Solitary Coronary Artery Fistulas: A Congenital Anomaly in Children and Adults. A Contemporary Review

Abstract

Congenital solitary coronary artery fistulas (CAFs) in adults are uncommon anomalies, which by themselves may resemble the whole spectrum of cardiac presentations from asymptomatic behavior to life-threatening and catastrophic events with syncope or shock and even sudden death. It may take decades to collect a reasonable series of patients in adults and children. From the literature between 1993 and 2004, 236 patients with CAFs were considered for evaluation. The present review is intended to assist cardiologists who are unfamiliar with congenital CAFs in adults by suggesting clues for decision making regarding diagnosis and management. Dyspnea and chest pain represented a frequent 91/128 (71%) clinical symptom in CAFs in adults while in the pediatric age group the majority were silent 105/133 (79%) and dyspnea and chest pain accounted for only 8% of the symptoms. The diagnostic modalities were mainly cardiac catheterization and coronary angiography. On the other hand, in the pediatric patients, echocardiography and coronary angiography mainly guided the diagnosis. Regarding treatment strategy in the reviewed subjects, percutaneous transluminal embolization was performed in 18% of the pediatric and in only 5% of the adult subjects. Surgical ligation (46% vs. 38%) and conservative medical strategies (36% vs. 24%) were reported in both pediatric and adult groups. Presentations of CAFs vary considerably in both groups. These differences include the diagnostic modalities, spontaneous closure, spontaneous rupture, and management. From this review, it seemed that--but it may be biased--surgical ligation remains the major mainstay for closure of CAFs in adult and pediatric populations. Recommendations are necessary for antibiotic prophylaxis and antiplatelet and/or anticoagulant therapy for prevention of endocarditis and thrombotic events in patients with CAFs associated with coronary artery dilatation or aneurysmal formation of the fistulous tract.

Fístula coronaria gigante. Presentación clínica, caracterización angiográfica, tratamiento y seguimiento a largo plazo: Caso clínico

“…La evolución natural de las fístulas coronarias es poco conocida, se describe un bajo porcentaje de cierre espontáneo, 1-2% 19,20 , la mayoría se mantiene en el tiempo y algunas de ellas pueden crecer en el tiempo hasta llegar a producir sobrecarga de volumen y falla cardiaca como suponemos ocurrió en el caso descrito, o producir isquemia miocárdica por robo coronario. Otras, aun sin crecer pueden ser fruto de complicaciones, como trombosis en el vaso nativo, aneurisma y ruptura o endocarditis bacteriana entre ellas 2,8,9,[13][14][15] .…”

Unusual fistulas and connections in the cardiovascular system: A pictorial review

“…Majority of fistulas are single, but multiple and complex fistulas have been reported. Cardiovascular anomalies are associated in 5%-30% of cases [6] . Left-to-right shunt is seen in 90% of cases [7] ; however, the shunt ratio is not large, as a result of which most patients are asymptomatic, especially in the adult population.…”

“…It has been reported in 0.05%-0.25% of patients undergoing coronary angiography, with an estimated prevalence of 0.002% in the general population [5] . Pathologically, it is characterized by dilation of involved vessel, thinned fistulous wall, thrombosis, atherosclerotic changes, myocardial hypertrophy and fibrosis [6] . As discussed above, the communication of the coronary artery could be with the lumen of a cardiac chamber (right ventricle -41%, right atrium -26%, left atrium -5%, left ventricle -3%), coronary sinus (7%), superior vena cava (SVC) (1%), pulmonary artery (17%) or the pulmonary vein.…”

Coronary Artery Fistula and Its Imaging Modalities—A Review Article

“…However, periodic evaluation and follow up is crucial in either of the cases [22]. Medical management may include anti-platelet therapy and prophylactic precautions against bacterial endocarditis.…”

“…Medical management may include anti-platelet therapy and prophylactic precautions against bacterial endocarditis. Another non-surgical option is trans-catheter embolization of the fistulous connection [17] [22]. If CAF is large, it is recommended for surgical intervention however, surgery is not risk free.…”