Performance of 2004 American Heart Association recommendations for treatment of Kawasaki disease

Abstract

Objective: The 2004 American Heart Association (AHA) statement included a clinical case definition and an algorithm for diagnosing and treating suspected incomplete Kawasaki disease (KD). We explored the performance of these recommendations in a multicenter series of US patients with KD with coronary artery aneurysms (CAAs).

Methods: We reviewed retrospectively records of patients with KD with CAAs at 4 US centers from 1981 to 2006. CAAs were defined on the basis of z scores of >3 or Japanese Ministry of Health and Welfare criteria. Our primary outcome was the proportion of patients presenting at illness day < or =21 who would have received intravenous immunoglobulin (IVIG) treatment by following the AHA guidelines at the time of their initial presentation to the clinical center.

Results: Of 195 patients who met entry criteria, 137 (70%) met the case definition and would have received IVIG treatment at presentation. Fifty-three patients (27%) had suspected incomplete KD and were eligible for algorithm application; all would have received IVIG treatment at presentation. Of the remaining 5 patients, 3 were excluded from the algorithm because of fever for <5 days at presentation and 2 because of <2 clinical criteria at >6 months of age. Two of these 5 patients would have entered the algorithm and received IVIG treatment after follow-up monitoring. Overall, application of the AHA algorithm would have referred > or =190 patients (97%) for IVIG treatment.

Conclusions: Application of the 2004 AHA recommendations, compared with the classic criteria alone, improves the rate of IVIG treatment for patients with KD who develop CAAs. Future multicenter prospective studies are needed to assess the performance characteristics of the AHA algorithm in febrile children with incomplete criterion findings and to refine the algorithm further.

FIGURE 1

Evaluation of suspected incomplete KD. (1) In the absence of a standard method for diagnosis, this algorithm cannot be evidence-based but represents the informed opinion of the expert committee. Consultation with an expert should be sought whenever assistance is needed. (2) Infants <6 months of age on the seventh day of fever without other explanation should undergo laboratory testing and, if evidence of systemic inflammation is found, echocardiography, even if the infants meet no clinical criteria for diagnosis. (3) Characteristics suggesting KD are listed in Table 1. Characteristics suggesting diseases other than KD include exudative conjunctivitis, exudative pharyngitis, discrete intraoral lesions, bullous or vesicular rash, and generalized adenopathy. Alternative diagnoses should be considered (Table 2). (4) Supplemental laboratory criteria include albumin levels of <3.0 g/dL, anemia for age, elevation of ALT level, >450 000 platelets per mm³ after the seventh day, white blood cell count of >15 000 cells per mm³, and >10 white blood cells per high-power field in the urine. (5) Treatment can begin before echocardiography is performed. (6) Echocardiograms are considered positive for purposes of this algorithm if any of 3 conditions are met, that is, the z score for the anterior interventricular or right coronary artery is >2.5, coronary arteries meet Japanese Ministry of Health and Welfare criteria for aneurysms, or there are >3 other suggestive features, including perivascular brightness, lack of tapering, decreased left ventricular function, mitral regurgitation, pericardial effusion, or z scores for the anterior interventricular and right coronary arteries between 2 and 2.5. (7) If the echocardiogram is positive, then treatment should be given to children within 10 days after the onset of fever and to those beyond the 10th day with clinical and laboratory signs of ongoing inflammation. (8) Typical peeling begins under the nail beds of fingers and then toes. Echo indicates echocardiography; f/u, follow-up. (Reproduced with permission from Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation. 2004;110(17):1709.)

FIGURE 2

Number of clinical criteria present at the time of initial evaluation for 195 patients with KD and CAAs. Clinical criteria included rash, conjunctival injection, oral changes, lymphadenopathy, and extremity changes.

FIGURE 3

Classification and projected treatment on the basis of data obtained at the first evaluation for 195 patients with KD and CAAs with application of the 2004 AHA recommendations for the diagnosis and treatment of KD. ^a When both the ESR and CRP level were recorded, a CRP level of ≥3.0 mg/dL or ESR of ≥40 mm/hour was considered a positive marker of systemic inflammation, regardless of the other value. ^b Positive supplemental criteria included (1) white blood cell count of ≥15 000 cells per mm³, (2) anemia for age, (3) platelet count of ≥450 × 10³ cells per mm³ if fever had been present for ≥7 days at the time of presentation, (4) albumin level of ≤3.0 g/dL, (5) ALT level elevation for age, and (6) urinary white blood cell count of >10 cells per high-power field.

FIGURE 4

Classification and projected treatment on the basis of data obtained at the first evaluation for 37 infants ≤6 months of age with KD and CAAs with application of the 2004 AHA recommendations for the diagnosis and treatment of KD. ^a When both the ESR and CRP level were recorded, a CRP level of ≥3.0 mg/dL or ESR of ≥40 mm/hour was considered a positive marker of systemic inflammation, regardless of the other value. ^b Positive supplemental criteria included (1) white blood cell count of ≥15 000 cells per mm³, (2) anemia for age, (3) platelet count of ≥450 × 10³ cells per mm³ if fever had been present for ≥7 days at the time of presentation, (4) albumin level of ≤3.0 g/dL, (5) ALT level elevation for age, and (6) urinary white blood cell count of >10 cells per high-power field.