Kawasaki disease – experience of Pediatric University Hospital, Sofia, Bulgaria, 1993–2014. Part II: cardiovascular manifestations and treatment

Abstract

Kawasaki disease (KD) is a childhood vasculitis syndrome and the coronary arteries are the main target of the vascular damage. The outcome is formation of coronary lesions (CL) that develop in 20%-25% of untreated children. KD is the leading cause of myocardial infarction in infancy. Its consequences among young people are acute coronary syndrome and susceptibility to early atherosclerosis, if the disease were to remain unrecognized. We investigated the cardiac manifestations during the acute phase of the disease, the values of the fever, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) and the therapeutic factors that created increased coronary risk in our cohort of patients. The study is retrospective . In the cohort (n = 107), 30.8% had coronary lesions, including 19.6% coronary aneurysms and 11.2% significant dilatations. We found association between myocarditis, papillary and left ventricular dysfunction in the acute phase of KD and the risk of coronary aneurysms (p < 0.001). The expressively elevated CRP levels and the persistent fever during the early subacute phase correlated significantly with coronary risk (p < 0.002). The treatment with intravenous immunoglobulin (IVIG) reduced the risk of coronary aneurysms 4.8 times (p < 0.002). A follow-up was performed in 53 children with coronary lesions during the first year of the disease. A longitudinal follow-up was performed in 38 patients. Their results indicate that cardiac monitoring is obligatory for all patients who have experienced KD.