Background: Some patients with Kawasaki disease (KD) develop large coronary aneurysms (diameter ≥ 6mm) and coronary stenosis, leading to ischemic heart disease. This study determined the long-term outcome for patients with Kawasaki disease complicated by significant coronary aneurysm.
Methods and Results: From the database in Seoul National University Children’s Hospital between December 1986 and December 2013, medical records of 83 patients (61 men and 22 women) with large coronary aneurysms (diameter ≥ 6mm) were retrospectively reviewed. Information on patient demographics, catheter or surgical interventions, and most recent status was collected. From these data, we calculated the survival rate, cumulative coronary intervention rate, coronary artery bypass graft surgery rate. The mean age at onset was 4.28 ± 2.62 years, and the mean observational period was 13.2 ± 6.5 years. The maximum coronary artery internal diameter ranged from 6.1 to 25mm (median 9mm). Giant coronary aneurysm was 57 patients (68.7%) and large coronary aneurysm (6-8mm) was 26 patients (31.3%). Coronary aneurysms had progressed to coronary artery stenosis and/or complete occlusion in 42 patients (50.6%). The overall freedom from coronary intervention, coronary artery bypass surgery, or coronary thrombi was respectively 85.5%, 85.5%, and 90.3%. Catheter and surgical coronary interventions (median 1 intervention; range 1 to 5 interventions) were performed in 20 patients (24.1%) at 9 month to 18 years after onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8mm vs ≥8mm). Myocardial infarction occurred in 8 (8.98 %) patients. During this study period, 1 patient died and 98% survival rates was seen in our patients group.
Conclusion: The long-term survival of patients with Kawasaki disease complicated by large coronary aneurysm is good even though a fourth of patients underwent multiple catheter or surgical interventions. Further research should focus on the indications for and effectiveness of percutaneous and surgical coronary interventions in large coronary aneurysm (≥ 6mm).