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

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.