Lifetime cardiovascular management of patients with previous Kawasaki disease

Abstract

Kawasaki disease (KD) is an inflammatory disorder of young children, associated with vasculitis of the coronary arteries with subsequent aneurysm formation in up to one-third of untreated patients. Those who develop aneurysms are at life-long risk of coronary thrombosis or the development of stenotic lesions, which may lead to myocardial ischaemia, infarction or death. The incidence of KD is increasing worldwide, and in more economically developed countries, KD is now the most common cause of acquired heart disease in children. However, many clinicians in the UK are unaware of the disorder and its long-term cardiac complications, potentially leading to late diagnosis, delayed treatment and poorer outcomes. Increasing numbers of patients who suffered KD in childhood are transitioning to the care of adult services where there is significantly less awareness and experience of the condition than in paediatric services. The aim of this document is to provide guidance on the long-term management of patients who have vascular complications of KD and guidance on the emergency management of acute coronary complications. Guidance on the management of acute KD is published elsewhere.

Increased Neutrophil Respiratory Burst Predicts the Risk of Coronary Artery Lesion in Kawasaki Disease

“…Previous pathological studies in KD patients showed all cardiac tissues are associated with the acute inflammatory phase of the disease. Vasculitis leads to the destruction of normal arterial structure, followed by aneurysm expansion, especially affecting the proximal coronary artery, destruction of intima and media, and replacement of myocytes by fibroblasts and connective tissue (1). The most important complication is serious coronary artery lesions (CAL).…”

CT Angiography or Cardiac MRI for Detection of Coronary Artery Aneurysms in Kawasaki Disease

“…There is consensus that radiation exposure (due to imaging), is associated with an increase in lifetime cancer risk, especially in children and should be kept to a minimum ( 18 , 19 ). Recent guidelines consider this radiation exposure and therefore mention CMR as a good imaging technique in KD ( 6 ), however, our data suggest cCTA is the more preferred modality for the visualization vascular morphology, thrombus formation and calcified lesions. CMR is valuable to visualize tissue characteristics and physiology, and has been used to demonstrate and in particular ischemia and tissue damage following myocardial infarction in KD ( 20 , 21 ).…”

“…Invasive CAG is not routinely used because of its invasive nature and risk of complications. In a recent overview, the need for guidance for the long-term management of KD patients was emphasized, suggesting non-invasive modalities such as echocardiography and CMR, and only when other modalities cannot be used, to consider low radiation dose computed tomography (CT) ( 6 ). As the limitations of echocardiography are known, we have been performing additional imaging such as cCTA and CMR, in selected patients in our national referral center ( 7 ).…”

Cardiovascular Involvement in Kawasaki Disease Is Much More Than Mere Coronary Arteritis

“…It is conjectural whether endothelial dysfunction correlates with occurrence of CAAs (123)(124)(125)(126). Patients with KD and CAAs have been reported to have an increased risk of myocardial infarction and early deaths (11,116,(127)(128)(129)(130). Recent literature has, however, suggested that long term cardiovascular morbidity in patients with KD may not only be restricted to patients who have been detected to have CAAs (11,131).…”