The aetiology of congenital coronary artery fistulas remains a challenging issue. Coronary arteries with an anatomically normal origin may, for obscure reasons, terminate abnormally and communicate with different single or multiple cardiac chambers or great vessels. When this occurs, the angiographic morphological appearance may vary greatly from discrete channels to plexiform network of vessels. Coronary arteriovenous fistulas (CAVFs) have neither specific signs nor pathognomonic symptoms; the spectrum of clinical features varies considerably. The clinical presentation of symptomatic cases can include angina pectoris, myocardial infarction, fatigue, dyspnoea, CHF, SBE, ventricular and supraventricular tachyarrhythmias or even sudden cardiac death. CAVFs may, however, be a coincidental finding during diagnostic coronary angiography (CAG). CAG is considered the gold standard for diagnosing and delineating the morphological anatomy and pathway of CAVFs. There are various tailored therapeutic modalities for the wide spectrum of clinical manifestations of CAVFs, including conservative pharmacological strategy, percutaneous transluminal embolisation and surgical ligation.
Keywords: aetiology; angiographic morphology; clinical picture; congenital and acquired; coronary artery fistula; treatment.