Abstract O.65: Long-term Outcome of Patients with Giant Coronary Aneurysms caused by Kawasaki Disease: A 30-year Experience in a Single Center in Taiwan

Abstract

Background: Patients with coronary artery aneurysms are at risk for thrombotic and stenotic complications later in their lives. The longitudinal changes of giant coronary aneurysms caused by Kawasaki disease (KD) and their long-term outcome are still unclear in Taiwan. Methods: By retrospective chart review of patients diagnosed with Kawasaki disease complicated by giant coronary aneurysms, we analyze incidence of ischemic event, survival rates, and related risk factors. Results: Between 1984 and 2012, 28 patients (24 male and 4 female) developed giant aneurysms. The mean age at onset and observe period was 3.8 years (range from 0.41 - 10.3 years) and 12 years (0 to 36.8 years), respectively. The initial median coronary Z-score was +5.75 (+0.62 to +19.06) in left main coronary artery, +4.49 (-0.18 to +11.19) in left anterior descending artery, +6.71 (+1.79 to +14.46) in right coronary artery. None of the giant aneurysms regressed during follow-up. The 5- and 35-year survival rates were 0.92 and 0.69, respectively. Except the three deaths, four additional male patients were diagnosed with acute myocardial infarction (AMI), and 2 of these diagnoses occurred within 1 year of KD onset. The incidence of AMI or death in patients with giant aneurysms was 26%, and 67% of the AMI occurred within the first year of KD onset. Clinical evidence of ischemia as documented in nuclear medicine studies was also noted in a further 9 patients (33%), and only one was female. AMI/death-free survival rates were 76% and 69% at 10 and 20 years after KD onset, respectively. Ischemia event-free survival rates were 63% and 36% at 10 and 20 years after disease onset. Men tended to have AMI and ischemia, and only one ischemia patient was female. The hazard ratio of gender on the ischemia events was 2.70 (95% CI: 0.33 - 21.73, P = 0.38). Conclusions: The long-term survival of KD patients with giant coronary aneurysms is guarded. Ischemic heart disease is the major cause of morbidity and mortality. Male KD patients, once they had giant aneurysms, tend to have ischemic events. Other risk factors need to be validated in a larger cohort.