Excerpt

Coronary artery fistulas are rare abnormalities of the coronary arteries with an incidence of 0.08–0.3 % [1]. After orthotopic cardiac transplantation the incidence increases to approximately 8 %, which is very likely related to repeated endomyocardial biopsies usually taken from the right ventricular portion of the interventricular septum [2]. Fistulas can occur in any of the three major coronary arteries. Most of these fistulas are related to the right coronary artery (RCA) or the left anterior descending artery (LADA); however, the circumflex coronary artery (CX) is also affected infrequently (RCA in approximately 55 % of cases, LCA in 35 %, and both in 5 %) [3]. More than 90 % of coronary artery fistulas drain into the low-pressure venous system resulting in a left-to-right shunt. Fistula drainage occurs into the left ventricle [4] in only 3 % of cases. Most fistulas present single communications; nevertheless, multiple fistulization has been described. Fistulas are usually of congenital origin but there are also acquired forms. The clinical presentation can be asymptomatic or vary from fatigue to dyspnea, orthopnea, angina pectoris, endocarditis, arrhythmias, stroke, and myocardial ischemia or infarction. …