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AB1102 Kawasaki disease and giant aneurysm in mexican children: evolution and clinical characteristics: a 5-year experience
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  1. M.T. Brana1,
  2. S. Osorio2,
  3. L. Aparicio2,
  4. A. Rodriguez1,
  5. E. Faugier2,
  6. R. Maldonado2
  1. 1Pediatric Rheumatology
  2. 2Hospital Infantil de Mexico, Federico Gomez, Hospital Infantil de Mexico Federico Gomez, Mexico, Mexico

Abstract

Background Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis, predominantly involving medium-sized arteries. It mainly affects children younger than five years and it is the leading cause of acquired heart disease in children in developed countries. Of unknown pathogenesis, KD severe complication is the occurrence of coronary artery lesions. Without early treatment, there is a 15% to 25% incidence of coronary artery lesions. Management with intravenous immunoglobin (IVIG), combined with aspirin, effectively decrease the incidence of this lesions to a 4%. The long-term prognosis is determined by the initial and current level of coronary artery involvement. Methods to predict which children are at higher risk for coronary aneurysms have been sought to determine prognosis and select patients for more rigorous treatment and follow-up.

Objectives To describe the clinical presentation and evolution in addition to laboratory findings in Mexican paediatric population who developed giant aneurysms diagnosed with KD during the past 5 years. By identifying mayor risk factors in our population, an effective score could be used to select children for evaluation of additional therapies to prevent coronary artery aneurysms that occur despite treatment with IVIG.

Methods Retrospective cohort study of the Children’s Hospital of Mexico Federico Gomez, last 5 years. We reviewed the data form the clinical archives of the patients who developed giant aneurysms after the diagnosis of KD from 2011 to 2016. A total of 84 patients with KD, 7% developed giant aneurysms. The variables analysed, apart from the typical clinical and laboratory findings of KD, include size and Z score of the aneurysms, involution through follow up, cardiac morbidity and mortality, and treatment strategy.

Results: Results The mean age of patients at diagnostic was 17 months, and 84% were males. Only 33% of the patients developed complete KD, while 66% were diagnosed as incomplete. All patients presented with a positive Harada score. IVIG was administer in 83% of the patients, and a second dose was needed in 33%. Infliximab was used in 33% of the patients. One patient died due to cardiogenic shock. Results from echocardiography in the follow-up show that 33% of the patients have evolved to even larger aneurysms and 50% present no changes. Of the patients with a longer follow-up, 4 years after diagnostic, 33% have developed arrhythmias and 16% myocardial infraction. All are at high risk of sudden death.

Conclusions The late diagnosis is the characteristic present in all patients which developed giant aneurysms, making imperative to identify clinical and laboratory findings that will help identify KD in Mexican paediatric population to avoid cardiac complications.

Reference [1] Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Brian W. McCrindle, et al. Circulation. published online March 29, 2017; Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2017 American Heart Association.

Disclosure of Interest None declared

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