Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry

Abstract

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD. Methods and Results A 34-institution international registry of 1651 patients with KD who had CAAs (maximum CAA Z score ≥2.5) was used. Time-to-event analyses were performed using the Kaplan-Meier method and Cox proportional hazard models for risk factor analysis. In patients with CAA Z scores ≥10, the cumulative incidence of luminal narrowing (>50% of lumen diameter), coronary artery thrombosis, and composite major adverse cardiovascular complications at 10 years was 20±3%, 18±2%, and 14±2%, respectively. No complications were observed in patients with a CAA Z score <10. Higher CAA Z score and a greater number of coronary artery branches affected were associated with increased risk of all types of complications. At 10 years, normalization of luminal diameter was noted in 99±4% of patients with small (2.5≤Z<5.0), 92±1% with medium (5.0≤Z<10), and 57±3% with large CAAs (Z≥10). CAAs in the left anterior descending and circumflex coronary artery branches were more likely to normalize. Risk factor analysis of coronary artery branch level outcomes was performed with a total of 893 affected branches with Z score ≥10 in 440 patients. In multivariable regression models, hazards of luminal narrowing and thrombosis were higher for patients with CAAs of the right coronary artery and left anterior descending branches, those with CAAs that had complex architecture (other than isolated aneurysms), and those with CAAs with Z scores ≥20. Conclusions For patients with CAA after KD, medium-term risk of complications is confined to those with maximum CAA Z scores ≥10. Further risk stratification and close follow-up, including advanced imaging, in patients with large CAAs is warranted.

Keywords: Kawasaki disease; cardiovascular outcomes; coronary artery; risk factors.

Figure 1. Cumulative incidence calculated using Kaplan–Meier survival analysis of complications for patients with coronary artery aneurysms after Kawasaki disease in patients with at least 1 lesion with a coronary artery Z score ≥10.

Dotted lines represent the estimated standard error around the cumulative incidence.

Figure 2. Cumulative incidence of luminal narrowing, coronary artery thrombosis, and normalization of luminal diameter, calculated using Kaplan–Meier survival analysis, for coronary artery aneurysms after Kawasaki disease in coronary artery branches with coronary artery Z score ≥10 stratified by coronary artery branches.

LAD indicates left anterior descending coronary artery; LCX, left circumflex artery; LMCA, left main coronary artery; RCA, right coronary artery; and maximum coronary artery Z scores.

Figure 3. Cumulative incidence of normalization of luminal diameter, calculated using Kaplan–Meier survival analysis, for coronary artery aneurysms (CAAs) after Kawasaki disease stratified by level of coronary artery involvement.