Mortality among persons with a history of kawasaki disease in Japan: Mortality among males with cardiac sequelae is significantly higher than that of the general population

Abstract

Background: The long-term prognosis of those having a history of Kawasaki disease (KD) is still unknown. Methods and Results: Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new definite diagnosis of KD. Patients were followed-up until December 31, 2004 or their death. Standardized mortality ratios (SMRs) were calculated based on the Japanese vital statistics data. Of 6,576 patients enrolled, 36 (27 males, 9 females) died and the SMR was 1.14. Despite the high SMRs during the acute phase, the mortality rate was not high after the acute phase for the entire group of patients. Although the SMR after the acute phase was 0.71 for those without cardiac sequelae, 10 males (but none of the females) with cardiac sequelae died during the observation period; and the SMR for the male group with cardiac sequelae was 2.55 (95% confidence interval: 1.23-4.70). Conclusions: The mortality rate among males with cardiac sequelae because of KD was significantly higher in this cohort than in the general population. On the other hand, those for females with the sequelae and for both males and females without sequelae were not elevated.