Circumflex Coronary Artery Fistulae Draining into Right Atrium: A Case Report

Coronary artery fistula (CAF) is an anomaly making a coronary artery communicate with a heart cavity or a great vessel, thus bypassing the myocardial capillary bed. CAF is frequently diagnosed as an incidental finding. Herein, we present the case of a 4-year-old boy. He was referred for a pediatric cardiology assessment due to a continuous murmur at the middle sternal border. Echocardiogram showed dilated left coronary artery and an abnormal diastolic flow in the right atrium. The right-sided chambers were slightly enlarged, but ventricular contractility was normal. CAF was suspected. Angiography and CT confirmed the diagnosis of coronary fistula from the circumflex coronary artery to the right atrium. Successful transcatheter closure with an Amplatzer PiccoloOccluder was performed with complete occlusion.

Current characteristics of congenital coronary artery fistulas in adults:A decade of global experience

AIM:To describe the characteristics of coronary artery fistulas(CAFs) in adults,including donor vessels and whether termination was cameral or vascular. METHODS:A PubMed search was performed for articles between 2000 and 2010 to describe the current characteristics of congenital CAFs in adults.A group of 304 adults was collected.Clinical data,presentations,diagnostic modalities,angiographic fistula findings and treatment strategies were gathered and analyzed.With regard to CAF origin,the subjects were tabulated into unilateral,bilateral or multilateral fistulas and compared.The group was stratified into two major subsets according to the mode of termination;coronary-cameral fistulas(CCFs) and coronary-vascular fistulas(CVFs) . A comparison was made between the two subsets. Fistula-related major complications[aneurysm formation,infective endocarditis(IE) ,myocardial infarction(MI) ,rupture,pericardial effusion(PE) and tamponade] were described.Coronary artery-ventricular multiple micro-fistulas and acquired CAFs were excluded as well as anomalous origin of the coronary arteries from the pulmonary artery(PA) . RESULTS:A total of 304 adult subjects(47%male) with congenital CAFs were included.The mean age was51.4 years(range,18-86 years) ,with 20%older than 65 years of age.Dyspnea(31%) ,chest pain(23%) and angina pectoris(21%) were the prevalent clinical presentations.Continuous cardiac murmur was heard in 82%of the subjects.Of the applied diagnostic modalities,chest X-ray showed an abnormal shadow in 4%of the subjects.The cornerstone in establishing the diagnosis was echocardiography(68%) ,and conventional contrast coronary angiography(97%) .However,multislice detector computed tomography was performed in 16%.The unilateral fistula originated from the left in 69%and from the right coronary artery in 31%of the subjects.Most patients(80%) had unilateral fistulas,18%presented with bilateral fistulas and 2%with multilateral fistulas.Termination into the PA was reported in unilateral(44%) ,bilateral(73%) and multilateral(75%) fistulas.Fistulas with multiple origins(bilateral and multilateral) terminated more frequently into the PA(29%) than into other sites(10.6%)(P=0.000) . Aneurysmal formation was found in 14%of all subjects.Spontaneous rupture,PE and tamponade were reported in 2%of all subjects.In CCFs,the mean age was 46.2 years whereas in CVFs mean age was 55.6 years(P=0.003) .IE(4%) was exclusively associated with CCFs,while MI(2%) was only found in subjects with CVFs.Surgical ligation was frequently chosen for unilateral(57%) ,bilateral(51%) and multilateral fistulas(66%) ,but percutaneous therapeutic embolization(PTE) was increasingly reported(23%,17%and 17%,respectively) . CONCLUSION:Congenital CAFs are currently detected in elderly patients.Bilateral fistulas are more frequently reported and PTE is more frequently applied as a therapeutic strategy in adults.

Congenital solitary coronary artery fistulas characterized by their drainage sites

Last centuries have witnessed tremendous sophistication and progress in the detection,diagnosis and treatment of coronary artery fistulas(CAFs).In many countries,CAFs were reported to be visualized and treated using several imaging techniques and different management strategies.Reports from nearly all continents of the globe have contributed to the description of CAFs,not only in Asia and Europe but also throughout North and Latin America.However,these reports have to be cautiously analyzed as many of them were published as a case report and careful interpretation is warranted due to possible publication bias.A literature search was performed using PubMed search interface to select papers dealing with congenital CAFs in adult population between 2000-2009.A total of 233 subjects were collected,and analysed according to their drainage site and treatment modality.They were divided into two subgroups:percutaneous transluminal embolization group(PTE group,n=122)and surgical ligation group(SL group,n=111).In the SL group,atherosclerotic coronary artery disease(19%)and associated congenital lesions(23%)were more prevalent compared with the PTE group(9%and 8%),respectively.Infective endocarditis was more frequently seen in the SL group besides syncope,congestive heart failure and hemopericardium.In both groups multimodality diagnostic workup composed of several non-invasive and invasive imaging techniques for fistula visualization were performed and drainage sites into the different cardiac chambers and intrathoracic great vessels were similarly distributed in the two groups.

Incidental congenital coronary artery vascular fistulas in adults:Evaluation with adenosine-13N-ammonia PET-CT

AIM To assess the functionality of congenital coronary artery fistulas(CAFs) using adenosine stress ^(13)N-ammonia positron emission tomography computed tomography(PET-CT).METHODS Congenital CAFs were incidentally detected during coronary angiography(CAG) procedures in 11 adult patients(six males and five females) with a mean age of 64.3 years(range 41-81). Patients were collected from three institutes in the Netherlands. The characteristics of the fistulas(origin, pathway and termination), multiplicity of the origins and pathways of the fistulous vessels were assessed by CAG. Five patients underwent adenosine pharmacologic stress ^(13)N-ammonia PET-CT to assess myocardial perfusion and the functional behavior of the fistula. RESULTS Eleven patients with 12 CAFs, 10 unilateral and one bilateral, originating from the left anterior descending coronary artery(n = 8), right coronary artery(n = 2) and circumflex(n = 2). All fistulas were of the vascular type, terminating into either the pulmonary artery(n = 11) or coronary sinus(n = 1). The CAG delineated the characteristics of the fistula(origin, pathway and termination). Multiplicity of the origins and pathways of the fistulous vessels were common in most fistulas(8/12, 67% and 9/12, 75%, respectively). Multiplicity was common among the different fistula components(23/36, 64%). Adenosine pharmacologic stress ^(13)N-ammonia PET-CT revealed normal myocardial perfusion and ejection fraction in all but one patient, who showed a reduced ejection fraction.CONCLUSION PET-CT may be helpful for assessing the functional status of congenital CAFs in selected patients regarding clinical decision-making. Studies with a larger patient series are warranted.

Transcatheter closure of coronary artery fistula using Guglielmi detachable coil

BackgroundCoronary 动脉管(CAF ) 是一个稀罕异例。Transcatheter CAF 闭合用各种各样的材料被介绍了，但是仅仅很少数据在 Guglielmi 可拆卸的卷(GDC ) 上是可得到的。为 transcatheter CAF 闭合使用 GDC 的优点是更可控制的，因此安全得多什么时候与另外的卷相比。这份报告关于我们在用在我们的 hospital.Methods &#x00026 的 fibered GDC 的 CAF 的 transcatheter 闭合的经验；ResultsFrom 2002 到 2007，有有 CAF 的 10 个病人(年龄范围：28 ～ 56 27 岁的孩子， 7 男性) 经历了 transcatheter CAF 闭合。有发源从的 19 CAF 的一个总数恰好冠(n = 5 ) ，左降声调符号(n = 3 ) ，左前面的下降动脉(n = 10 ) 并且左主要箱子(n = 1 ) 。为每管的卷推广的中部的数字是 3 (范围：1 ～ 6 ) 。肺的动脉是 CAF 的远侧的通讯的最普通的地点(n = 14 ) ，由正确中庭列在后面(n = 3 ) ，左中庭(n = 1 ) 并且左室(n = 1 ) 。在 GDC 推广以后的立即的冠的 angiography 没在 12 揭示剩余分流(63.2%) CAF，在 5 的流动(26.3%) 的重要减小，当(10.5%) 2 不能由于小尺寸被关上时。九(90%) 病人们在 3 ～ 8 个月以内经历了重复 angiography。在 12 CAF 之中，那被堵塞立即推广以后，有有不足道的剩余流动的 2 CAF。在有显著地减少的流动的 6 CAF 之中立即推广以后， 2 在后续 angiography 完全被堵塞。总共， 12 (70.5%) CAF 完全被堵塞并且 5 (29.5%) CAF 仍然有不足道的剩余流动，它不需要任何另外的卷推广。在一个平均数期间列在后面在上面 4.3 &#x000b1；0.7 年，所有病人仍然是症状和复杂并发症 free.ConclusionsThe fibered GDC 是为 CAF 的经皮的闭合的一个安全、有效的方法。

Coronary Artery Fistula and Its Imaging Modalities—A Review Article

Coronary artery fistula (CAF) is known be a rare defect of coronary artery. When there is an abnormal pre-capillary communication between single or multiple branches of the coronary artery and any of the cardiac chambers or great vessels, it is designated as CAF. It can be found in both congenital and acquired form. Congenital origin of fistula occupies higher percentage compared to the acquired one. A wide variety of imaging modalities have been lunched to provide the best possible way to access and treat the defect. With regard to the assessment of the coronary artery fistula, trans-thoracic Doppler echocardiography and multi-slice CT (MSCT) angiography have commendable role, however, MSCTA comes in the leading position.

Rare multiple fistulas with large saccular aneurysms originating from left anterior descending artery and left main coronary artery

A 49-year-old female patient consulted us for a cardiac evaluation before undergoing colon adenocarcinoma surgery. Three years prior, the patient underwent coronary angiography for dyspnea. The coronary angiography examination revealed a fistula originating from the left anterior descending artery and left main coronary artery, which had soft aneurysmal sacs and most likely drained into the pulmonary artery. Parasternal short axis echocardiography revealed a color flow that could be related to the fistula, but the other echocardiographic findings were normal. The patient did not accept the proposed examination and invasive treatment.

Giant Aneurysm of a Coronary-Pulmonary Artery Fistula:A Rare Cause of a Diastolic Murmur

A coronary-pulmonary artery fistula with giant aneurysmal dilatation is an extremely rare clinical constellation.The natural course of this disease and the incidence of complications are unknown.Hence,optimal treatment,particularly in asymptomatic patients,is still a matter of debate.Here we report a case of a 71-year-old asymptomatic woman with a diastolic murmur.Comprehensive cardiovascular assessments including cardiac computed tomography and invasive coronary angiography revealed a coronary-pulmonary artery fi stula with giant aneurysmal dilatation.The patient was managed conservatively and has now been followed up for 5 years without any events.

Surgical Correction of Coronary Artery Ectasia Combining Congenital Coronary Artery Fistula

Background:Coronary artery ectasia(CAE)complicated with concomitant congenital coronary artery fistula(CCAF)is rare.This study characterizes the clinical characteristics of CAE combining CCAF,and reports a single-institution experience with surgical correction of CAE combining CCAF.Methods:A total of 24 symptomatic patients(8 males,median 52.5 years old)who underwent surgical correction of CAE combining CCAF in this center were reviewed.Based on the size of ectatic segment,the CAE were classified as a giant CAE(>20 mm,n=14)and a non-giant CAE(≤20 mm,n=10).Individualized surgical approaches were chosen.The patients were followed up for a median of 3.8 years.Results:The overwhelming majority of CAEs were solitary,and only 4.2%of CAEs were associated with multiple lesions.CAEs were predominantly located in the right coronary artery with predilection to women more than to men(2:1).95.8%of patients with the CCAF had single fistula defect.The right atrium was the most frequent drainage site(33.3%)followed by the left ventricle(25.0%).Surgical mortality was 4.2%.All 22 follow-up patients survived with recovery from symptoms and New York Heart Association(NYHA)functional class I-II.In 10 patients with non-giant CAEs undergoing closure of fistula alone,favorable in-hospital outcomes were recorded,but residual fistula(one patient)and acute inferior wall myocardial infarction related to intracoronary thrombosis(one patient)were observed at follow-up.In 11 patients with giant CAEs undergoing aneurysm resection plus distal bypass grafting at the time of closure of fistula,favorable in-hospital outcomes and encouraging midterm results were recorded.Additionally,in 3 patients with giant CAEs undergoing closure of fistula plus aneurysmal plication,adverse events occurred,including surgical death related to rupture of the ectatic segment(one patient),perioperative myocardial infarction caused by acute thromboembolism(one patient),nonfatal inferior wall myocardial infarction related to intracoronary thrombosis(one patient)at follow-up.Conclusion:Individualized surgical approaches based on the size and the location of ectatic coronary artery as well as fistula should be offered to symptomatic patients with CAE combining CCAF.

Congenital Coronary Artery Fistula in Children:A Review of 28 Cases with Clinical and Imaging Outcomes

Background:Congenital coronary artery fistula(CCAF)is a rare anomaly.Treatment strategies tend to close the defect with a symptomatic and significant shunt,primarily based on expert consensus and case series.Results for long-term follow-up in children are limited Methods:We conducted a retrospective study to assess clinical and imaging outcomes of children with CCAF at Siriraj Hospital,Thailand during 2000–2020.Patients with single ventricle were excluded.Treatment strategies[surgical closure(SC),and percutaneous closure(PC)]were classified and the clinical outcomes at the follow-up in 2021,including coronary thrombosis,myocardial ischemia,and the results of cardiovascular imaging were reviewed.Results:Twenty-eight children with CCAF were included in the study.The median age at diagnosis was 2.5 years(2 days–18 years).Presenting symptoms were audible murmur(82%)and heart failure(35%).Most of fistulae arose from the right coronary artery(12/28)and exited at the right atrium(11/28).In recent visits(0.5–14 years follow-up),six patients with asymptomatic small CCAF were managed by watchful follow-up without complications.PC was primarily treated in 11 children:7 underwent successful procedures;1 had a residual shunt and required re-intervention;1 had ischemic symptoms immediately after the procedure with left coronary occlusion that required device removal plus SC and 2 were technically unable to place the device,requiring SC.Four patients were waiting for interventions(1 PC and 3 SC).Cardiovascular imaging surveillance that followed closure demonstrated asymptomatic thrombus formation in three patients(1 PC and 2 SC).No mortality presented.Conclusion:CCAF with significant shunt is indicated to close either SC or PC.Ischemic events are rare but have been reported after closure.In addition,thrombus formation should be watched for post-intervention.Surveillance with cardiovascular imaging is recommended after defect closure(ideally 1–5 years post closure),or at interval follow-ups in patients with symptoms to evaluate possible recanalization,thrombus,or ischemia.Life-long clinical and echocardiographic follow-up is warranted.Watchful follow-up is acceptable for hemodynamically insignificant fistula without complication in the series.

A Rare Case of Transcatheter Closure of Both Inlet and Outlet of a Left Coronary Artery-to-Left Ventricular Fistula with Giant Coronary Artery Aneurysm

A congenital coronary artery fistula(CCAF)combined with giant coronary aneurysm(CAA)is a rare congenital cardiac abnormality.We reported an 8-year-old patient who underwent transcatheter closure of both inlet and outlet of a proximal left coronary artery(LCA)-to-left ventricular(LV)fistula with CAA of 41 mm×28 mm in diameter,during which acute occlusion of left anterior descending coronary artery(LAD)occurred immediately after device implantation at the inlet of fistula.We managed to prevent the patient from major adverse cardiac events by conservative therapy with dual antiplatelet agents instead of surgical removal of the device.The patient recovered well and had been follow-up for 2 years with no late complications reported.

Acquired Coronary Cameral Fistula Due to Post Stent Balloon Dilatation: Dual Coronary Artery Perforations into the Left Ventricle—What Is the Right Treatment?

A fifty-year-old female with recent history of LAD stent placement for instent restenosis, presented with chest pain and ventricular fibrillatory arrest. Angiography revealed total occlusion of her LAD stent. She underwent IVUS study, balloon angioplasty and stent placements. Post balloon dilatation of the under-deployed distal stent resulted in dual coronary artery perforations with extravasation of contrast into the LV cavity, a Type 4 Ellis coronary artery perforation (CAP). No extravasation was noted into the pericardium. Immediately a covered stent was deployed which completely sealed both perforation sites with resultant TIMI grade 3 flow. Under-deployment of stents is a common occurrence and is underappreciated. It can happen due to various reasons. Not many options exist at that time but to use a high pressure balloon and post dilate the stent. One rare complication is CAP due to post stent dilatation, with incidence reported as 0.1% to 3.0% of PCI procedures. Among the various type of CAP, Ellis Type 4 is of the least frequent however no studies have looked at its exact incidence rate. Prompt recognition and quick intervention are essential to good patient outcome. We chose to deploy a covered stent over the perforation with interim balloon tamponading. Deployment of the stent successfully sealed both the CAPs. Remarkably the patient remained stable and did not complain of chest pain throughout the procedure. The patient did well;she was discharged on dual antiplatelet therapy and is continuing to do well. We report a rare case of 2 distal LAD perforations that drained into the LV (an Ellis Type 4 CAP) caused by post stent dilatation that were successfully treated with a single covered stent. We report successful management of this case along with review of literature about management and dilemmas encountered is such instances.

Assessment of Right Coronary Artery Fistula by Multiplane Transesophageal Echocardiography

Coronary artery fistula(CAF) is a relatively uncommon cardiovascular disease. It is an abnormal communication between a coronary artery and a cardiac chamber, great vessels, or other vascular structures. Most are congenital. Multiplane transesophageal echocardiography(multiplane TEE. MTEE) can reveal the proximal dilation, the course and the drainage site of CAF. Presented in this paper was a report of right coronary artery-left ventricle fistula , for the first time,right coronary-left ventricle fistula diagnosed by MTEE and confirmed by angiography.

Left Coronary Artery Fistula Identified by Color Doppler Flow Imaging

Coronary artery fistula (CAF) is an abnormal communication between a coronary artery and a cardiac chamber, great vessel,or other vascular structures. The presence of the fistula is usually identified by angiography. In this paper, the diagnosis of left coronary artery-right ventricle fistula was made by color Doppler before angiography? and it was confirmed by surgery.Color Doppler flow imaging is a noninvasive method which can reveal the proximal dilatation, the course and the draining site of CAF.

Novel treatment of coronary artery fistulae concealing severe coronary artery lesion： using thrombus aspiration catheter as a delivery guide

在这份情况报告，我们在场多重冠的动脉 fistulae 发源从的吸藏近似左前面的下降(男孩) 并且到在一样的肺的动脉的正确湾穴 valsavla 和 empting 放。我们堵塞了由把血栓渴望导管用作一个交货指南的男孩 fistulae。就我们的 knowlege 而言，这是在血栓渴望导管的帮助下的冠的 fistulae 的吸藏的第一个案例。我们的经验可以建议血栓渴望导管能在与困难的 anotomy 对待冠的动脉 fistulae 被使用。

Application of interventional diagnostic and therapeutic technique for coronary artery fine branch fistula

Objective:To explore the value of angiographic diagnosis and interventional therapy of the coronary artery fine branch fistula.Methods:All of the 18 patients with coronary artery fine branch fistula underwent selective coronary arteriography,7 underwent interventional therapy, while 8 underwent prosthesis for coronary artery fistula (CAF) under extracorpored circulation. Results:Among 18 cases of coronary artery fine branch fistula, 7 happened in right coronary artery (38.9%), 11 in left coronary artery (61.1%). Among the 11 cases in left coronary artery,5 happened in descending anterior branch, 5 occurred in left circumflex branch, 1 arised from both left anterior branch and left circumflex branch. Among the 18 cases, there are 10 cases of coronary-to-pulmonary artery fistula (55.6%), 5 cases of fistula draining into right atrium (27.8%), 2 cases of fistula draining into left atrium (11.1%) and 1 draining into right ventricle (5.6%). Interventional treatment was successful in 7 patients. During the 12 months’ follow-up, there was no cardiovascular events. Conclusion:Selective coronary angiography is the first choice for diagnosing the coronary artery fine branch fistula. In respect of therapy, besides of surgical treatment, intervention is still a rather good measure presently.

Congenital coronary artery fistulas complicated with pulmonary hypertension: Analysis of 211 cases

AIM To compare the behavior of pulmonary hypertension(PHT) associated with coronary artery fistulas(CAFs) between the Asian and Caucasian subjects.METHODS CAFs may be complicated with PHT secondary to leftto-right shunt. Literature review limited to the English language. A total of 211 reviewed patients were collected. Of those, 111 were of Asian and 100 were of Caucasian ethnic origin. The mean age of the Asian and the Caucasian groups of patients were 48.9(range 19-83) and 49.9 years(range 16-85), respectively. In both groups, right heart catheterization was the most commonly(95%) used method for determining pulmonary artery pressure. RESULTS From all of the reviewed subjects, PHT was found in 49 patients(23%), of which 15 were Asian and 34 were Caucasian. In 75% of PHT subjects, mild to moderate PHT was reported and 76% of the fistulas had a vascular mode of termination. Treatment was surgical in 61%, followed by percutaneous therapeutic embolization(27%) and finally conservative medical management in 12% of PHT subjects. PHT was associated with a slight female gender predominance. The majority demonstrated mild to moderate PHT. PHT was reported more frequent in the Caucasian compared with the Asian ethnicity group. The majority of fistulas in patients with PHT had a vascular mode of termination. The results of this review are intended to be indicative and require cautious interpretation.CONCLUSION The likelihood for a CAF patient to develop PHT is presented when possessing the following features, with a Caucasian female having a fistula with a vascular mode of termination.

Bilateral coronary-pulmonary artery fistula with paroxysmal atrial arrhythmia:a case report

Coronary-pulmonary artery fistula(CPAF)is a relatively rare type of congenital coronary artery variation,which is mostly asymptomatic and is discovered incidentally by coronary artery CT imaging or angiography.However,CPAF can cause a phenomenon of inter-artery steal and hemodynamic disorders,leading to myocardial ischemia,arrhythmia,heart failure,syncope and even cardiac sudden death in severe cases.Here we reported a case of a 53-year-old female who complained of intermittent palpitations and had a history of paroxysmal atrial fibrillation.The coronary angiography revealed bilateral coronary artery fistulae to pulmonary artery.In this case,the multiple fistulas were very rare and significant.Multidisciplinary treatment should be considered,including cardiologists,surgeons,anesthesiologists and nurses,etc.Regular follow-up is essential for long-term disease management after diagnosis or surgery.

Overview of coronary artery variants, aberrations and anomalies

Coronary artery anomalies and variants are relatively uncommon congenital disorders of the coronary artery anatomy and constitute the second most common cause of sudden cardiac death in young competitive athletes. The rapid advancement of imaging techniques, including computed tomography, magnetic resonance imaging, intravascular ultrasound and optical coherence tomography, have provided us with a wealth of new information on the subject. Anomalous origin of a coronary artery from the contralateral sinus is the anomaly most frequently associated with sudden cardiac death, in particular if the anomalous coronary artery has a course between the aorta and the pulmonary artery. However, other coronary anomalies, like anomalous origin of the left coronary artery from the pulmonary artery, atresia of the left main stem and coronary fistulae, have also been implicated in cases of sudden cardiac death. Patients are usually asymptomatic, and in most of the cases, coronary anomalies are discovered incidentally during coronary angiography or on autopsy following sudden cardiac death. However, in some cases, symptoms like angina, syncope, heart failure and myocardial infarction may occur. The aims of this article are to present a brief overview of the diverse coronary variants and anomalies, focusing especially on anatomical features, clinical manifestations, risk of sudden cardiac death and pathophysiologic mechanism of symptoms, as well as to provide valuable information regarding diagnostic workup, follow-up, therapeutic choices and timing of surgical treatment.