Abstract
Coronary artery ectasia is the most common coronary artery lesion associated with Kawasaki disease (KD), and is felt to regress with no long-term sequelae. We sought to determine the natural history of ectasia and associated factors. From 1990-2001, 143 patients (69% male) were identified with sufficient echocardiographic documentation of ectasia. The median age at KD was 2.7 years (range, 2.3 months to 15 years), with 73% receiving IVIG. Aneurysms were also present in 31% (11% with giant aneurysms). Serial measurements of coronary artery dimensions of non-aneurysmal proximal segments were converted to Z scores based on body surface area (BSA). Patients had a median of 4 echocardiograms each (range, 2 to 15) over a median interval of 1.6 years (range up to 9.8 years). Mixed linear regression was used to determine trends and factors associated with the serial Z scores for the right (zRCA), left main (zLMCA) and left anterior descending (zLAD) coronary arteries. Initial mean (SD) zRCA was 0.60 (1.07), zLMCA 0.99 (1.28) and zLAD 0.87 (1.40). There were no significant trends in Z scores over time, indicating a lack of regression or progression when measurements were normalized for BSA. Further, no trend was noted when analysis was limited to arteries with initial Z scores above 2. Higher zRCA was independently related to no IVIG treatment (p=0.08), higher initial zRCA (p=0.001), lower initial zLMCA (p=0.001), and higher zLAD (p=0.001). Higher zLMCA was independently related to male gender (p=0.01), presence of any aneurysms (p=0.01), higher initial zLMCA (p=0.03), lower initial zRCA (p=0.07) and higher zLAD (p=0.001). Higher zLAD was independently related to higher initial zLAD (=0.001), higher initial zLMCA (p=0.03) and higher zRCA (p=0.001). In conclusion non-aneurysmal coronary artery segments appear to grow proportionately, and subsequent dimensions are related to initial dimensions and dimensions of other segments.
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Pyle, A., McCrindle, B. & Yeung, R. Natural History of Coronary Artery Ectasia in Children with Kawasaki Disease. Pediatr Res 53, 176 (2003). https://doi.org/10.1203/00006450-200301000-00134
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DOI: https://doi.org/10.1203/00006450-200301000-00134