Evaluation of coronary artery fistula using a color Doppler flow imaging technique

Two unusual cases with coronary artery fistula were reported. The diagnoses were first made by color Doppler flow imaging and confirmed by selective coronary angiography and operation. In the first case, color Doppler revealed a continuous flow signal within the dilated left coronary artery and a turbulent jet within the right atrium. In the second case, color Doppler showed turbulent flow signals which could be traced from the proximal left coronary artery, along the left ventricular posterior wall and into the left ventricle. The high velocities at the site of drainage of fistula were recorded by the continuous-wave Doppler and the calculated pressure gradient confirmed that the abnormal flow was from the systemic circulation. We are of the opinion that color Doppler flow imaging is a reliable technique for detecting coronary artery fistula and the combination of color Doppler with continuous-wave Doppler is essential for evaluating the hemodynamics of the shunt flow.     

Doppler echocardiographic features of coronary arteriovenous fistula. Complementary roles of cross sectional echocardiography and the Doppler technique.

The Doppler echocardiographic features of coronary arteriovenous fistula were investigated in eight patients with left or right coronary arteriovenous fistulas who had a continuous heart murmur in the upper precordial area and whose diagnoses were confirmed by coronary angiography. In four patients the dilated lumen of the coronary arteriovenous fistula was visualised by cross sectional echocardiography. Of these, three showed abnormal unidirectional continuous flow signals with broad velocity spectra in the fistula. Abnormal, powerful, unidirectional or bidirectional continuous Doppler signals were detected in part of the pulmonary artery in two of the eight patients, in part of the right ventricle in two, and in part of the right atrium in one; these signals were interpreted as indicating shunt flow. Although the opening of the fistula was difficult to visualise by cross sectional echocardiography, the pulsed Doppler technique helped identify the site in patients with dilatation of the coronary artery. In the remaining three patients with a small shunt no abnormal findings were obtained with cross sectional echocardiography or the Doppler technique. The size of the fistula below which no abnormal findings may be obtained by Doppler echocardiography still needs to be determined.     

Doppler echocardiographic features of coronary arteriovenous fistula. Complementary roles of cross sectional echocardiography and the Doppler technique

The Doppler echocardiographic features of coronary arteriovenous fistula were investigated in eight patients with left or right coronary arteriovenous fistulas who had a continuous heart murmur in the upper precordial area and whose diagnoses were confirmed by coronary angiography. In four patients the dilated lumen of the coronary arteriovenous fistula was visualised by cross sectional echocardiography. Of these, three showed abnormal unidirectional continuous flow signals with broad velocity spectra in the fistula. Abnormal, powerful, unidirectional or bidirectional continuous Doppler signals were detected in part of the pulmonary artery in two of the eight patients, in part of the right ventricle in two, and in part of the right atrium in one; these signals were interpreted as indicating shunt flow. Although the opening of the fistula was difficult to visualise by cross sectional echocardiography, the pulsed Doppler technique helped identify the site in patients with dilatation of the coronary artery. In the remaining three patients with a small shunt no abnormal findings were obtained with cross sectional echocardiography or the Doppler technique. The size of the fistula below which no abnormal findings may be obtained by Doppler echocardiography still needs to be determined.     

Prenatal diagnosis of fetal isolated right coronary artery to left ventricle fistula

Coronary artery fistula (CAF) is a rare malformation and is seldom reported during pregnancy. Right coronary artery fistula commonly drains into the right ventricle, right atrium, or pulmonary artery. We describe here a rare case of fetal CAF draining into the left ventricle using cross‐sectional and color Doppler echocardiography. We also summarized our experience in the diagnosis of this uncommon malformation, in which tracing the origin, course, and outlet of the abnormal intra‐cardiac flow played a key role.     

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.     

Recognition of abnormal connections of coronary arteries with the use of Doppler color flow mapping

Anomalous connection of a coronary artery to a ventricle or pulmonary artery causes shunting of blood from the coronary circuit and may produce myocardial ischemia. Such a coronary anomaly may occur in isolation or with other defects. Doppler color flow mapping and two-dimensional echocardiography were used to diagnose anomalous coronary connections in 13 patients, 1 day to 7 years of age, over a 1 year period. The diagnoses were anomalous origin of the left coronary artery from the pulmonary trunk in five patients, a coronary artery to left ventricle fistula or coronary artery to pulmonary artery fistula in four patients with other complex defects, right ventricular sinusoids in two patients with pulmonary atresia and intact ventricular septum and an isolated coronary artery fistula in two patients. In all cases, the abnormal coronary connection was recognized on the basis of an abnormal, continuous or to and fro flow pattern in the fistula and its connections as demonstrated by scanning in multiple views with Doppler color flow mapping. The low spatial resolution of Doppler color flow mapping limits the anatomic detail available; nonetheless, it is a significant advance in the noninvasive diagnosis of abnormal coronary connections.     

Noninvasive detection of a coronary arteriovenous fistula by two-dimensional color Doppler echocardiography

The diagnosis of a left circumflex coronary artery--coronary sinus fistula draining into the right atrium was confirmed by echocardiographic analysis. Two-dimensional echocardiography revealed a dilatation of the left main coronary artery, the left circumflex artery, the coronary sinus and a fistulous communication. Pulsed Doppler echocardiographic study showed a turbulent, continuous flow within the dilated coronary artery, in the coronary sinus and at the site of its drainage into the right atrium. There was a diastolic flow-reversal in the descending aorta in absence of aortic insufficiency. Color Doppler echocardiography enhanced the finding of the correct diagnosis by simultaneously displaying blood flow within various parts of the heart and the coronary vessels. This technique aided in establishing the exact location of the fistula by demonstrating a turbulent blood flow, even in narrow cardiac structures. The echocardiographic diagnosis was confirmed by coronary angiography.     

E-Flow血流显像技术对胎儿冠状动脉瘘的诊断价值

目的探讨E-Flow血流显像技术对胎儿冠状动脉瘘的诊断价值。方法在胎儿超声心动图检查中除使用常规彩色多普勒外加用了E-Flow血流显像技术,产前诊断冠状动脉瘘5例。使用Alokaα10彩色超声诊断仪,探头频率为3.5～5.0 MHz。对二维超声(2D)发现胎儿一侧心房、心室异常增大或冠状动脉增粗的患者,进一步探查冠状动脉。先以彩色多普勒超声(CDFI)获取心腔内异常血流,再以连续多普勒(CW)获取舒张期为主的高速血流频谱,初步证实该异常血流来自于冠状动脉。此时启动E-Flow显像模式追溯该异常血流。结果 5例胎儿冠状动脉瘘均出生后经超声证实。其中2例为左冠状动脉右心室瘘、1例为左冠状动脉右心房瘘,1例为右冠状动脉右心房瘘,1例为右冠状动脉右心室瘘。E-Flow显像技术能够敏感地捕捉冠状动脉瘘血流,清晰显示冠状动脉瘘的起源、瘘管的走行以及瘘口的位置、大小,其血流显像优于传统的彩色多普勒。结论应用E-Flow显像技术可以在产前更加准确、快速地诊断胎儿冠状动脉瘘。推荐E-Flow血流显像技术成为胎儿冠状动脉瘘的诊断工具之一。     

Diagnosis of anomalous left coronary artery by Doppler color flow mapping: distinction from other causes of dilated cardiomyopathy.

BACKGROUND. Anomalous origin of the left coronary artery from the pulmonary trunk is difficult to diagnose reliably by two-dimensional echocardiography. Therefore, Doppler color flow mapping was tested in 29 patients with dilated cardiomyopathy or anomalous left coronary artery, or both. METHODS AND RESULTS. All patients with anomalous left coronary artery (10 patients) or dilated cardiomyopathy (27 patients) (excluding those with other known causes for cardiomyopathy) examined between January 1988 and May 1991 were identified. The direction of flow in the three main segments of the left coronary system was determined by Doppler color flow mapping. In all 10 patients with anomalous left coronary artery, flow mapping demonstrated an abnormal jet from the left coronary artery into the pulmonary trunk and retrograde flow in at least two segments of the left coronary system. The diagnosis was confirmed in all 10 patients at operation. Doppler color flow mapping, performed in 19 of the 27 patients with dilated cardiomyopathy, demonstrated anterograde flow in at least one segment of the left coronary system in 16 of the 19 patients; flow direction was not determined in the other 3 patients. Coronary artery anatomy was confirmed by aortic root or left ventricular angiography in 14 patients and at autopsy in 1 patient and was not directly confirmed in 4 patients. Left ventricular function spontaneously improved to normal in three of the latter four patients, a clinical course not consistent with anomalous left coronary artery. The left coronary artery appeared to arise from the aortic root by two-dimensional echocardiographic imaging alone in all patients with dilated cardiomyopathy and in 5 of 10 patients with anomalous left coronary artery (50% false negative diagnoses). CONCLUSIONS. Detection of an abnormal jet into the pulmonary trunk and retrograde flow in the left coronary system by Doppler color flow mapping is reliable for diagnosing anomalous left coronary artery whereas two-dimensional echocardiographic imaging alone is often inconclusive or misleading. Determining flow direction in the left coronary system in patients with dilated cardiomyopathy is useful for excluding anomalous left coronary artery but is technically more difficult to document in this condition than in anomalous left coronary artery.     

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-cameral fistula in association with intracardiac tumour and aortic stenosis in an adult

We present a patient with the unexpected association of left ventricular tumor, a fistula between the right coronary and the right atrium, and senile valvar aortic stenosis. He had anginal complaints. Doppler echocardiography revealed moderate aortic stenosis with mild aortic and moderate mitral regurgitation. A tumour was detected in the left ventricle. Selective coronary angiography disclosed normal anatomy with a fistula originating from the proximal right coronary artery and draining into the right atrium. He refused operative treatment and is still alive, 1 year after the diagnosis was made, without complications.     

Single Coronary Artery with Right Ventricular Fistula: Case Report and Literature Review

We report a rare case of a 6-year-old boy who was diagnosed with coronary artery fistulae communicating with the right ventricle and a left single coronary artery. Preoperative angiography showed a dilated and tortuous single coronary artery draining into the right ventricle. Two coronary artery fistulae draining into the right ventricle were detected at operation and both of these were ligated. Postoperative angiography showed that the single coronary artery diameter was almost normalized, although the vessel was still slightly tortuous. His clinical course was uneventful. In this report, we summarize cases of coronary artery fistula with single coronary artery that have been reported in the literature as well as our case.     

Assessment of Coronary Artery Fistula by Color Doppler Echocardiography

Two patients with coronary artery fistula (CAF) were studied by color Doppler echocardiography, left coronary artery-right ventricle fistula in a child and right coronary artery-left ventricle fistula in an adult. Diagnosis was made by transthoracic echocardiography (TTE) and one adult by multiplane transesophageal echocardiography (TEE), respectively. The proximal dilated coronary artery, the course, and the drainage sites were demonstrated with color Doppler echocardiography in both patients.     

Diagnosis of coronary artery fistula by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging

Ten consecutive patients with a coronary artery fistula, aged 1 day to 4 years, were studied by two-dimensional echocardiography, pulsed Doppler ultrasound and color flow imaging. All patients underwent cardiac catheterization, and seven patients had surgical closure of the fistula. The origin, course and site of drainage of the coronary artery fistula were correctly identified prospectively by echocardiographic examination in all patients. Color flow imaging was particularly helpful in visualizing the site of drainage of the fistula. Diameters of the right and left coronary arteries at their origin and of the aortic root were measured from two-dimensional echocardiographic frames and compared with measurements obtained in normal children. The ratio of coronary artery diameter to aortic root diameter in normal children was 0.14 +/- 0.03 (mean +/- SD) for the right coronary artery and 0.17 +/- 0.03 for the left coronary artery. These normal ratios were greatly exceeded for coronary arteries feeding the fistula, and ranged from 0.68 to 0.84 for the right coronary artery and from 0.34 to 0.52 for the left coronary artery. All anatomic information needed for surgical treatment of coronary artery fistula was consistently obtained by echocardiography with color flow imaging. The fistula was closed from within the heart in five patients and by ligation from the epicardial surface in two patients. In these latter patients, intraoperative color flow imaging at the time of ligation proved to be extremely valuable in achieving complete closure.     

Multiple coronary artery fistulae communicating through a sinusoid and emptying into the left ventricle

This case report describes the occurrence of multiple coronary artery fistulae emptying into the left ventricle and includes a small communication into the left atrium. The initial diagnosis of a coronary artery fistula was made by standard and nonstandard transthoracic two-dimensional echocardiogram and Doppler interrogation. Later, multiple coronary fistulae communicating through a sinusoid and draining into the left-sided chambers were confirmed by angiography.     

Right coronary artery coronary sinus fistula with coronary sinus ostium stenosis

Coronary artery fistula (CAF) is a rare anomaly of the coronary artery. The draining site of a right coronary artery (RCA) fistula may usually be the right ventricle, right atrium, or pulmonary artery. Here, we present a patient with right coronary artery to coronary sinus fistula (RCACSF) complicated by aneurysmal dilatation of the coronary sinus (CS) and stenosis of CS ostium.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Pre- and Postoperative Echocardiography Findings in Three Unusual Cases

Diagnosis of anomalous left coronary artery from the pulmonary artery was prospectively established by color Doppler echocardiography in three patients. In two asymptomatic girls, aged 10 years and 5 years respectively, referred for evaluation of a murmur with normal ECG and chest X ray, two-dimensional and pulsed-Doppler examination showed no intracardiac abnormalities. Color flow mapping detected flow in a dilated right coronary artery and left coronary artery to pulmonary artery shunting. Color guided pulsed-Doppler examination permitted further evaluation of coronary flow. Another child had a dilated cardiomyopathy with an echo-dense anterolateral papillary muscle and mitral insufficiency. Postoperative echoes after subclavian to left coronary artery anastomosis and ligation of the left coronary artery at its origin showed residual high-velocity shunting resulting in reoperation in one case and a moderate supravalvular pulmonic stenosis in another. These findings further emphasize the benefit of color Doppler echocardiography in the pre- and postoperative evaluation of anomalous left coronary artery. (ECHOCARDIOGRAPHY, Volume 8, September 1991)     

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.     

Non-invasive diagnosis of a right coronary artery-right ventricle fistula by Doppler echocardiography]

A six-year-old girl with an atypical continuous precordial murmur, was suspected of having a coronary arteriovenous fistula. A markedly dilated right coronary artery was revealed by two-dimensional echocardiography. The pulsed Doppler examination showed a diastolic retrograde flow in the ascending aorta, with a normal flow in the left ventricular outflow tract. With the sample volume in the right ventricle a continuous turbulent flow was observed. Color flow mapping showed a turbulent, systolic-diastolic flow in the right ventricle. The fistula was confirmed by cardiac catheterization.     

Postoperative iatrogenic left coronaroventricular fistula. Demonstration by color two-dimensional Doppler

The authors report the case of an asymptomatic 67 year old patient, in whom, 6 years after aortic valve replacement, Doppler color flow mapping showed the presence of a coronary artery--left ventricular fistula. The normality of preoperative coronary angiography suggested that this fistula was created during peroperative left ventricular purging: the implantation of a needle through the right ventricle and interventricular septum. A iatrogenic lesion of a septal branch probably caused the communication between the left anterior descending artery and the left ventricle. Postoperative normalisation of the left ventricular end diastolic dimension, the absence of dilatation of the left main coronary on 2D echocardiography, the narrowness of the Doppler color jet and the absence of a significant end diastolic Doppler signal in the aortic isthmus indicated a fistula of small size and simple Doppler echocardiographic follow-up was decided upon.