure 2). Angiographically there was diffuse hypokinesis of the right ventricle and moderate tricuspid regurgitation. In the second patient, in the first year after transplantation, 20 right ventricular biopsy procedures were performed obtaining a total of 80 tissue specimens. One year after transplantation coronary angiography showed a fistula between a septal perforator of the left anterior descending artery into the right ventricle (Figure 3) which, two months later, was unchanged in morphology and, as in the first case, the size of the shunt was small. Angiographically, there was moderate tricuspid regurgitation and apical hypokinesis of the left ventricle. If a relationship between the fistulas and biopsies is postulated, from a total 196 biopsy procedures obtaining 748 tissue specimens, the rate of this complication would be calculated at 1.5%. It cannot be ruled out, however, that the fistulas had been congenitally present in the donor hearts.(ABSTRACT TRUNCATED AT 250 WORDS)     

Coronary to pulmonary artery fistulas: an incidental finding with challenging therapeutic options

We report the incidental finding of 2 coronary to pulmonary artery fistulas observed at coronary angiography performed in a 48-year-old man presenting with acute inferior ST-elevation myocardial infarction (STEMI). Coronary angiography revealed an acute thrombotic occlusion of the mid segment of the right coronary artery (RCA), which was treated with thromboaspiration and bare-metal stenting. Significant stenoses of the left anterior descending (LAD) and left circumflex (LCX) arteries were also observed during angiography, as well as the presence of 2 large coronary to pulmonary artery fistulas, one originating from the proximal LAD and the other from the ostial RCA. The clinical evolution was uneventful and the patient underwent successful coronary bypass grafting of the LAD and LCX lesions associated with ligation of the coronary artery fistulas 6 weeks later. The fact that 2 large coronary to pulmonary artery fistulas were observed during an acute coronary syndrome in a previously asymptomatic patient with extensive coronary artery disease is of particular interest, because it allowed early surgical correction of this rare inborn coronary anomaly before the development of late and irreversible left ventricular dysfunction.     

Evaluation of Coronary Artery Fistula by Color-Flow Doppler Echocardiography

An asymptomatic newborn infant with a left coronary artery to right ventricular apex fistula was evaluated using color-flow Doppler techniques. Color flow mapping during diastole showed a prominent turbulent flow signal that could be traced from the proximal left coronary artery, along the interventricular septum, to the right ventricular apex. Color flow Doppler is an important complement to two-dimensional imaging for the identification and location of coronary artery fistulas.     

Sudden death in a patient with multiple left anterior descending coronary artery fistulas to the left ventricle

Coronary fistulas to cardiac chambers are an infrequent anomaly and usually are found casually. Although the majority of patients are asymptomatic, in rare cases it may cause coronary steal and cardiac ischemia. We present a patient with a left anterior descending coronary artery with multiple small fistulas to the left ventricle that suffered angina and an episode of ventricular fibrillation that required electrical cardioversion and an intracardiac defibrillator.     

冠状动脉瘘的CT血管成像表现

目的 分析冠状动脉瘘的CT血管成像表现.方法 回顾性分析12717例行冠状动脉多排螺旋CT血管成像检查病例,其中诊断冠状动脉瘘66例.综合多种后处理图像,记录冠状动脉瘘的起源、瘘口、冠状动脉有无斑块及狭窄.其中14例患者行冠状动脉造影,比较两种检查方法结果的异同.结果 66例冠状动脉瘘中,起源于双侧冠状动脉21例,左冠状动脉26例,右冠状动脉19例;而瘘口在肺动脉41例,左心房10例,右心房8例,左心室4例,冠状静脉2例,右心室1例.14例患者冠状动脉造影显示的瘘管起源和瘘口部位均与多排螺旋CT血管成像一致.31例冠状动脉瘘同时伴冠状动脉斑块形成,其中狭窄程度≥50%者7例.结论 双侧冠状动脉起源的冠状动脉瘘并不少见,且瘘口在肺动脉者最多.CT血管成像可作为冠状动脉瘘的首选检查手段,可为该病的治疗提供重要的术前信息.

Abstract：

Objective To analyzed the computed tomography angiography (CTA) features of the coronary artery fistulas. Methods Sixty-six coronary artery fistulas were diagnosed out of 12 717 patients underwent the coronary artery multiple detector CTA examination. The origin and drainage site of the coronary artery fistulas and the plaque and stenosis of the coronary artery were observed by post-processing analysis on various images. Coronary artery angiography was performed in 14 out of 66 coronary artery fistulas patients. Results Coronary artery fistulas arose from bilateral coronary artery system in 21 cases, from left coronary artery in 26 cases and from right coronary artery in 19 cases. The majority of coronary artery fistulas entered into pulmonary artery (41 cases). The rest drainage sites included left atrium (10 cases), right atrium (8 cases),left ventricle (4 cases), coronary sinus (2 cases) and right ventricle (1 case). The findings of CTA and coronary artery angiography were consistent in 14 patients with DSA examination. Coronary artery plagues were evidenced in 31 cases and stenosis was greater than 50% in 7 coronary artery fistulas patients. Conclusions Multiple coronary artery fistulas are not rare, and pulmonary artery is the most frequent drainage site. When suspecting the coronary artery fistulas, coronary artery CTA can be the first choice of diagnose. CTA can supply adequate information for therapy.     

Coronary artery fistulas: clinical and therapeutic considerations

Coronary artery fistulas vary widely in their morphological appearance and presentation. These fistulas are congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel, or other structure, bypassing the myocardial capillary network. The majority of these fistulas arise from the right coronary artery and the left anterior descending coronary artery; the circumflex coronary artery is rarely involved. Clinical manifestations vary considerably and the long-term outcome is not fully known. The patients with coronary fistulas may present with dyspnea, congestive heart failure, angina, endocarditis, arrhythmias, or myocardial infarction. A continuous murmur is often present and is highly suggestive of a coronary artery fistula. Differential diagnosis includes persistent ductus arteriosus, pulmonary arteriovenous fistula, ruptured sinus of Valsalva aneurysm, aortopulmonary window, prolapse of the right aortic cusp with a supracristal ventricular septal defect, internal mammary artery to pulmonary artery fistula, and systemic arteriovenous fistula. Although noninvasive imaging may facilitate the diagnosis and identification of the origin and insertion of coronary artery fistulas, cardiac catheterization and coronary angiography is necessary for the precise delineation of coronary anatomy, for assessment of hemodynamics, and to show the presence of concomitant atherosclerosis and other structural anomalies. Treatment is advocated for symptomatic patients and for those asymptomatic patients who are at risk for future complications. Possible therapeutic options include surgical correction and transcatheter embolization. Historical perspectives, demographics, clinical presentations, diagnostic evaluation, and management of coronary artery fistula are elaborated.     

Coronary artery to left ventricular fistula demonstrated by transthoracic echocardiography

Coronary artery fistulas are rare coronary anomalies which generallyrequire coronary angiography for definitive diagnosis. Improvementsin ultrasound technology has enabled direct, transthoracic visualizationof long portions of coronary arteries. We report a patient witha symptomatic coronary to left ventricular fistula, which wasdiagnosed with transthoracic echocardiography.     

Congenital coronary artery fistulas in adults: surgical treatment and outcome

BACKGROUND: Congenital coronary artery fistulas, a subgroup of anomalies of the coronary arteries, are an extremely rare cardiac defect. Most patients are asymptomatic, and if symptoms are presented, they depend on the underlying anatomy. Knowledge of those fistulas is important for prognosis and management. METHODS: Thirteen adult patients with congenital coronary fistulas (8 male, 5 female) were operated in our department during the last decade (1990-1999). Mean age was 61.5+/-10.8 years. Diagnosis was made by coronary angiography, and 15 congenital coronary artery fistulas were found. RESULTS: All patients were symptomatic with clinical symptoms depending on the associated cardiac disorder. Coronary artery fistulas originated from the proximal left descending artery (n=10), left main stem (n=3), circumflex artery (n=1), right coronary artery (n=1), and drained into the main pulmonary artery (n=14) and left ventricle (n=1). Nine fistulas (60%) were interrupted on the outside of the heart, and six fistulas (40%) were closed through the opened pulmonary artery. There was no surgical death and no fistula-related complication. CONCLUSIONS: Surgical closure of congenital coronary artery fistulas in adults can be performed with a very low risk, and closure is recommended to prevent complications.     

Coronary Artery Fistulas: A Review of the Literature and Presentation of Two Cases of Coronary Fistulas with Drainage into the Left Atrium

We report 2 cases of infants presenting with a murmur shortly after birth and diagnosed with coronary artery fistulas with drainage into the left atrium. The first infant had a fistulous communication between the left main coronary artery and the left atrial appendage and presented with signs and symptoms of heart failure. The infant was repaired surgically in the first week of life. The second infant was asymptomatic and had a fistulous communication between the right coronary artery and the left atrium. The infant will have the fistula closed in the cardiac catheterization laboratory when the child is older. The literature on coronary artery fistulas is reviewed, and the diagnosis and management of coronary artery fistulas is discussed.     

Acquired coronary artery fistula after open heart surgery for congenital heart disease

BACKGROUND: Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported. METHODS: From 1998 to 2003, 10 patients with coronary artery fistula detected by echocardiography after open heart surgery for congenital heart disease were included. Their ages ranged from 2 months to 41 years (median 4.2 years). The underlying heart disease was tetralogy of Fallot in five patients, ventricular septal defect in three, double chamber right ventricle in one, and transposition of the great arteries with ventricular septal defect in the remaining one. RESULTS: Of these 10 patients, the coronary artery fistula originated from the left coronary artery in four, right coronary artery in two, and unknown origin in the remaining four. The coronary artery fistula drained into the right ventricle in nine and into the left ventricle in the remaining one. The incidence of acquired coronary artery fistula after open heart surgery for congenital heart disease was 0.44% (8/1832). The identified risk factors for acquired coronary artery fistula were reoperation and right ventricular muscle resection in ventricular septal defect. After follow-up for 0.5-12 years (mean 4.1+/-3.3 years), the coronary artery fistula persisted, but neither symptoms nor significant left-to-right shunt was noted. CONCLUSIONS: Acquired coronary artery fistula is a rare complication after cardiac surgery. Reoperation and resection of right ventricular hypertrophic muscle increase the risk of this complication. Although shunt flow did not increase during follow-up, the significance of acquired coronary artery fistula needs further investigation.     

Coronary artery fistulas in pulmonary atresia and ventricular septal defect

Pulmonary atresia with ventricular septal defect is an anomaly with highly variable anatomy. Rarely, a coronary artery-to-pulmonary artery fistula may contribute to pulmonary blood flow. Since 1996, we have treated 4 patients with coronary-pulmonary fistula associated with pulmonary atresia and ventricular septal defect. Two fistulas originated from the left coronary, one from the right coronary, and one from a right-sided solitary coronary system. All terminated in the main pulmonary artery, which was adequate in all cases. The fistulas were managed by direct internal closure. Total intracardiac repair was then accomplished in all patients at the same sitting. There was one death. In children with favorable anatomy, direct closure of the fistula from the pulmonary artery is adequate and allows single-stage intracardiac repair.     

Assessment of congenital coronary artery fistulas by transesophageal color Doppler echocardiography

PURPOSE: Coronary angiography is the gold standard for imaging the coronary tree, but the relation of coronary artery fistulas to other structures, and their origin and course, may not be apparent. We evaluated the ability of multiplane color Doppler transesophageal echocardiography to identify coronary fistulas. PATIENTS AND METHODS: Twenty-one patients with angiographically confirmed coronary artery fistulas were investigated by transesophageal echocardiography in four Italian hospitals between January 1997 and May 2001. RESULTS: Transesophageal echocardiography correctly diagnosed fistulous connection in all 21 patients. This included 6 patients with connections from the left circumflex artery (into the right chambers of the heart in 5 patients, and into the left ventricle in 1 patient), 10 patients with a fistula arising from the left anterior descending artery or left main coronary artery (with drainage into the right ventricle or main pulmonary artery), and 5 patients with a fistula from the right coronary artery (with drainage sites in the lateral aspect of the right ventricle, the low posterior right atrium, or the superior vena cava). In 4 of the 21 patients, angiography did not identify the precise site of a fistula into the coronary sinus or right ventricle. CONCLUSION: Color Doppler transesophageal echocardiography is useful in the diagnosis and in the precise localization of coronary artery fistulas.     

Cardiac stab wound resulting in a left anterior descending artery to left ventricular fistula with delayed pericardial tamponade

We describe the third known case of a traumatic left anterior descending artery to left ventricular fistula. Emergency operative repair was required on day 7 for delayed pericardial tamponade. The significant incidence of late serious sequelae with traumatic coronary artery fistulas suggests that early surgery is warranted.