Congenital Coronary Artery Fistula: Report of Five Patients, Diagnostic Problems and Principles of Management

Abstract

The detection of a coronary artery fistula (CAF) is usually by chance and, because of the to-and-fro characters of the murmur, a misdiagnosis of a patent ductus arteriosus is frequently made. Correct diagnosis can only be reached after hemodynamic investigation completed by angiography. This lesion is often accompanied by few or no symptoms in infancy, but may become symptomatic with aging even if the shunt is small: complications such as endocarditis, rhythm disturbances, aneurysmatic dilation and rupture have been reported. Five cases of CAF have been studied in our institution: 3 of them have been operated upon in extracorporeal circulation with good results. Principles of management are controversial: many authors advocate delay of treatment until symptoms appear: in our opinion, based on the natural history of the disease and on our experience, closure of the fistula in pediatric age, even in asymptomatic patients, is the treatment of choice.

Bilateral coronary artery fistulas terminating at the same location case report

“…The incidence of this anomaly ranges from 0.2-0.4% in selected series of congenital heart disease patients, and its incidence in the overall population is estimated to be about 0.002% [1][2][3]. The frequency of bilateral coronary to pulmonary fistula is quite rare.…”

Recurrent coronary artery fistula

“…Coronary anterial fistulae are rare but have clinical importance because of the attendant complications (Liberthson et at., Lowe et at. , 1981 ;Lowe and Sabiston, 1982;Macri et al, 1982;Meyer et al, 1975;Rittenhouse et ai., 1975;Stanley et al, 1981 ;Theman and Crosby , 1981 Wilde and Watt, 1980). They are usually congenital but they may be iatrogenic due to inadvertent anastomosis of an aortocoronary vein graft to the left anterior descending coronary vein (przybojewski, 1982;Starling et al, 1981), secondary to penetrating or blunt chest injuries (Alter et al, 1977;Austin et al, 1977;Fallahnejad et al, 1980;Pellegrini et al, 1980;Snyder etal., 1978;ThandroyenandMatisonn, 1981;Vemeyre et al, 1979) or rarely to malignancy (Koller et al, 1980).…”

“…Congential coronary fistulae usually drain to the right side of the heart or the coronary sinus resulting in left -toright shunt; however, they may drain into the left atrium or left ventricle producing a picture of aortic regurgitation (from left-to-left shunt) and coronary steal. Although they may be asymptomatic and may remain so for many years or even diminish and close spontaneously (Francis et al, 1979;Lowe et al, 1981;Mahoney et al, 1982) they are frequently associated with symptoms and complications especially during middle and old age (Alter et al, 1977;Austin et al, 1977;Fallehnejad et al, 1980;Koller et al, 1980;Liberthson et al, 1979;Lowe et al, 1981;Lowe and Sabiston, 1982;Macri et al, 1982;Meyer et al, 1975;Pellegrini et al, 1980;Przybojewski, 1982;Rittenhouse et at., 1975;Snyder et al, 1978;Stanley et at., 1981;Starling et al, 1981;Thandroyen and Matisonn, 1981;Theman and Crosby, 1981;Urrutia-S et al, 1983;Vemeyre et al, 1979;Vogelbach et al, 1979;Wilde and Watt, 1980). Symptomatology may be produced by a compromise of coronary perfusion (angina, arrhythmias, dyspnea), or by the left-to-right shunt (dyspnea, atrial tachyarrhythmias, congestive heart failure, pulmonary hypertension).…”

“…Although they may be small without clinical sequellae, in many instances they are associated with myocardial ischemia, angina, infectious endocarditis, thrombosis, aneurysmal dilatation and rupture, rhythm disturbances, dyspnea, left-to-right shunt with congestive heart failure or pulmonary hypertension, "signs of aortic leak and coronary steal, ' , embolization and acute myocardial infarction (Liberthson et al, 1979;Lowe et al, 1981;Lowe and Sabiston, 1982;Macri et al, 1982;Meyer et al, 1975;Rittenhouse et al, 1975;Stanley et al, 1981;Theman and Crosby, 1981;U rrutia-S et al, 1983;Vogelbach et al, 1979;Wilde and Watt, 1980). Since the incidence of these complications increases with age and surgical treatment carries a low mortality and is considered "curative," surgery is usually recommended in symptomatic and even in asymptomatic patients (Liberthson et al, 1979;Lowe et al , 1981;Lowe and Sabiston, 1982;Macri et al, 1982;Meyer et al, 1975;Rittenhouse et al, 1975;Urrutia-S et al, 1983;Wilde and Watt, 1980). The surgical approach is dictated by the exact anatomy, and varies from simple ligation without cardiopulmonary bypass to intracardiac repair with cardiopulmonary bypass and cardioplegic arrest with associated vein graft revascularization (Liberthson et al, 1979;Lowe et al, 1981;Lowe and Sabiston, 1982;Meyer et al, 1975;Stanley et al, 1981;Theman and Crosby, 1981;Wilde and Watt, 1980).…”

Coronary-Pulmonary Artery Fistula Associated with Right Heart Failure: Successful Closure of Fistula with a Graft Stent

“…[4][5][6][7][8][9] Coronary-pulmonary artery fistula is a very rare congenital anomaly of the coronary artery. Although coronary artery fistula is the most common congenital anomalies of the coronary arteries, the reported angiographic prevalence of this anomaly ranges only from 0.1% to 0.20/0.2,3 Many fistulas are small and found incidentally during coronary arteriography, and approximately half the patients with these anomalies remain asymptomatic; whereas the other half develop congestive heart failure, infective endocarditis, superior vena cava syndrome, arrhythmia, sudden death, myocardial ischemia induced by a coronary steal phenomenon, or rupture of an aneurysmal fistula.…”