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Large Coronary-Cameral Fistulas in an Adult Patient: A Rare Coronary Anomaly with Concealed Clinical Findings (Case Report and Literature Review)

1 Assistant Professor of Radiology, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
2 Professor of Cardiology, Fellow of Echocardiography, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
3 Associated Professor of Cardiology, Fellow of Echocardiography, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
4 Interventional Cardiologist, Associate Professor of Cardiology, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
5 Fellow of Echocardiography, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
6 Cardiologist, Fellow of Echocardiography, Shafa Cardiovascular Hospital, Gorgan, IR Iran
7 CT Technician, Rajaie Cardiovascular, Medical and Research Center, Tehran, IR Iran
8 Department of Pathology and Laboratory Medicine, University of California-Los Angeles (UCLA), Los Angeles, California, USA
*Corresponding author: Azin Alizadehasl, Associated Professor of Cardiology, Fellow of Echocardiography, Rajaie Cardiovascular, Medical, and Research Center, Tehran, IR Iran. Tel: +98-2123922190, E-mail:
Archives of Cardiovascular Imaging. 4(2): e40954 , DOI: 10.5812/acvi.40954
Article Type: Case Report; Received: Mar 30, 2016; Accepted: May 29, 2016; epub: Feb 25, 2016; collection: May 2016


Coronary-cameral fistulas (CCFs) constitute a rare anomaly that can be incidentally detected during angiography. CCFs are solitary, large or small assemblies that originate from coronary arteries and enter one of the cardiac chambers. We describe a 29-year-old woman, who referred to our clinic with the chief complaints of palpitation, atypical chest pain, and dyspnea on exertion (functional class II). Multimodality imaging confirmed the diagnosis of a CCF from the left main with extension to the right atrium and drainage therein. The CCF was closed percutaneously with a patent ductus arteriosus occluder successfully.

Keywords: Coronary-Cameral Fistula; Coronary Anomaly; Echocardiography; Coronary Computed Tomography Angiography

1. Introduction

The anomalies of the coronary arteries comprise those of termination, origin, structure, and course. Anomalies of termination are termed coronary artery fistulas and are classified as congenital anomalies. Coronary artery fistulas can create a communication between the coronary capillary bed and a chamber of the heart or between the coronary artery and the systemic or pulmonary circulation. These lesions are identified in pathophysiological aspects and termed coronary arterial venous fistulas. A coronary artery connected to the pulmonary artery may likewise be categorized through this grouping. Nonetheless, if a coronary artery arises from the trunk of the pulmonary artery with no direct connection with the aorta, it is grouped as an anomalous origin (origin of the coronary artery from the pulmonary artery).

Most CA fistulas- including CCFs- are small, do not create any symptoms, and are clinically undetectable till echocardiography or coronary arteriography is done for an unrelated reason; they frequently do not cause any complications and may spontaneously resolve, although coronary artery fistulas with a larger size might cause symptoms, signs, or complications.

We herein present a case report as well as a literature review of papers on CCFs in adults.

2. Case Presentation

A 29-year-old woman with the chief complaints of palpitation, dyspnea on exertion (functional class II), and atypical chest pain referred to our adult congenital heart disease clinic. Physical examinations showed blood pressure of 105/60 mmHg, heart rate of 82 bpm, and temperature of 37°C. Cardiac auscultation demonstrated a continuous murmur (grade II) at the left sternal border. ECG revealed a normal sinus rhythm with a normal axis and without ischemic changes.

Transthoracic echocardiography demonstrated top normal left atrial (LA) size, mild left ventricular (LV) enlargement with suboptimal contractility (ejection fraction ~ 50%), and normal right ventricular (RV) size and function without any valvular heart disease. There was an abnormal continuous turbulent flow initiating from the left the coronary sinus of Valsalva with a tortuous course and terminating in the right atrium (RA), near the interatrial septum. Transesophageal echocardiography was performed for better evaluation and revealed a dilated left main coronary artery (1.2 cm) (Figure 1A and 1B; Video 1 in Supplementary File) with an abnormal continuous turbulent flow, which passed the LA and drained into the RA, adjacent to the interatrial septum. (The size of the fistula origin at the RA side was 0.5 cm) Computed tomography (CT) angiography reported a dilated left main, which was divided into 3 branches: the left circumflex artery, left anterior descending artery, and another tortuous and elongated fistulous tract. The finding confirmed the diagnosis of a CCF from the left main with extension to the RA and drainage therein (Figure 2A and 2B). Given the patient’s history of chest pain, she was subjected to exercise stress echocardiography. However, the test was terminated in stage I due to her vertigo and new wall-motion abnormality. Consequently, based on the steal phenomenon and exercise-induced ischemia, the closure of the cameral fistula between the left main coronary artery and the RA was planned. The fistula was successfully closed with a percutaneous patent ductus arteriosus occluder (Figure 3A and 3B).

Figure 1.
A and B, Transesophageal echocardiography, short-axis view (white arrows), shows a dilated left main coronary artery with an anomalous course of the left coronary artery to the interatrial septum and drainage into the right atrium.
Figure 2.
A and B, Coronary computed tomography angiography shows a dilated left main coronary artery with an anomalous course of the left coronary artery to the interatrial septum.
Figure 3.
A, Diagnosis of a coronary fistula, which originates from the left main artery and ends in the right atrium, can be confirmed by cardiac catheterization (Video 3 in Supplementary File). B, left main coronary artery injection shows a closed coronary-cameral fistula with no substantial residual flow (Video 4 in Supplementary File).

3. Discussion

3.1. Anatomy

Typically, two coronary arteries rise from the root of the aorta and taper gradually as they branch to supply the myocardium. A fistula happens if a substantive communication arises that bypasses the myocardial capillary bed and connects with a low-pressure cardiac cavity (ventricle or atria) as a CCF or a branch of the systemic or pulmonary system. A direct communication between a CA and one of the cardiac chambers (the CCF) has been noted. The origin of a fistula is infrequently bilateral, involving both LA and RA systems. A fistulous opening into a chamber or the drainage is commonly single or, uncommonly, double if both coronary arteries are involved (1-4).

CCFs may be small or large and incline to enlarge over time. Often, the limits of what constitutes a fistula and what constitutes a normal vessel are debated.1 The major sites of origin of fistulas are the right CA (RCA) (40-60%), left anterior descending (30-60%), left circumflex artery, and a combination thereof. The majority of the termination sites of fistulas are in the right side of the heart (90%): in the RV followed by the RA, the coronary sinus, and the pulmonary vasculature. The remaining termination sites are in the LV, followed by the LA (1, 3).

CCF communications might be congenital and acquired. Congenital CCFs may be an isolated finding or may appear in the context of other structural heart defects or congenital cardiac anomalies such as critical pulmonary stenosis or atresia with an intact interventricular septum and in PA branch stenosis, hypoplastic left-heart syndrome, tetralogy of Fallot, coarctation of the aorta, and aortic atresia (1-3). Acquired CCFs might infrequently arise as a result of traumas such as gunshot wounds and stab wounds. They might also happen after cardiac surgery or invasive cardiac catheterization with percutaneous transluminal coronary angioplasty (PTCA), pacemaker implantation, or even endo-myocardial biopsy too (1, 4).

3.2. Embryology

CCFs are often created by the aberrancies of normal embryological development. They may appear as the persistence of the sinusoidal lumen of the primitive tubular heart that supplies the myocardial blood flow in the early embryonic period. Another description might be the faulty growth and development of the distal branches of the recti-form vascular network of the CA (1-2, 5). CCFs also happen in 45-50% of the patients with pulmonary atresia who have the intact ventricular septum. Their morphogenesis is assumed to begin early in the development with embryological myocardial lacunae when the myocardial blood supply is derived from its own lumen. Consequently, the coronary arteries connect with these intra-myocardial trabecular spaces (3, 6).

3.3. Epidemiology

CA fistulas account for 0.2 - 0.4% of congenital cardiac defects. Nearly 50% of pediatric coronary vasculature anomalies are CA fistulas. CCFs might present in patients at any age but are frequently suspected early in childhood when a murmur is noticed in an asymptomatic child or with symptoms of congestive heart failure (CHF). Older children with murmurs may present with symptoms of coronary insufficiency. In a multi-center review, substantially more problems in terms of operative risks and post-operative complications happened after age 20 years (3-4).

3.4. Pathophysiology

A small CCF typically does not cause any hemodynamic compromise. However, a larger fistula may create the CA steal phenomenon, which leads to the ischemia of the segment of the myocardium perfused by the CA. The pathophysiological mechanism of the CA fistula is myocardial stealing or reduction in the myocardial blood flow distal to the site of the CA fistula connection. The mechanism is associated with the diastolic pressure gradient and runoff from the coronary vasculature to a low-pressure receiving cavity. If the fistula is large, the intra-coronary diastolic perfusion pressure increasingly diminishes (3, 6-7). The coronary vessel attempts to compensate by the progressive enlargement of the ostia and feeding artery. Finally, the myocardium beyond the site of the fistula's origin is at risk of ischemia, which is most frequently evident in relation with increased myocardial oxygen demand during exercise or activity. Over the time, the CA leading to the fistulous tract increasingly dilates, which — in turn — might progress to frank aneurysm formation, intimal ulceration, intimal rupture, medial degeneration, atherosclerotic deposition, calcification, mural thrombosis, side-branch obstruction, and — infrequently — rupture.

The issues that determine the hemodynamic importance of the fistulous connection contain the size of the communication, the resistance of the recipient chamber, and the potential for the development of myocardial ischemia. Infrequently, high-output CHF has been explained (3, 8).

CCFs might mimic the physiology of various heart lesions. Fistulas that drain into the RA have a physiology similar to that of an atrial septal defect (ASD). Fistulas that drain into the LA do not cause a left-to-right shunt however do cause a volume load similar to that in mitral regurgitation. And fistulas that drain into the LV have a physiology similar to that of aortic insufficiency (6, 8).

3.5. History

Almost all children with a small CCF who have no symptoms may have a continuous murmur heard on routine physical examinations In infants, virtually chest pain is diagnosed symptomatically like diaphoresis, irritability, tachypnea, tachycardia, and pallor. Therefore, in infancy, CCFs may be accompanied by signs of low-output CHF. Older patients may present with signs of low-output CHF, syncope, arrhythmias, chest pain, and even infrequently endocarditis. Rarely, patients with large fistulas may present with high-output CHF. In older patients, symptoms may include dyspnea on exertion, palpitations, chest pain, and fatigue (3, 8).

3.6. Physical Examination

CCFs are suspected subsequent the detection of a continuous murmur on routine physical examination. On clinical examination, the murmur is indicative of a PDA but is heard lower on the sternal border than normal; therefore, the location is frequently atypical for a PDA. Furthermore, the continuous murmur of a CCF might have an unusual diastolic prominence, and also frequently peaks in mid to late diastole — that is un-characteristic of the systolic accentuation in a patient with a PDA.

If the CCF connects to LV, only an early diastolic murmur might be heard because a little coronary flow is evident during the period of systole.

Some patients with fistulas with a large shunt may present with signs of CHF and chest pain. Wide pulse pressure and collapsing pulse might be noted. The apex beat is diffuse with a palpable or audible third heart sound (S3) gallop in a large fistula. Heart sounds are frequently reduced in strength. A holosystolic murmur of mitral valve (MV) regurgitation is audible at the apex (8-13).

3.7. Diagnostic Considerations

Other situations to consider in the differential diagnosis of a CCF contain the following:

- Ruptured the aneurysm of sinus of Valsalva

-Ventricular septal defect (VSD) with aortic regurgitation

- Venous hums

- Intra-thoracic systemic fistulas

- Congenitally systemic fistula to the pulmonary vein

-- Pulmonary arteriovenous malformation

- Aorto-pulmonary window

- Acquired or congenital pulmonary vein stenosis

Conservative management might be appropriate in some patients because of spontaneous closure happens in 23% of small fistulas, primarily those rising from the left coronary arteries system. Patients should be advised that they need to receive proper antibiotics for any dental, oropharyngeal, urological or gastrointestinal tract surgical procedures if associated and concomitant with a cyanotic heart disease (10-13).

3.8. Imaging
3.8.1. Chest Radiography

Chest radiography findings are usually normal in a CCF, except in the presence of a important shunt flow, at which time cardiomegaly might be evident. Furthermore, pulmonary venous congestion and also interstitial edema can be seen (5-6).

3.8.2. Electrocardiography

Electrocardiography (ECG) findings are typically normal. In some cases, however, ECG may reveal changes in the setting of larger fistulas. ECG may show the effects of volume load on the LV and LA. Infrequently, in the presence of coronary steal, ischemic changes and/or arrhythmias may be evident (3, 6, 14).

3.8.3. Echocardiography

Echocardiography is helpful and beneficial in diagnosing most CCFs and might reveal the following:

- LA and LV enlargement as a result of a signifimayt shunt flow or reduced regional or global dysfunction as a result of myocardial ischemia.

- Dilatation of the CA: The feeding CA often looks enlarged, ectatic, and tortuous.

- High-volume flow: This might be detected by color-flow imaging at the beginning or along the length of the vessel.

- Drainage of the fistula: The site of the drainage must be carefully sought. Frequently, it is evident as a disturbed flow most often within the RV.

- A squirt of color flow into a chamber without important dilatation of the CA in cases of small CA fistulas (5, 12, 14).

3.8.4. Cardiac Catheterization and Aortography

Cardiac catheterization still remains the modality of choice for defining the CA patterns of structure and flow. Most often, intra-cardiac pressures are normal and the shunt flow is modest. In addition, therapeutic embolization using occlusive coils or devices can be done via catheterization (5, 12-15).

3.8.5. Magnetic Resonance Imaging and Computed Tomography Imagining

Reliably, complete, noninvasive three-dimensional imaging of the coronary vasculature is beneficial. Conventionally, magnetic resonance imaging has been a good substitute for imaging proximal coronary abnormalities, and novel imaging arrangements have improved anatomic imaging as well as indices of the coronary flow and also function. Spatial resolution is often limiting, and the distal course and insertion site of the fistulous connection can not be well imaged.

Multi-detector row computed tomography (MDCT) cardiac imaging providing outstanding distal CA and side branch imaging. Imaging of an entire three-dimensional volume and the heart may be obtained within 20 seconds, with better temporal and spatial resolution than magnetic resonance imaging. Several authors now advocate the consideration of MDCT in the imaging of CA anomalies (6, 15-17).

A retrospective study by Lim et al. (7). suggested that CT angiography is beneficial and helpful in detecting CA fistulas. The study included 6, 341 patients who underwent coronary CT angiography. CA fistulas were found in 56 (0.9%) patients, a higher percentage than has generally been found using conventional angiography. Moreover, CT angiography found CA fistulas to lead most commonly to the PA, rather than -as conventional angiography has indicated to the ventricle (14).

3.8.6. Nuclear Imaging

Stress thallium assessments can be used to document the areas of myocardial ischemia before and also after trans-catheter or operative repair (14-17).

3.9. Treatment
3.9.1. Medical

In childhood, the utmost patients with CCFs are asymptomatic; though, some patients might present with symptoms of increased fatigability, dyspnea on exertion and also possibly signs of high output CHF. Infrequently, patients might present with chest pain, palpitations, or signs of exercise related ischemia and coronary insufficiency 8, 14, 18-19 Direct medical management for symptomatic relief might be used till operative repair may be performed. Spontaneous closure might happen in small fistulas. Small fistulous associates in asymptomatic patients might be followed up medically. Most lesions expand gradually and warrant operative correction, either by trans-catheter or surgical methods.

Endocarditis and other complications are the risks that involved, and patients should be monitored. In older individuals, the fistulas might rarely become obstructed with progressive atherosclerosis and even cause the resolution of symptoms (8, 12, 20-21).

3.9.2. Catheterization

The diagnostic catheterization must be done initially with or without supplementary therapeutic intervention. Initial diagnostic catheterization must both determine the hemodynamic importance of the fistula and make available a detailed angiographic study of the anatomy of the abnormality (9, 14, 22-24).

Procedural options may be optimized by a cautious identification of the number of fistulous connections, nature of the feeding vessel (vessels), the sites of drainage, and also quantification of the myocardium at risk of injury or loss and the hemodynamic shunt associated to the fistula. The purpose of management is the obliteration of the fistula whereas preserving the normal CA blood flow. The risk of the presence of the fistula must be balanced with the risk of the complications related with procedures for the occlusion of the fistula (23-25).

3.9.3. Therapeutic Transcatheter Embolization Indications

Given the natural progress in larger fistulas to dilate over time with the rise in the risk of endocarditis, thrombosis, or rupture the general advice is to close all, however small, fistulous connections. In doubtful conditions, close echocardiographic or angiographic follow-up imaging should be provided to detect the enlargement of the feeding vessel in asymptomatic patients. Patients with large fistulas, multiple openings, or importantly aneurysmal dilatation may not be ideal candidates for transcatheter closure (1, 6, 14). Indications

Given the natural progress in larger fistulas to dilate over time, with the rise in risk of endocarditis, thrombosis, or rupture, the general advice is to close all however the small fistulous connections. In doubtful conditions, close echocardiographic or angiographic follow up imaging must be provided to detect the enlargement of the feeding vessel in asymptomatic patients. Patients with large fistulas, multiple openings or importantly aneurysmal dilatation might not be ideal candidates for trans-catheter closure (1, 6, 14). Technique

Trans-catheter embolization methods using coils, bags or other devices might be done on an outpatient basis at the time of diagnostic assessments or later and may prevent the requirement for surgical intervention. Commonly, the course of the fistulous tract is defined angio-graphically, selectively catheterized, and wired along its whole length. Then a delivery catheter or sheath is located ante-gradely or retro-gradely along the stabilizing wire to deliver a proper occlusive coil or device. Frequently multiple devices or coils might be needed for complete occlusion.

Often the trans-catheter approach is a relativey complicated intervention and needs an knowledgeable and skilled operator and interventionalist with expertise in both coronary arteriography and also embolization procedures. Embolization often needs complex catheter manipulation as well as the selection of different wires and catheters (24-29).

3.9.4. Surgery Indications

Indications for surgery are the same as those mentioned in embolization part. Some fistulas are inappropriate for the trans-catheter approach and are rather addressed surgically. These CA fistulas might contain fistulas with multiple connections, acute angulations and circuitous routes, that render catheter positioning hard or even impossible (12, 14, 21, 28-29). Techniques

Surgical repair typically is approached via median sternotomy and also cardiopulmonary bypass. The feeding vessel must be recognized and its course and site of insertion must be delineated. Also the site of the assumed fistulous drainage must be recognized prior to the establishment of cardiopulmonary bypass. Trans-esophageal echocardiographic imaging has been very helpful in assisting of procedure.

A classic procedure encompasses opening the chamber or cavity into which the fistula drains, recognizing the fistula, and closing the site of drainage with a suture or patch. If the fistula go into the ventricle or the feeding vessel is large, the CA should be opened and the opening to the fistula is closed with the suture. The arterio-tomy is then closed. Large aneurysms might need excision (12, 26-29).

3.10. Mortality and Morbidity

Fistula related complications are present in 11% of cases younger than 20 years and in 35% of patients who are older than 20 years.

Fistulas may be related with complications like myocardial ischemia, MV insufficiency from chronic ischemia, ischemic cardiomyopathy and CHF from volume overload, bacterial endocarditis, even sudden cardiac death, and also premature atherosclerosis. Usually, small fistulas remain clinically silent and are diagnosed in an echocardiography or autopsy. Indeed in a small fistulas, myocardial blood supply is not compromised enough to create symptoms. Even spontaneous closure frequently happens; but, some might dilate over the time.

Larger fistulas gradually enlarge over time, and complications like myocardial infarction, CHF, arrhythmias, aneurysm formation, rupture, endocarditis and even death are more likely to rise in older cases. Spontaneous closure has been so infrequently reported in the large fistulas.

The mortality rate of surgical repair of CA fistulas normally ranges between 0 and 4%. Variants that may increase the surgical risk contain the fistula between the RCA and the LV and also the presence of giant aneurysms. The complications of surgery contain myocardial ischemia or infarction (in 3% of cases) and also CA fistula recurrence (4% of cases) (3, 14, 27, 29).

3.11. Follow-Up

Follow-up care should be provided after hospital discharge to check for sign of ischemia or recurrence of CCFs. Patients who have undergone CA surgical interventions especially cases who have sustained cardiac muscle loss must have ongoing cardiac follow up monitoring, which might contain stress studies and repeat angiography as required. cases treated surgically and with trans-catheter procedures must receive maintenance doses of antiplatelet drugs and, possibly, an anticoagulant regime for the first six months post-operatively till the operative surface has undergone endothelialization. Cases with continuing aneurysmal dilatations might benefit from continued antiplatelet agents use (1, 3, 14, 29).


Supplementary material(s) is available ||.


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Figure 1.

A and B, Transesophageal echocardiography, short-axis view (white arrows), shows a dilated left main coronary artery with an anomalous course of the left coronary artery to the interatrial septum and drainage into the right atrium.

Figure 2.

A and B, Coronary computed tomography angiography shows a dilated left main coronary artery with an anomalous course of the left coronary artery to the interatrial septum.

Figure 3.

A, Diagnosis of a coronary fistula, which originates from the left main artery and ends in the right atrium, can be confirmed by cardiac catheterization (Video 3 in Supplementary File). B, left main coronary artery injection shows a closed coronary-cameral fistula with no substantial residual flow (Video 4 in Supplementary File).