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Acute Intensification May Reduce Persistent Coronary Artery Damage from Kawasaki Disease in Infants Younger Than 6
Kirsten B Dummer1, Samuel R Dominguez2, Michelle Grenier3
1Division of Cardiology, Department of Pediatrics, University of California San Diego/Rady Children's Hospital, San Diego, CA.
Insights
Intensifying initial therapy for infants under 6 months with Kawasaki disease (KD) appears to prevent severe coronary artery aneurysms (CAA). Most infants treated within 10 days showed only mild or no CAA by one year.
Area of Science:
- Pediatrics
- Cardiology
- Immunology
Background:
- Kawasaki disease (KD) is a critical illness in infants, potentially leading to coronary artery aneurysms (CAA).
- Early diagnosis and treatment are crucial to prevent cardiac complications in young children with KD.
Purpose of the Study:
- To evaluate the outcomes of infants under 6 months with KD who received intensified initial therapy versus standard therapy.
- To characterize the incidence and severity of coronary artery aneurysms (CAA) in this vulnerable population.
Main Methods:
- A retrospective cohort study included 71 infants under 6 months diagnosed with KD.
- Data on demographics, laboratory values, treatment (including intensified therapy with intravenous immunoglobulin (IVIG) plus additional agents), and echocardiogram results were collected.
- Intensified therapy was defined as any therapy beyond initial IVIG.
Main Results:
- Of 71 patients, 79% received intensified therapy. Coronary artery aneurysms (CAA) were present in 46.5% on initial echocardiograms within 24 hours of IVIG.
- Six additional patients developed CAA later. Ten patients on intensified therapy developed medium or giant aneurysms, with 6 remodeling completely.
- Of 15 patients with normal initial echocardiograms who did not receive intensification, 13 remained normal, and 2 had transient mild dilation. Only 7% had residual CAA at 1-year follow-up, none with Z-score > +3.
Conclusions:
- In this observational study, infants under 6 months with KD treated within 10 days of fever onset and receiving intensified therapy showed no more than mild CAA by 1 year.
- These findings suggest that intensified therapy may be beneficial in preventing severe cardiac sequelae in young infants with KD.
- Larger, multicenter studies are warranted to confirm these results and establish causal relationships.
Objective:
To characterize outcomes in patients <6 months old with Kawasaki disease who did or did not receive intensification of initial therapy.
Study Design:
A retrospective cohort study of infants <6 months admitted to Rady Children's Hospital San Diego (n = 48) and Children's Hospital Colorado (n = 23) between January 1, 2014, and January 12, 2023, with Kawasaki disease diagnosed by American Heart Association criteria within 10 days of fever onset. Data included demographics, laboratory values, treatment, and echocardiogram results. Intensified therapy was defined as at least 1 therapy in addition to intravenous immunoglobulin.
Results:
Of 71 patients, 33 (46.5%) had an initial echocardiogram obtained within 24 hours of intravenous immunoglobulin that demonstrated coronary artery aneurysms (CAA) (z score of the left anterior descending or right coronary artery ≥+2.5). CAA subsequently developed in an additional 6 patients. In total, 79% of patients received intensified therapy, most commonly infliximab (98%). Ten patients who received intensified therapy developed medium or giant aneurysms of whom 6 remodeled completely during the study period. Of the 15 patients with initially normal findings on echocardiogram who did not receive intensification, 13 remained normal and 2 developed transient, mild CA dilatation (z score ≥+2 and <+2.5). Echocardiograms closest to 1-year follow-up demonstrated 5 (7%) patients with residual CAA, none with z score >+3.
Conclusions:
In this observational series, no infants <6 months treated within 10 days of fever onset with intensification of initial therapy had more than mild CAA by 1 year after illness onset. Larger, multicenter studies are needed to support causal inference.
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