Late Coronary Aneurysm Formation after Kawasaki Disease: a Review of Mechanistic and Clinical Aspects

Abstract

Author's summary Evolution of coronary artery aneurysms (CAAs) has been an ominous clinical finding in patients with Kawasaki disease (KD). CAAs generally emerge within the first few weeks in patients with KD, and appear to be strongly associated with the failure to timely initiate specific management strategies. CAA formation might occasionally arise as a late-onset phenomenon long after the index KD episode. Late CAAs in the setting of KD have particular mechanistic and clinical characteristics. Late CAAs in the setting of KD may be confused with other CAA types (atherosclerotic, etc.), and are possibly underdiagnosed in clinical practice.

Multisystem Inflammatory Syndrome Versus Kawasaki Disease: Potential Differences in Pathogenetic and Clinical Implications

“… 2 Elevation of activated neutrophil count and serum cytokine levels generally account for clinical and associated pathological findings (including necrotizing arteritis) in the setting of KD. 3 , 4 Notably, serum levels of IL-8 and IL-10 were previously reported to be higher in the setting of MIS-C compared with KD. 2 As an important outcome, IL-8 might potentially trigger a strong prothrombotic response.…”

“… 2 , 5 Importantly, KD might also be emerge in children with a previous SARS-CoV-2 infection. 2 Notably, children with KD are generally younger (mostly infants and young children) 3 , 4 than those with MIS-C, potentially suggesting differential impact of age-related factors on the interaction between certain antigenic triggers and immune response. 2 Taken together, a recent SARS-CoV-2 infection (3-6 weeks earlier) most likely suggests MIS-C, particularly in relatively older children with specific clinical findings; yet this might not completely rule out a coincidental KD episode.…”

“… 2 - 4 , 6 , 7 Coronary vasculitis in KD might potentially end up with CAA formation and coronary stenosis, particularly in those who do not receive particular disease-modifying agents including intravenous immunoglobulin (IVIG) in a timely manner (within the first 10 days following fever onset) or in IVIG nonresponders. 3 , 4 , 6 , 7 Interestingly, patients with KD who are managed properly and have normal coronary arteries on initial coronary imaging might occasionally suffer from CAAs that attain their maximum size by about 6 weeks following disease onset. 3 , 6 These notions might also apply to patients with MIS-C despite the lower incidence of coronary sequelae in these patients.…”

“… 3 , 4 , 6 , 7 Interestingly, patients with KD who are managed properly and have normal coronary arteries on initial coronary imaging might occasionally suffer from CAAs that attain their maximum size by about 6 weeks following disease onset. 3 , 6 These notions might also apply to patients with MIS-C despite the lower incidence of coronary sequelae in these patients. 2 Accordingly, did the authors perform serial coronary imaging on echocardiogram (following initial evaluation) in terms of CAA formation during the first few weeks?…”

Spontaneous Coronary Artery Dissection in the Setting of Duchenne Muscular Dystrophy: More Questions Than Answers

“…It is well known that steroids might potentially exert a detrimental impact on vascular structures leading to vascular frailty that might result in potential complications including coronary aneurysm formation in susceptible subjects. 6 Importantly, ataluren, the disease-modifying agent in the setting of DMD, might potentially enhance muscle dystrophin expression, yet might have potential side effects including vomiting, hypertension, etc., 7 that all might have facilitated the evolution of SCAD (through mechanisms including Valsalva strain, direct vascular injury, etc.) 4 in the patient.…”