Medium‐Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry
Authors: McCrindle Brian W. , Manlhiot Cedric , Newburger Jane W. , Harahsheh Ashraf S. , Giglia Therese M. , Dallaire Frederic , Friedman Kevin , Low Tisiana , Runeckles Kyle , Mathew Mathew , Mackie Andrew S. , Choueiter Nadine F. , Jone Pei‐Ni , Kutty Shelby , Yetman Anji T. , Raghuveer Geetha , Pahl Elfriede , Norozi Kambiz , McHugh Kimberly E. , Li Jennifer S. , De Ferranti Sarah D. , Dahdah Nagib , Altman Carolyn A. , Anderson Brett R. , Beaulieu Emilie , Boychuk Carolyn E. , Braunlin Elizabeth , Burns Jane C. , Carr Michael R. , Crean Andrew , Colyer Jessica H. , Dempsey Adam , Desjardins Laurent , Dillenburg Rejane , Dionne Audrey , Ferris Anna , Gewitz Michael , Grcic Michelle M. , Greenway Steven C. , Harris Kevin C. , Hayden‐Rush Christina , Hill Kevin D. , Jain Supriya , Kimball Thomas R. , Lang Sean M. , Lin Ming‐Tai , Mahle William T. , Mondal Tapas , Portman Michael A. , Renaud Claudia , Sexson Tejitel S. Kristen , Szmuszkovicz Jacqueline R. , Texter Karen M. , Thacker Deepika , Tierney Elif Seda Selamet , Thomas Thomas , Tremoulet Adriana H. , Wagner‐Lees Sharon , Warren Andrew 
Published: July 2020

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.