Demonstration of coronary-to-pulmonary fistula with MDCT and conventional angiography

The coronary pulmonary fistulas are usually discovered incidentally during routine cardiac catheterization after the development of atherosclerotic coronary artery disease. Here we present a 59-year-old man with anginal symptoms who was found to have two fistulas originating from the left coronary system and right coronary sinus and draining into the pulmonary trunk. We delineated the courses of the fistulas in detail using both of the imaging techniques of MDCT and conventional angiograhy.     

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.     

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。     

Coronary to pulmonary artery fistula--diagnosis by transesophageal echocardiography

Coronary artery fistulas (CAFs) are rare coronary anomalies which generally require coronary angiography for definitive diagnosis. CAFs most commonly drain into the right atrium or right ventricle, but occasionally can drain into the pulmonary artery. We report the case of an asymptomatic coronary to pulmonary artery fistula, which could only be definitively diagnosed with transesophageal 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.     

Myocardial ischemia secondary to a large coronary-pulmonary fistula--a case report

Coronary-pulmonary fistulas are rare. The majority of these fistulas arise from the left anterior descending or the right coronary arteries; the circumflex coronary artery is rarely involved. The majority of patients are asymptomatic, but heart failure, angina, myocardial infarction, endocarditis, and dyspnea have rarely been reported. The management is controversial and recommendations are based on anecdotal cases or very small retrospective series. A case of a 62-year-old female is reported who presented with chest pain and was found to have myocardial ischemia on SPECT sestamibi. Cardiac catheterization revealed no obstructive coronary artery disease and a large coronary pulmonary fistula communicating from the left circumflex coronary artery to the left pulmonary artery.     

Multiple coronary artery fistulas with a huge right coronary artery showing exacerbation during 16 years of follow-up.

A 38-year-old Japanese woman underwent cardiac evaluation to assess an arrhythmia detected after the delivery of her fourth baby. At the age of 22 years, she had undergone cardiac evaluation, including catheter examination, because of a heart murmur. Coronary angiography showed a huge right coronary artery (RCA) draining to the right atrium and a normal left coronary artery. Because the left to right shunt ratio was trivial and the patient was asymptomatic, conservative observation was undertaken. On the current admission to hospital, there was a grade III continuous murmur at the second left sternal border. The patient underwent right and left cardiac catheter examination in March 2000. Selective coronary angiography demonstrated an enlarged lumen of the RCA, an enlarged aneurysmal cavity in the terminal portion of the RCA, and the postero-descending artery being filled by collateral circulation from the left coronary artery. Moreover, 2 new fistulas had appeared from the left coronary system. An oxygen saturation study showed that the pulmonary to somatic flow ratio (Qp/Qs) was 2.2. The patient underwent surgical treatment in July 2000 and on coronary angiography 1 month later, there was no abnormal shunt flow from either coronary artery into the aneurysmal cavity, although the RCA was still enlarged.     

Bilateral coronary artery to left ventricular fistulas

Congenital coronary artery fistulas are important coronary artery anomalies. Bilateral coronary artery fistulas are very rare and coronary artery to left ventricular communications are even more infrequent. This report describes a patient with bilateral right and left coronary artery fistulas draining into a common channel and emptying into the left ventricular cavity.     

Bilateral coronary artery fistulas communicating with main pulmonary artery and left ventricle: case report

Coronary artery fistula is an uncommon congenital malformation that generally drains into the main pulmonary artery or the right side of the heart. This is a case report on bilateral coronary artery fistulas communicating with the main pulmonary artery and the left ventricle. A 65-year-old woman was investigated for recurrent chest pain. Coronary angiography revealed this anomalous coronary artery connection without evidence of atherosclerotic coronary artery disease. The rarity of coronary artery fistulas involving both the main pulmonary artery and the left ventricle is emphasized.     

Right coronary artery hepatic vein fistula: a case report

There is a slight predominance for coronary artery fistulas that involve the right coronary artery, while multiple fistulas have also been reported. The usual site of termination is one or more of the low-pressure structures in the heart or the great vessels such as the right or left atria, right ventricle, coronary sinus, pulmonary artery, or superior vena cava. However, a coronary fistula that drains into a hepatic vein has not been reported in the literature. Therefore, this is the first case report indicating a right coronary artery fistula that drains into the middle hepatic vein.     

Successful Double Percutaneous Alcohol and Coil Embolization of Bilateral Coronary-to-Pulmonary Artery Fistulas

We report the case of a 70-year-old man with recent myocardial infarction who was admitted for further evaluation of his effort angina. Cardiac catheterization and selective coronary angiography excluded significant coronary atherosclerotic disease of the coronary arteries. Double coronary-to-pulmonary artery fistulas, one originating from the proximal right, the other from the left anterior descending coronary arteries, and draining into the right and left branch of the pulmonary artery, respectively, were observed. A left-to-right shunt was visualized at angiography. The patient was successfully treated with percutaneous alcohol and coil embolization of both coronary artery fistulas with total resolution of clinical symptoms. At 6-month follow-up a coronary angiography confirmed complete disappearance of the fistulas. We conclude that a "coronary steal" phenomenon caused by the fistulas induced myocardial ischemia in this patient and that percutaneous transcatheter exclusion with chemical and mechanical devices is a safe. effective, and reasonable alternative to traditional cardiac surgery.     

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.     

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.     

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.     

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

BACKGROUND: Transcatheter closure of coronary artery fistulas has emerged as a successful alternative to surgery. We describe various techniques and short-term findings in 15 patients who were taken up for transcatheter closure of these fistulas. METHODS AND RESULTS: Fifteen patients (aged 2-55 years; 12 males) with coronary artery fistulas underwent percutaneous transcatheter closure between June 1997 and December 2002. Site of origin of these fistulas were: right coronary artery in 7, left anterior descending coronary artery in 4, left main coronary artery in 2 and left circumflex coronary artery in 2 patients. Drainage site of these fistulas were: right ventricle in 9, right atrium in 4 and pulmonary artery in 2 patients. Out of these 15 fistulas, 14 were congenital and one was iatrogenically produced following inadvertent cutting balloon angioplasty of a septal perforator in a patient with chronic total occlusion of left anterior descending coronary artery. Various occlusion devices used to close these fistulas were: conventional metallic coils in 10, floppy tips of coronary angioplasty guidewires in 2, Amplatzer duct occluder in 1 and Amplatzer septal occluder in 2 patients. One of our patients had a coronary artery fistula draining by two openings into the right atrium, both of which were successfully closed using 2 Amplatzer duct occluders. Check angiogram after the procedure revealed complete occlusion in 13 (86.6%) and small residual flow in 2 patients. Follow-up studies at 3-55 months (mean 18 months) showed complete abolition of shunt in all patients with no evidence of recanalization leading to recurrence of shunt. CONCLUSION: Transcatheter closure of coronary artery fistulas is feasible and safe in the anatomically suitable vessels. Use of floppy tips of coronary angioplasty guidewires reduces the cost of the procedure significantly. which is an important consideration in developing countries like India.     

Rare case of coronary to pulmonary vein fistula with coronary steal phenomenon

Coronary artery fistulas are abnormal connections between coronary artery territories and cardiac chambers or major vessels,most of them are congenital.Patients with coronary artery fistula can be asymptomatic or present with different symptoms like angina.Cardiac computed tomography(CT)is one of the best modalities for diagnosis.We present an elderly patient that presented with angina symptoms,non invasive stress test was positive for ischemic heart disease,coronary angiogram could not reveal any obstructive lesions,but an abnormal branch of the left descending coronary artery(LAD),cardiac CT showed fistula that connect left anterior descending coronary artery to left superior pulmonary vein.Our case is extremely rare as most of the reported cases were fistulas between LAD and pulmonary artery,but in our case the fistula between LAD and left superior pulmonary vein.In addition,our patients’symptoms resolved with anti-ischemic medical treatment without any surgical intervention.     

Closure of a coronary fistula with a transcatheter implantable coil

Large sized coronary artery fistulas are rare and diagnosed in only 0.05% of adult catheterized patients. Only a minority of these fistulas are operated upon. We describe a percutaneous technique to close a left coronary artery fistula draining into the right atrium in a 30-yr-old male patient. The fistula was closed by implantation of a trefoil coil, inserted through a catheter selectively advanced into the fistula.     

Closure of a coronary fistula with a transcatheter implantable coil.

Large sized coronary artery fistulas are rare and diagnosed in only 0.05% of adult catheterized patients. Only a minority of these fistulas are operated upon. We describe a percutaneous technique to close a left coronary artery fistula draining into the right atrium in a 30-yr-old male patient. The fistula was closed by implantation of a trefoil coil, inserted through a catheter selectively advanced into the fistula.