180 long term follow up of a non-progressive left main coronary artery fistula to right atrium

Abstract A 63 years old man, with multiple cardiovascular risk factor, presented at the emergency department with a transient ischemic attack during an hypertensive crisis. Electrocardiogram revealed a sinus rhythm and no sign of ischemia. No critical stenosis were detected at ultrasonography of the supra-aortic trunks. Brain CT showed an arachnoid cyst in right posterior cerebellar site. Transthoracic echocardiographic showed normal wall motion with a left ventricular ejection fraction of 66%. The transesophageal echocardiography, performed for searching possible emboligenic heart disease, revealed a fistulous aneurysmal formation arising from the left main coronary artery. Angio-CT confirmed a CAF between distal left main coronary artery and right atrium, with a retro-aortic serpiginous course and an ectasic vascular structure (above 8.35 mm), this pathological vessel also had an aneurismatic formation of maximum diameter of 1 cm. CT scan detected also a 50% soft eccentric plaque in mid circumflex coronary artery and a less than 50% soft eccentric plaque in the right coronary artery. An elective hospitalization was performed in order to better assess the CAF. Coronary angiography revealed no significant coronary atherosclerotic disease and confirmed the CAF with its convoluted course. No left to right shunt was found either in cardiac magnetic resonance (Qp/Qs close to 1) or in right heart catheterization. Maximal stress test with cycloergometer was negative for clinical and electrocardiographic inducible ischemia (120W). CAF is a rare vascular abnormality, occurring in only 0.002% of the general population. The majority of CAFs have a congenital origin, but occasionally they may be acquired after cardiac surgery or repeated myocardial biopsies in cardiac transplantation. The CAFs may increase in size over time, although this does not occur invariably, and complications include: myocardial ischemia (due to myocardial ‘steal’), thrombosis and embolism, heart failure, atrial fibrillation, rupture, endocarditis/endarteritis, arrhythmias and also spontaneous rupture of the CAF causing hemopericardium. Current treatment options for CAF include surgery or transcatheter closure. Considered the age of the patient, the absence of clinical symptoms and the absence of left to right shunt, a conservative strategy was chosen. The patient had strict follow up, with clinical and echocardiographic assessment every 6 months, and with annually CT scan and stress test. During these 9 years of follow up the CAF remained stable, and the patients clinically asymptomatic.