What are the clinical, laboratory, and echocardiographic characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease?
Kawasaki disease shock syndrome is a severe phenotype associated with greater inflammation, myocardial dysfunction, coronary artery abnormalities, and resistance to standard immunoglobulin therapy.
OBJECTIVE: We sought to define the characteristics that distinguish Kawasaki disease shock syndrome from hemodynamically normal Kawasaki disease. METHODS: We collected data prospectively for all patients with Kawasaki disease who were treated at a single institution during a 4-year period. We defined Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of > or =20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indices of ventricular systolic and diastolic function during acute and convalescent Kawasaki disease. RESULTS: Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the definition for Kawasaki disease shock syndrome. All received fluid resuscitation, and 7 (54%) required vasoactive infusions. Compared with patients without shock, patients with Kawasaki disease shock syndrome were more often female and had larger proportions of bands, higher C-reactive protein concentrations, and lower hemoglobin concentrations and platelet counts. Evidence of consumptive coagulopathy was common in the Kawasaki disease shock syndrome group. Patients with Kawasaki disease shock syndrome more often had impaired left ventricular systolic function (ejection fraction of <54%: 4 of 13 patients 31% vs 2 of 86 patients 4%), mitral regurgitation (5 of 13 patients 39% vs 2 of 83 patients 2%), coronary artery abnormalities (8 of 13 patients 62% vs 20 of 86 patients 23%), and intravenous immunoglobulin resistance (6 of 13 patients 46% vs 32 of 174 patients 18%). Impairment of ventricular relaxation and compliance persisted among patients with Kawasaki disease shock syndrome after the resolution of other hemodynamic disturbances. CONCLUSIONS: Kawasaki disease shock syndrome is associated with more-severe laboratory markers of inflammation and greater risk of coronary artery abnormalities, mitral regurgitation, and prolonged myocardial dysfunction. These patients may be resistant to immunoglobulin therapy and require additional antiinflammatory treatment.
Kanegaye et al. (2009) studied this question.
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