Coronary arteriovenous fistulae are a coronary anomaly, presenting in 0.002% of the general population. Their etiology can be congenital or acquired. We present a review of recent literature related to their epidemiology, etiology, pathophysiology, clinical presentation, diagnostic approach, and therapeutic management.

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Coronary arteriovenous fistulae: a review

Coronary artery fistula (CAF) is a relatively rare anatomic abnormality of the coronary arteries that afflicts 0.002% of the general population and represents 14% of all the anomalies of coronary arteries. Its clinical relevance focusses mainly on the mechanism of "coronary steal phenomenon", causing myocardial functional ischaemia even in the absence of stenosis, hence common symptoms are angina or effort dyspnoea. The suggested diagnostic approach is guided by the patient's symptoms and consists of a number of instrumental examinations like ECG, treadmill test, echocardiography, computed tomography scan, cardiac magnetic resonance and coronary angiography. If it is not an incidental finding, coronary angiography is required in view of the optimal therapeutic planning. Small-sized fistulae are usually asymptomatic and have an excellent prognosis if managed medically with clinical follow-up with echocardiography every 2 to 5 years. In the case of symptomatic, large-sized or giant fistulae an invasive treatment, by transcatheter approach or surgical ligation, is usually a reasonable choice, and both strategies show equivalent results at long-term follow-up. Antibiotic prophylaxis for the prevention of bacterial endocarditis is recommended in all patients with coronary artery fistulae who undergo dental, gastrointestinal or urological procedures. A life-long follow-up is always essential to ensure that the patient is not undergoing progression of disease or further cardiac complications.

Coronary Artery Fistulae: Anatomy, Diagnosis and Management Strategies.

Coronary artery fistula: a review.

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 was 51.4 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, multi-slice 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.

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

Objective To report surgical treatment of congenital coronary artery fistulas (CAF) in 52 patients between May 1988 and July 1997. Methods Fifty two patients ranged from 9 months to 58 years (mean 15 7±16 4 years) were operated. There were 36 patients without other coexisting cardiac defects, 9 of them over 20 years old were symptomatic, while only one less than 20 years old had clinical findings before operation. Sixteen patients associated with other cardiac lesions. The fistulae originated from right coronary artery in 37 patients (71 2%), and from left coronary artery system in 15 patients (28 8%). The sites of CAF drainage were to right ventricle, right atrium, left ventricle, left atrium and pulmonary artery in 22 (43 3%), 16(30 8%), 6(11 5%), 3(5 8%) and 5(9 6%) patients respectively. Results Correction under cardiopulmonary bypass was done in all patients with no mortality. The proximal opening of a fistula was closed through an arteriotomy on the anomalous coronary artery in 10 patients. Closure of the distal opening of a fistula draining into a cardiac chamber or the pulmonary artery was performed in 26 patients. In 16 patients, both the proximal and distal opening were closed. Two and 3 distal openings of a fistula were found in 6 and 3 patients respectively. The mean diameter of fistulas in 43 patients with single ostium was 7 34±4 12 mm (2 15 mm). No residual shunt was found at discharge from the hospital. Thirty seven patients were followed up for a mean period of 3 51±1 72 years (1 month 8 years). There were no clinical symptoms in all patients during follow up. ST T change persisted in one patient. Conclusion Early surgical management is a safe and effective treatment for congenital coronary fistula. Accurately locating and closing of the opening of CAF is the key point to prevent residual shunt. Other associated cardiac defects should be treated simultaneously.

Surgical treatment of 52 patients with congenital coronary artery fistulas

Coronary artery fistulas (CAFs) are infrequent anomalies, coincidentally detected during coronary angiography (CAG). To elucidate the currently used diagnostic imaging modalities and applied therapeutic approaches. Five Dutch patients were found to have CAFs. A total of 170 reviewed subjects were subdivided into two comparable groups of 85 each, treated with either percutaneous ‘therapeutic’ embolisation (PTE group) or surgical ligation (SL group). In our series, the fistulas were visualised with several diagnostic imaging tests using echocardiography, multidetector computed tomography, and CAG. Four fistulas were unilateral and one was bilateral; five originated from the left and one originated from the right coronary artery. Among the reviewed subjects, high success rates were found in both treatment groups (SL: 97% and PTE: 93%). Associated congenital or acquired cardiovascular disorders were frequently present in the SL group (23%). Bilateral fistulas were present in 11% of the SL group versus 1% of the PTE group. The fistula was ligated surgically in one and abolished percutaneously in another. Medical treatment including metoprolol was conducted in two, and watchful waiting follow-up was performed in one. Several diagnostic imaging techniques are available for assessment of the anatomical and functional characteristics of CAFs.

G. P. Kimman

Papers

Diagnostic and therapeutic approach of congenital solitary coronary artery fistulas in adults: Dutch case series and review of literature