Background and Objective: Long term prognosis of Kawasaki disease (KD) with giant aneurysm is not yet well understood. We conducted a nationwide survey of KD with giant aneurysm for recent 10 years, and analyzed cardiac events of those patients.
Methods: Nationwide epidemiological survey of KD has been conducted every 2 years since 1970 in Japan. We performed questionnaire survey based on 16th - 21th (1999 - 2010) nationwide epidemiological data.
Results: We send questioners to 275 facilities asking about 415 patients who were reported to have giant aneurysm (>=8mm), and collected the data of 334 patients (80.5%). We excluded 84 non-giant aneurysm patients and 36 duplicated cases and difined finally 214 patients. Out of 214 patients, 13 deaths and 32 AMIs were described (6.1% and 15.0%, respectively). The first AMI attack was mostly reported within a few months from KD onset (medium 5 months (0-85 months)). AMI was occurred one time in 26 patients, and two times in 6 cases. Myocardial ischemia was observed in 80% of AMI patients, and 12 patients were received coronary artery bypass graft. Thirteen patients were reported to be dead (medium 1 month (0-23 months) from KD onset). There were 6 cardiac deaths within 1 month from KD onset (5: rupture of aneurysm, 1: AMI). The others were all caused by AMI except for 2 accidental death. Four out of 6 AMI deaths were caused by the first AMI attack. For the remaining 2 AMI deaths, the period from the first AMI to second fatal AMI was 1 month and 6 month, respectively. There were no death reported byond 2 years from KD onset.
Conclusion: AMI and cardiac death of KD with giant aneurysm occurred mostly in early phase of KD onset. Coronary rupture occurs within a month after onset, and 84% AMI and all AMI death occurs within 2 years after onset. Patients survival become promising after 2 years from onset. These evidences indicate that therapeutic strategy up to 2 years after onset is extremely important for prognosis of KD with giant aneurysm.