Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

Abstract

Background and objectives: Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms.

Subjects and methods: The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database.

Results: The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD 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 (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death.

Conclusions: Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms.

Keywords: Coronary aneurysm; Mucocutaneous lymph node syndrome; Myocardial infarction; Prognosis.

Fig. 1. Kaplan-Meier actuarial freedom from cardiac events. (A) Freedom from any intervention between coronary aneurysm size (6-8 mm vs. ≥8 mm). Trans-catheter and/or surgical coronary interventions were performed in 20 patients, resulting in a 33.7% cumulative coronary intervention rate at 20 years after KD onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (p=0.148). (B) Freedom from myocardial infarction. The median elapsed time from KD onset to myocardial infarction was 3.0 months (range: 0.5-31.2 months). Cumulative survival rate was 89.6% and most myocardial infarction occurred in the first year after onset of giant coronary aneurysm. KD: Kawasaki disease.