Cardiovascular imaging in children and adults following Kawasaki disease

Abstract

Kawasaki disease (KD) is a paediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. Two guidelines exist regarding the follow-up of patients after KD, by the American Heart Association and the Japanese Circulation Society. After the acute phase, CAA-negative patients are checked for cardiovascular risk assessment or with ECG and echocardiography until 5 years after the disease. In CAA-positive patients, monitoring includes myocardial perfusion imaging, conventional angiography and CT-angiography. However, the invasive nature and high radiation exposure do not reflect technical advances in cardiovascular imaging. Newer techniques, such as cardiac MRI, are mentioned but not directly implemented in the follow-up. Cardiac MRI can be performed to identify CAA, but also evaluate functional abnormalities, ischemia and previous myocardial infarction including adenosine stress-testing. Low-dose CT angiography can be implemented at a young age when MRI without anaesthesia is not feasible. CT calcium scoring with a very low radiation dose can be useful in risk stratification years after the disease. By incorporating newer imaging techniques, detection of CAA will be improved while reducing radiation burden and potential complications of invasive imaging modalities. Based on the current knowledge, a possible pathway to follow-up patients after KD is introduced. Key Points • Kawasaki disease is a paediatric vasculitis with coronary aneurysms as major complication. • Current guidelines include invasive, high-radiation modalities not reflecting new technical advances. • Cardiac MRI can provide information on coronary anatomy as well as cardiac function. • (Low-dose) CT-angiography and CT calcium score can also provide important information. • Current guidelines for follow-up of patients with KD need to be revised.

Keywords: (Cardiac) MRI; Cardiac imaging; Coronary aneurysm; Kawasaki disease; MDCT.

Fig. 1

Coronary artery lesion in Kawasaki disease during follow-up. Extensive calcification (arrow) with ossification and bone marrow elements (insert, 400×) in the thrombosed and re-canalized left anterior descending artery from the explanted heart of a 29-year-old man who suffered from Kawasaki disease at age 3 years. The aneurysms remodelled, and the patient was discharged from follow-up at the age of 7 years when the coronary artery appeared normal by echocardiogram. The patient presented with progressive congestive symptoms at age 29 years and required cardiac transplantation. Characteristic ‘lotus root’ appearance of the artery results from thrombosis with recanalization (stars). Only one lumen remains patent (far left). Haematoxylin and eosin stain, 40×

Fig. 2

Imaging techniques for the follow-up of Kawasaki disease. a Echocardiogram of a giant aneurysm of the left main coronary artery (LMCA) and LAD. b Stress and rest SPECT Technetium-99M scan (myocardial perfusion scan) demonstrates ischemia of the inferior and septal wall. c Conventional CAG shows a giant aneurysm of the LAD and a smaller aneurysm of the right circumflex artery (RCX). d Cardiac MRI shows an aneurysm of the LAD. e Cardiac MRI indicates a myocardial infarction of the infero-posterior wall. f Multi-slice CT contrast-enhanced angiography with calcified aneurysms of the proximal LAD and right coronary artery (RCA). g CT calcium-score with calcifications of the proximal LAD. h 3D-CT angiography with a calcified aneurysm of the RCA

Fig. 3

Flowchart for the monitoring of Kawasaki disease with current imaging modalities starting at 1 year after the disease. ^aWhen information is lacking about coronary arterial aneurysms (CAA) status, a calcium score may be indicated as a screening method. If positive, a CMRI with adenosine should be performed. ^bLong-term follow-up (cardiovascular counselling) of risk group 1 may be dictated by national health care policies and future studies. ^cAccording to the availability and experience of a centre with (low-dose) CT angiography. ^dWhich of the different revascularization options best improves prognosis is unclear to date. ^eAdditional tests to evaluate for progression to stenotic lesions