Outcomes Associated with Giant Coronary Artery Aneurysms after Kawasaki Disease: A Single-Center United States Experience

• Published in: The Journal of pediatrics Vol. 274; p. 114145
• Main Authors: Elias, Matthew D., Brothers, Julie A., Hogarty, Alexa N., Martino, Jordy, O'Byrne, Michael L., Patel, Chandni, Stephens, Paul, Tingo, Jennifer, Vetter, Victoria L., Ravishankar, Chitra, Giglia, Therese M.
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.11.2024
• Subjects: coronary artery aneurysms; Kawasaki disease; major adverse cardiovascular events; outcomes; ECMO; CAA; MRI; IVIG; LAD; RCA; LIMA; Kawasaki disease; outcomes; MACE; KD; OHT; coronary artery aneurysms; major adverse cardiovascular events; CABG; LMCA; MI; CA
• ISSN: 0022-3476; 1097-6833; 1097-6833
• DOI: 10.1016/j.jpeds.2024.114145

To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAAs) at a single US center. Medical records for all patients with KD and giant CAAs at a pediatric academic institution were reviewed. Primary outcomes included major adverse cardiovascular events (MACE) and normalization of CA luminal diameter, using Kaplan-Meier analyses. There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with a median age at diagnosis of 0.9 years (range, 0.2-13.3 years). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 patients (21.7%) at a median of 1.4 years (range, 0.04-22.6 years) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z scores of ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to a normal luminal diameter at a median of 3.6 years (range, 0.6-12.0 years). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z scores of ≥20. Despite regression to a normal luminal diameter in >25% of CAAs, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.