RESUMO
OBJECTIVES: 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. STUDY DESIGN: 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. RESULTS: 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%. CONCLUSIONS: 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.
RESUMO
Prolonged Casualty Care (PCC) is a major US military research focus area. PCC is defined as the need to provide patient care for extended periods when evacuation or mission requirements surpass capabilities and/or capacity. US military experts have called for more data relevant to PCC. In response, we aimed to develop an innovative research model using a tiered system of trauma care in the Western Cape of South Africa as a framework for studying relevant US military trauma care and outcomes in a natural prolonged care environment. The objective of this report is to describe the research model and to illustrate how various components of the model may be helpful to provide data relevant to US military PCC. To develop the model, we used a combination of published data, open access reports, and expert opinion to identify, define, and compare relevant components of the Western Cape trauma system suitable for researching aspects of US military PCC. Several key features of the research model are as follows: In the Western Cape, patients are referred from primary and secondary to tertiary facilities (analogous to escalating capabilities by advancing roles of care in the US military). Western Cape civilian trauma providers' capabilities range from prehospital basic life support to definitive trauma surgical and critical care (comparable to US military Tactical Combat Casualty Care to advanced definitive surgical care). Patterns of injuries (e.g., high rates of penetrating trauma and hemorrhagic shock) and prolonged times from injury to definitive surgical care in the Western Cape system have relevance to the US military. This civilian research model for studying PCC is promising and can inform US military research. Importantly, this model also fills gaps in the South African civilian system and is useful for other prolonged trauma care communities worldwide.