Echocardiography in Kawasaki Disease

Initial Evaluation and Imaging Standards

The American Heart Association recommends that echocardiography should be used as the initial imaging modality for cardiac assessment in children with Kawasaki disease due to its high sensitivity and specificity for detecting abnormalities of the proximal coronary artery segments 1, 2
The initial echocardiogram should be performed as soon as the diagnosis of Kawasaki disease is suspected, but treatment should not be delayed by the timing of the study 1
Sedation is frequently needed for children under 3 years of age and may be required in older, irritable children to obtain high-quality images 1
Studies should be performed with appropriate high-frequency transducers and supervised by an experienced pediatric echocardiographer 2
2D imaging should be performed with the highest-frequency transducer possible, even for older children, to allow high-resolution detailed evaluation of the coronary arteries 1

Coronary artery evaluation should focus on imaging the left main coronary artery (LMCA), left anterior descending (LAD), left circumflex, right coronary artery (RCA) (proximal, middle, and distal segments), and posterior descending coronary arteries 1
Assessment of left ventricular function is essential as myocarditis is universal in acute Kawasaki disease 3
Evaluation should include LV end-diastolic and end-systolic dimensions, shortening fraction, and ejection fraction 3
For uncomplicated cases, echocardiographic evaluation should be performed at 6-8 weeks after onset 3, 4
More frequent echocardiographic evaluation is needed for children at higher risk (persistently febrile or with coronary abnormalities, ventricular dysfunction, pericardial effusion, or valvular regurgitation) 3

While echocardiography is excellent for detecting coronary abnormalities, it has limitations in identifying thrombi and coronary artery stenosis 3
Coronary angiography offers more detailed definition of coronary artery anatomy and can detect coronary artery stenosis or thrombotic occlusion 4
Patients with more complex coronary artery lesions may benefit from coronary angiography after the acute inflammatory process has resolved (generally recommended 6-12 months after onset) 4

Relying on a single echocardiogram early in the disease course, as coronary abnormalities may develop later 1
Failing to use sedation when needed, resulting in suboptimal imaging 1
Overlooking assessment of ventricular function, which is commonly affected in Kawasaki disease 3
Assuming normal coronary arteries based on an initial echocardiogram in the first week of illness 1

The American College of Cardiology recommends echocardiography as a Class I indication for baseline studies and reevaluation in all pediatric patients with suspected or documented Kawasaki disease, indicating that the benefits substantially outweigh the risks and the procedure should be performed 5, 6

The American College of Cardiology recommends an echocardiographic schedule of at diagnosis, at 2 weeks after onset, and at 6-8 weeks after onset for uncomplicated cases without significant coronary involvement 5

The American College of Cardiology recommends against echocardiography (Class III indication) for long-term follow-up studies in patients with Kawasaki disease who had no coronary abnormalities during the acute phase of the disease process, unless coronary abnormalities were present at 4-6 weeks 5, 6