Centers of Excellence in Interventional Cardiology and Radiology

Pediatric Angiology

Coronary Arteriovenous Fistula (CAVF)

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Normally, 2 coronary arteries arise from the root of the aorta and taper progressively as they branch to supply the myocardium. A coronary artery fistula involves a sizable communication between a coronary artery, bypassing the myocardial capillary bed and entering either a chamber of the heart (coronary-cameral fistula) or any segment of the systemic or pulmonary circulation (coronary arteriovenous fistula) (but these lesions are often collectively termed coronary arterial-venous fistulae (CAVFs), because of the same pathophysiologycal mechanism). A coronary artery connection to the pulmonary artery (coronary-pulmonary artery fistula) may also be considered under this grouping.

The origin of a fistula is rarely bilateral, involving both left and right coronary artery systems. Most fistulae terminate in a venous chamber or vessel and, only rarely, into the left ventricle or the pericardium. The major sites of termination include the right side of the heart (90%), left ventricle, left atrium and the coronary sinus. The most frequent sites of termination in the right side of the heart, in descending order, are the right ventricle, right atrium, and pulmonary vasculature.

Coronary fistula communications can be congenital and acquired. Congenital coronary artery fistulae may occur as an isolated finding or may appear in the context of other congenital cardiac anomalies or structural heart defects, most frequently in critical pulmonary stenosis or atresia with an intact interventricular septum and in pulmonary artery branch stenosis, tetralogy of Fallot, coarctation of the aorta, hypoplastic left heart syndrome, and aortic atresia.

Acquired coronary artery fistula may rarely arise as a consequence of trauma such as a gun shot wound or a stab wound. They can also occur after cardiac surgery or invasive cardiac catheterization with percutaneous transluminal coronary angioplasty, pacemaker implantation, or endomyocardial biopsy, as a result of septal myectomy in association with hypertrophic cardiomyopathy, muscle bundle resection in operative repair of tetralogy of Fallot, as a complication of radiofrequency ablation of accessory pathways.

Most coronary artery fistulas are small, do not cause any symptoms, and are clinically undetectable until echocardiography or coronary arteriography is performed for an unrelated cause; they usually do not cause any complications and can spontaneously resolve. However, larger fistulae are usually 3 times the size of a normal caliber of a coronary artery and may or may not cause symptoms or complications.

Over time, the coronary artery leading to the fistulous tract progressively dilates, which, in turn, may progress to frank aneurysm formation, intimal ulceration, medial degeneration, intimal rupture, atherosclerotic deposition, calcification, side-branch obstruction, mural thrombosis, and, rarely, rupture.

Occasionally, high-output congestive heart failure has been described.

Coronary artery fistulae may mimic the physiology of various heart lesions: fistulae that drain:

to the systemic veins or right atrium have a physiology similar to an atrial septal defect;

to the pulmonary arteries have physiology similar to a patent ductus arteriosus;

to the left atrium cause a volume load similar to mitral regurgitation;

to the left ventricle have physiology similar to that of aortic insufficiency.

How Common are CAVFs?

Coronary artery fistula accounts for 0.2-0.4% of congenital cardiac anomalies. Approximately 50% of pediatric coronary vasculature anomalies are coronary artery fistulae. There is no race or sex predilection noted.

Coronary artery fistula may present in patients at any age but is usually suspected early in childhood when a murmur is detected in an asymptomatic child or with symptoms of congestive heart failure. Older children with murmurs may present with symptoms of coronary insufficiency.

Symptoms associated with CAVFs

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Most children with small coronary artery fistulae (CAF) are asymptomatic, and continuous murmur may be audible on routine examinations if the fistulae are moderate to large in size. In infants, angina (chest pain) may be recognized by symptoms such as irritability, diaphoresis, pallor, tachypnea, and tachycardia. Most infants present at age 2-3 months after the pulmonary vascular resistance has decreased with heart failure symptoms, such as tiredness during feeding, tachypnea and excessive diaphoresis during feeds, wheezing, episodic pallor, and failure to thrive. Thus, in infancy, they can present with signs of low-output congestive heart failure.

Older patients may present with signs of low-output congestive heart failure, arrhythmias, syncope, chest pain, and, rarely, endocarditis. Patients with large fistulae may present with high-output congestive heart failure, although rarely. In older patients, symptoms may include the following:

Dyspnea on exertion




Fistula-related complications include:

Myocardial ischemia

Mitral valve papillary muscle rupture from chronic ischemia

Ischemic cardiomyopathy

Congestive heart failure from volume overload

Bacterial endocarditis

Sudden cardiac death

Secondary aortic valve disease

Secondary mitral valve disease

Premature atherosclerosis.

Diagnosis of CAVFs

Some laboratory tests may be elevated ( cardiac enzyme, brain natriuretic peptide – in cases with heart failure).

Imaging studies:

Chest radiography

Electrocardiography (ECG)

Echocardiography is helpful in diagnosing most fistulae and may reveal the following:

1. Left atrial and left ventricular enlargement

2. Dilatation of the coronary artery

3. High-volume flow

4. Drainage of the fistula

5. Holodiastolic run-off in the descending aorta

6. A squirt of color flow into a chamber without significant dilatation of the coronary artery in cases of small coronary artery fistulas

7. A dilated coronary sinus.

Cardiac catheterization remains the modality of choice for defining coronary artery patterns of structure and flow. Most frequently, intracardiac pressures are normal and shunt flow is modest.

MRI has been a good alternative for imaging proximal coronary abnormalities, and newer imaging sequences have provided improved anatomic imaging as well as indices of coronary flow and function. Spatial resolution is often limiting, and the distal course and insertion of the fistulous connection may not be well imaged

Recently, multidetector row computed tomography (MDCT) cardiac imaging has provided excellent distal coronary artery and side branch imaging.

Stress thallium studies may be used to document areas of myocardial ischemia before and after operative repair


As we pointed above, in childhood, most patients with coronary artery fistulae are asymptomatic; however, some patients may present with symptoms of dyspnea on exertion, increased fatigability, and, possibly, signs of high-output congestive heart failure. Rarely, patients may present with angina, palpitations, or signs of exercise-related coronary insufficiency. Direct medical treatment for symptomatic relief can be used until investigations and operative repair can be performed. Spontaneous closure may occur in small fistulae. Small fistulous connections in the asymptomatic patient may be monitored. Most lesions enlarge progressively and warrant operative repair, either by transcatheter or surgical techniques.

In view of the natural progression in larger fistulae to dilate over time, with progressively increasing risk of thrombosis, endocarditis, or rupture, the general advice is to close all but the small fistulous connections. In borderline situations, provide close echocardiographic or angiographic follow-up imaging to identify enlargement of feeding vessel in asymptomatic patients. Patients with large fistulae, multiple openings, or significantly aneurysmal dilatation may not be optimal candidates for transcatheter closure.

Endocarditis and other complications are risks, and patients should be monitored for the same. In older individuals, the fistulae can rarely get obstructed with progressive atherosclerosis and cause resolution of symptoms.

The goal of treatment is the obliteration of fistulae, while preserving normal coronary blood flow. The risk of presence of fistula should be balanced with the risk of complications with procedures to occlude the fistula.

The primary therapeutic approach to coronary artery fistula (CAF) is interventional catheterization or surgery.


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1. Transcatheter embolization – minimally invasive

2. Cardiac surgical intervention : Some fistulae are unsuitable for the transcatheter approach and preferably are addressed surgically ( fistulae with multiple connections, circuitous routes, and acute angulations that make catheter positioning difficult or impossible ).

Procedural options can be optimized by careful identification of the number of fistulous connections, nature of feeding vessel or vessels, sites of drainage, and quantification of myocardium at risk for injury or loss and the hemodynamic shunt related to the fistula (ie, Qp:Qs)

Diagnostic cardiac catheterization should be performed initially with or without additional therapeutic intervention. Initial diagnostic catheterization should both define hemodynamic significance of the lesion and provide detailed angiographic assessment of the anatomy of the abnormality, in particular, the origin, course, regional narrowings, and the nature of the insertion. Transcatheter embolization techniques using coils, bags, or other devices can be performed on an outpatient basis at the time of diagnostic studies or later, and may obviate the need for cardiac surgical intervention.

The procedure is performed in catheterism lab. Usually, it is done under general anesthesia. Thus, after local asepsy, a small inguinal incision is made. Through this incision, the physician will introduce a small narrow tube into the femoral artery, then through this tube a catheter will be introduced which will reach the coronary arteries, under fluoroscopic control (X ray control). The interventionist will perform an angiogram, so the CAVF will be visualized.

Generally, the course of the fistulous tract is delineated angiographically, selectively catheterized, and wired along its entire length. A delivery catheter or sheath is then positioned antegradely or retrogradely along the stabilizing wire for delivery of a suitable occlusive coil or device. The occlusive device is positioned so as to minimize myocardial muscle loss or injury. Often multiple devices or coils may be required for effective occlusion.

Is it painful?

No, usually, the intervention is performed under general anesthesia.

How long does it take?

The duration of the procedure is about 1-2 hours and takes place in the cardiac catheterization laboratory.


The complications are rare; the complications are reduced by the proper preparation and the continuous surveillance of the patient. Potential complications:

• allergic reactions to administered substances, including renal disfunction

• reactions to anesthetic compounds

• arteriovenous fistulas at the vascular puncture site

• minor bleeding at the vascular puncture site

• fever

• headache, migraine

• infection

• gaseous embolism

• cardiac arrythmias

• extremely rare – cardiac perforation and cardiac tamponade (perforation of the cardiac wall and bleeding in the pericardial sac, which compresses the heart)

• stroke

• myocardial infarction

• inappropriate positioning or proximal extension of occlusive coils or devices may result in obstruction of side branches and muscle loss.

• intimal dissection of the coronary artery or thrombosis also may occur.

Before procedure

The preoperative assessment will establish if the closure of the defect can be done percutaneously or there is an indication of surgical closure of the defect. Physical examination and echocardiography are key elements which help making a decision.

Prior to the intervention, the interventional cardiologist must be prevented about any history of allergic reactions. Blood tests are taken including hemoglobin level, coagulation, renal function, and other specific tests.

The patient is admitted the day before the intervention, and he/she should not eat before the procedure.

After procedure

You will be connected to monitors that will constantly display your electrocardiogram (ECG or EKG) tracing, blood pressure, other pressure readings, breathing rate, and your oxygen level. You will be given pain medication for incisional pain or you may have had an epidural during surgery which will help with postoperative pain.

Since the procedure is minimally invasive, the postprocedural recovery is usually very fast. The majority of patients can leave the hospital the following day. Indications about recovery and postprocedural treatment will be clearly specified to all patients.

Treatment with aspirin and plavix must be administered 6 months post intervention in order to prevent blood clot formation; some patients may also need an anticoagulant regime. Patients with persisting aneurysmal dilatations may benefit from prolonged antiplatelet agents.

Patients remain at risk for development of endocarditis until the flow is stopped and should receive antibiotic prophylaxis for any dental, GI tract, and urologic procedures if associated with a cyanotic heart disease (unique dose of amoxicilin, ampicilin, or clindamicin in case of pennicilin allergy).

Follow-up monitoring may also include stress studies and repeat angiography – your doctor will decide.


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In summary, elective CAVFs treatment in childhood constitues optimal management, even in the asymptomatic patient. Although older patients with CAVF may remain asymptomatic, not develop complications, or have spontaneous CAVF closure, it is important to remember that most unoperated patients develop both symptoms and fistula-related complications with increasing age and incur increased morbidity and mortality when ligation is performed later in life.

that perform the procedure

Sună Mesaj