Bilateral Coronary Artery Fistulas and Pulmonary Sequestration. An Unusual Combination of a Rare Disease: Case Report and Review of the Literature

Coronary artery fistulas are a rare entity, especially when they origin from both the left and right coronary arteries. CAF may be asymptomatic but severe complications are possible. They are usually congenital but acquired forms have been described. The management of CAF remains undetermined and depends on various factors. Pulmonary sequestration is another rare congenital malformation. It is a non-functioning lung segment without continuity with the normal lung parenchyma. In this case report an 83-year-old male with newly diagnosed idiopathic pulmonary fibrosis, history of ischemic cardiomyopathy and severe tricuspid regurgitation was admitted for syncope. Due to episodes of non-sustained ventricular tachycardia an invasive coronary angiography was ordered which showed 2 coronary artery fistulas. The two CAF, arising respectively from the right coronary artery and the left anterior descending artery, both supplied blood to vessels in a sequestrated segment of the lower lung lobe. This is the first report of two coronary artery fistulas, arising from both left and right coronary artery, providing blood supply to a pulmonary sequester. Although this entity is extremely rare, knowledge of it can prevent inappropriate management and fatal results.