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 ≤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-Of195patients 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 ≥190 patients (97%) for IVIG treatment. CONCLUSIONS-Application of the 2004AHA 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.Address correspondence to Elizabeth S. Yellen, MD, Children's Hospital Boston, Department of Cardiology, 300 Longwood Ave, Boston, MA 02115. elizabeth.yellen@cardio.chboston.org. FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. NIH Public Access Author ManuscriptPediatrics. Author manuscript; available in PMC 2010 July 2. In 2004, the American Heart Association (AHA) published a statement on the diagnosis, treatment, and long-term management of KD, which subsequently was endorsed by the American Academy of Pediatrics. 5 The report included an algorithm to aid clinicians in the evaluation of patients with suspected KD who do not meet the complete case definition and thus may not be identified or receive timely referral for IVIG treatment. The AHA algorithm for patients with suspected incomplete KD was based on expert opinion and anecdotal reports, rather than large clinical trials or registries. In a retrospective, multicenter study, we sought to assess the performance of the AHA recommendations for IVIG treatment for children with CAAs attributable to KD. METHODS SubjectsWe performed a retrospective chart review of data for childre...

Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

“…92 Studies evaluating the incomplete KD diagnostic algorithm first proposed in the 2004 guidelines 1 suggest its usefulness in identifying patients who require treatment and in preventing coronary artery aneurysms. 93,94 Incomplete KD occurs most commonly in infants, who are at substantial risk of developing coronary artery abnormalities and who may have prolonged fever as the sole clinical finding or have subtle or fleeting clinical signs in addition to fever. Laboratory findings and cardiovascular sequelae in incomplete and complete cases appear the same.…”

Coronary Artery Dimensions in Febrile Children Without Kawasaki Disease

“…Seventeen patients (40%) defined their race or ethnicity as white, 10 (23%) as Hispanic, 7 (16%) as black, 3 (7%) as Asian, 1 (2%) as Indian, 1 (2%) as other, and 4 (9%) were unknown. The median duration of fever at the time of the echocardiogram was 6 days (range, [4][5][6][7][8][9][10][11][12][13][14]. Their diagnoses were pneumonia (n=9), osteomyelitis (n=8), urinary tract infection (n=5), viral syndrome (n=5), Epstein-Barr virus infection (n=3), cytomegalovirus infection (n=2), bronchiolitis (n=2), and 1 each with a diagnosis of fever of unknown origin, croup, stomatitis, gastroenteritis, influenza, toxin-mediated illness (deemed by a KD expert to be not consistent with KD), scarlet fever, purulent otitis media, and periorbital cellulitis.…”

Atypical Kawasaki Disease in 2-Months Old Infant Presenting with Aseptic Meningitis

“…For these reasons, it is recommended that infants younger than six months of age who have had more than seven days of fever of unknown aetiology and elevated inflammatory markers, to undergo echocardiography [11].…”