Coronary fistula - an unexpected preanaesthetic finding

Coronary artery fistulae are congenital vascular anomalies defined as aberrant communications between a coronary artery and a cardiac chamber, large vessel or another vascular structure. In the present case the preanaesthetic clinical assessment led to a fortuitous detection of a rare coronary artery anomaly, which changed the initial therapeutic option. A 21-year-old female patient was admitted for a right ankle fracture. She had a two years history of constrictive chest pain inconsistently generated by effort of medium intensity, which had not been investigated previously. Clinical examination identified a grade V systolic-diastolic murmur audible on the entire anterior thorax, with no other abnormalities. The patient underwent cardiological evaluation including transthoracic (TTE) and transesophageal echocardiography (TEE). TTE and TEE revealed an important dilatation of the left main coronary artery (LM) and a dilated circumflex artery (CX), with a very turbulent flow and a fistulous traject drawing most probably in the right atrium. The TTE and TEE evaluation raised the suspicion of a coronaro-cavitary fistula between the CX and the right atrium. The patient underwent coronary catheterization which confirmed a coronary fistula connecting CX with a superior vena cava-right atrium junction, with a hemodynamic significant left- to-right shunt. Surgeons opted for a conservative orthopedic management of the fracture; the patient continued to present exertional chest pain and was scheduled for interventional fistula closure. Our case confirms the importance of the preanaesthetic clinical examination, as a gold standard, that was decisive in identifying this rare, but potentially lethal congenital anomaly, as it triggered a series of tests, which established the diagnosis.