The role of echocardiography in Kawasaki disease

Kawasaki disease (KD) is an acute, self-limited vasculitis affecting young children. It can result in coronary artery abnormalities in a significant proportion of patients, especially if the diagnosis is missed or treatment gets delayed. Echocardiography is the imaging modality of choice for detection of coronary artery abnormalities and assessment of myocardial function. It is also useful for characterization and risk stratification of patients with KD. Echocardiography should be performed at the time of diagnosis and then again at 1-2 weeks and 4-6 weeks after treatment, for uncomplicated cases who do not have significant coronary artery involvement. Use of a standardized imaging protocol is necessary to detect and characterize coronary artery abnormalities, including standardization of measurements (Z scores). For patients with evolving abnormalities, more frequent assessment is necessary in order to detect thromboses in aneurysms. Long-term prognosis and management is dependent on both the maximal and current Z scores of aneurysms. Patients with large or giant aneurysms (i.e., Z score ≥ 10) are at the highest risk of both thrombosis and stenosis. Such patients need careful follow-up for subsequent cardiovascular events. Many of them would be candidates for advanced cardiovascular imaging and may require revascularization therapy. Serial echocardiography plays a key role in surveillance. In addition, stress echocardiography has proven useful as a modality to assess for inducible myocardial ischemia. Intravascular ultrasound has been recommended for functional and structural assessment of coronary arteries in children with KD.