Abstract
Background: Acute coronary syndrome (ACS), which is emerging in adults long after confirmed (followed-up or lost-to-follow), or missed Kawasaki disease (KD), is poorly characterized.Methods and Results: A Japanese retrospective nationwide hospital-based questionnaire survey of ACS during 2000–09 was conducted to characterize such patients. Among a total of 67 patients (median age 35, male 76%) recruited, low conventional coronary risks (≤1/6) was noted in 75%, a diagnosis of ST-elevation and myocardial infarction or cardiac arrest in 66%, medication before ACS in 22% (warfarin in 4%), and no prior history of acute myocardial infarction in 94%. One-month mortality was 19%. KD diagnosis was made in 32 during acute illness (Group A), in which 17 were lost to follow, and retrospectively in the other 35 from coronary imaging at ACS (Group B). Group A developed ACS at lower coronary risks (≤2/5 in 87 vs. 65% in group B, p = 0.043) at a younger age (26.5 vs. 40 yo, p < 0.001). In group A, followed-up patients developed ACS under medication before ACS (87 vs. 0% in lost-to-follow patients, p < 0.001) for giant aneurysm in culprit lesions (69 vs. 29%, p = 0.030). One-month mortality was comparable between groups A and B, and between patients followed-up and lost-to-follow in group A. The culprit lesion in group A was characterized by the association of an aneurysm ≥6 mm in acute KD (100%), lack of significant stenosis (61%) or giant aneurysm (50%) in the long-term (median interval 16 y), and the presence of intravascular ultrasound-derived calcification at ACS (86%).Conclusions: The present retrospective nationwide questionnaire survey demonstrated nationwide emergence of initial ACS in young adults at low coronary risks, who are followed-up or lost-to-follow after confirmed KD and initial coronary aneurysms ≥6 mm.
Highlights
Kawasaki disease (KD) is a common acute febrile disorder of unknown etiology in young children, which is associated with systemic vasculitis especially in coronary circulation [1, 2]
A confirmed diagnosis of KD was made by medical doctors in 32 during acute illness (Group A, verified by medical documents in 22; obtained by interview with family members in 10), among which 17 were lost to follow up at acute coronary syndrome (ACS)
A missed KD diagnosis was made retrospectively in the other 35 patients in accordance with coronary imaging (n = 35) (n = 34 at the registered ACS events, n = 1 at previous acute myocardial infarction (AMI) in adulthood) and autopsy (n = 0) at ACS (Group B), in which episodes suggestive of acute KD during childhood were obtained in the parent interviews for 2 patients
Summary
Kawasaki disease (KD) is a common acute febrile disorder of unknown etiology in young children, which is associated with systemic vasculitis especially in coronary circulation [1, 2]. Up to 25% of KD patients develop coronary aneurysms leading to lethal coronary vascular events, which occurs typically early after the acute illness in infants associated with giant aneurysms [1, 2] This disease is a great concern to cardiologists as well as pediatricians, because grown-up KD patients with coronary sequelae exhibit functional and structural alterations in the coronary arteries [3,4,5,6,7]. Such patients, seem to have a coronary event long after the acute illness in adulthood, which is poorly characterized [8,9,10,11,12]. Acute coronary syndrome (ACS), which is emerging in adults long after confirmed (followed-up or lost-to-follow), or missed Kawasaki disease (KD), is poorly characterized
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
