[Follow up and review of patients with Kawasaki disease complicated with giant coronary artery aneurysms for a decade: a single-institution experience].

OBJECTIVE

To evaluate the prognosis and clinical features of patients with giant coronary artery aneurysm (GCAA) caused by Kawasaki disease (KD).

METHOD

KD complicated with GCAA was diagnosed in 55 patients between January 2003 and December 2012 in Guangzhou Women and Children's Medical Center.Of the 55 patients, 48 were studied(43 boys, 5 girls). According to the follow-up time, these patients were divided into four groups: ≤ 1 year follow-up group, > 1-3 years follow-up group, > 3-5 years follow-up group, and > 5-10 years follow-up group. These patients were investigated at the acute stage, and were followed up respectively at two weeks, one month, three months, six months, and one year after hospitalization and every 3 to 6 months after a year. All patients regularly accepted echocardiography and electrocardiographic examination, while some cases accepted CT coronary angiography (CTA) examination and coronary angiography (CAG) examination to confirm the condition of GCAA.

RESULT

(1) The age of 48 patients ranged from 2 months to 10 years. Twenty cases were less than 1 age (42%), 30 cases were under 3 years of age (62%) and follow-up time was (4.0 ± 3.1) years. Age at endpoint was 1.5 to 19 years, mean (6.8 ± 4.2) years respectively. (2) In ≤ 1 year follow-up group, the proportions of no significant changes, retraction, and stenosis were 48%, 48% and 4% respectively. In > 1-3 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 39%, 39% and 22% respectively. In > 3-5 years follow-up group, the proportions of no significant changes, retraction, and stenosis were 30%, 35% and 35% respectively. In > 5-10 years follow-up group, the proportions of no significant changes, retraction, stenosis were 30%, 20% and 50% respectively. Compared with the ≤ 1 year group, a significant increase in the proportion of coronary artery stenosis occurred at the other three groups.Significant difference in the stenosis rate could be found between the ≤ 1 year group and the other three groups respectively (χ(2)=6.026, 11.121, 15.652; P=0.019, 0.002, 0.001). (3) The rate of retraction in bilateral GCAA group (20 cases) was lower than unilateral GCAA group (28 cases ); bilateral GCAA group had lower rate than the unilateral group(15% (3/20) vs. 36% (10/28)). There was no significant difference in coronary artery outcome between two groups (P > 0.05) . (4) There were six severe ischemic heart disease in 48 cases including 2 deaths. (5) CTA showed coronary artery wall thickening, mural thrombus and calcification, the CAG could display coronary artery occlusion and recanalization and collateral vessels formation.

CONCLUSION

KD complicated with GCAA may occur in infants under one year of age, especially infants under 6 months of age. A significant increase in the proportion of coronary artery stenosis occurred with the follow-up time extended. The proportion of bilateral GCAA patients who had ischemic heart disease and died was higher than the unilateral cases. The combined application of echocardiography, CTA and CAG may greatly help to discover coronary thrombosis, stenosis and occlusion.