Abstract 216: Natural History of Kawasaki Disease with Medium-sized Coronary Aneurysm: Long-term Follow-up in a Tertiary Medical Center

Abstract

Objectives: Patients with coronary artery aneurysms are at risk for thrombotic and stenotic complications later in their lives. The longitudinal changes of medium-sized coronary aneurysms caused by Kawasaki disease (KD) and their long-term outcome are still unclear in Taiwan. Methods: We retrospectively reviewed medical records of KD patients with medium-sized coronary aneurysms (4-8mm). The longitudinal change of coronary diameters were re-evaluated by domestic coronary z score calculator. The coronary artery diametes were transformed to standard deviation units from the mean (Z-score) normalized for body surface area. We also look for the potential risk factors for the persistence of coronary lesions. Results: Between 1983 and 2012, 56 KD patients suffered from medium aneurysms. The male to female ratio was 41:15. The mean age of disease onset was 2.13 years old, and 39.3% (22 of 56) was diagnosed at below 1 year old. The mean follow up duration was 8.2 years. No death occurred in this group of patient, and only one patient with persistent coronary aneurysm and stenosis/calcification had clinical evidence of myocardial ischemia. However, coronary aneurysms persisted in 24 (42.8%) patients and were associated with stenosis in 8 patients (14.3%), and calcification in 4 patients (7.1%). The coronary aneurysm persistence-free survival rates at 6 months, and 1, 2, 5 years after KD onset were 81%, 69%, 52%, 43%, respectively. Chi-square analysis revealed KD patients with history of ever deterioration of coronary Z score were significantly associated with the persistence of coronary aneurysms ( P = 0.0125). However, the initial aneurysm Z score (+4.43 vs +4.49), male gender or IVIG use were not risk factors to the persistence of coronary aneurysms. Conclusion: The prognosis of Kawasaki disease patient who developed medium size coronary aneurysm was good. However, those with ever deterioration of coronary Z scores were high risk group to have persistent coronary aneurysms. Specific therapies to promote vascular health in these patients should be advocated.