Journal of Pediatric Cardiology and Cardiac Surgery

Kawasaki disease (KD) is the most prevalent acquired vasculitis in children; coronary artery dilation and aneurysms are the most important complications of this disease. Alternatively, coronary artery dilation can be observed in rare congenital coronary artery anomalies, such as anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). At the age of 3, the patient suffered from typical KD, which was successfully treated with immunoglobulin infusion; however, right coronary dilation was noted at 4 days of illness. She was followed-up for the persistent right coronary artery dilation. At the age of 6, she complained of chest pain, and exercise stress test revealed ST segment depression in leads V3–V6, which resulted in the tentative diagnosis of coronary artery fistula. She was referred to our hospital for further evaluation and treatment. X-ray coronary computed tomography leads to the diagnosis of ALCAPA and stress myocardial scintigraphy confirmed ischemia of the anterolateral wall of the left ventricle; therefore, she underwent successful direct reimplantation of the anomalous left coronary artery to the aortic root. Here, we report the case of a patient with ALCAPA who was misdiagnosed as having coronary artery dilation associated with KD, and the patient was followed-up without appropriate treatment. We found that misdiagnosis of congenital anomalies can be a pitfall of echocardiographic evaluation of coronary arteries in patients with acute KD, and clinicians must be able to properly distinguish between both the plausible causes of dilation.