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Kawasaki Disease Vaishali Soneji Lafita, MD

Kawasaki Disease Vaishali Soneji Lafita, MD. Presentation – Patient 1. 10 years old male with Kawasaki Disease Possible mildly ectatic posterior descending coronary artery on Echo. CTA was performed. Heart rate control Contrast enhanced axial images of the coronary arteries

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Kawasaki Disease Vaishali Soneji Lafita, MD

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  1. Kawasaki DiseaseVaishali Soneji Lafita, MD

  2. Presentation – Patient 1 • 10 years old male with Kawasaki Disease • Possible mildly ectatic posterior descending coronary artery on Echo. • CTA was performed. • Heart rate control • Contrast enhanced axial images of the coronary arteries • Multiplanar and 3D reformats

  3. CTA – Patient 1

  4. CTA – Patient 1

  5. CTA – Patient 1

  6. CTA – Patient 1

  7. Presentation – Patient 2 • 13 years old male with Kawasaki Disease and known coronary aneurysm presents for CTA. • Heart rate control • Contrast enhanced axial images of the coronary arteries • Multiplanar and 3D reformats

  8. CTA – Patient 2

  9. CTA – Patient 2

  10. CTA – Patient 2

  11. Presentation – Patient 3 • 13 years old female with Kawasaki Disease and known coronary aneurysm diagnosed on Echocardiogram presents for CTA.

  12. CTA – Patient 3

  13. Kawasaki Syndrome • AKA – Mucocutaneous Lymph Node Syndrome • First characterized in 1967 by Dr. Tomisaku Kawasaki of Tokyo • Multisystem vasculitis • Self limited • Leading cause of acquired heart disease in the US.

  14. Epidemiology • Syndrome of young children • >80% are < 4y/o • Most are between 1-2y/o • Very rare > 8y/o • Males > females • Japanese and Korean ancestry > other ethnicities • 4,000-8,000 cases/yr

  15. Etiology • Unknown • Leading hypothesis • Infectious with immune-mediated reaction • No single etiologic agent identified

  16. Clinical Manifestationand Diagnosis • No recognized prodrome • Acute onset • Begins with fever • Other symptoms begin within 2-5 days • Syndrome • Fever for 5d + 4/5 symptoms • Fever for 5d + 3/5 symptoms + coronary artery abnormalities

  17. Signs/symptoms • Nonpurulent bilateral conjunctivits • Oropharyngeal changes • Erythema, redness, cracking, peeling, injected pharynx, strawberry tongue • Polymorphous erythematous rash • Most evident with fever • Primarily on trunk, may be pruritic • Cervical lymphadenopathy • Often unilateral • One node >1.5cm • Feet/Hand changes • Erythema (palms/soles), edema, induration, desquamation

  18. Signs/Symptoms • Cardiac findings • Leading cause of morbidity and mortality • May involve pericardium, myocardium, endocardium, valves, coronary arteries

  19. Signs/Symptoms • 20-25% of untreated pts develop coronary artery abnormalities/aneurysms • Within 4 weeks of onset • Dilation can be detected at 10d • Peak 18-25d • Giant aneurysm >8mm • Greatest risk thrombosis, stenosis, MI

  20. Complications • Cardiac manifestations can be deadly • Most deaths occur between 2-12 weeks post illness onset • Coronary aneurysm thrombosis – MI • Aneurysm rupture • Myocarditis - CHF

  21. PredictingCoronary Artery abnormalities • Harada score • White blood cell count > 12K • Platlet count < 350K • CRP >3+ • Hct < 35% • Albumin < 3.5 g/dL • Age < 12 months • Male sex • Used to determine management

  22. Treatment • Decrease inflammation • Prevent thrombosis • Overall goal • Protect the myocardium and coronary arteries • Modes of treatment • IVIG • Steroids • TNF –alpha antagonists • Aspirin • abciximab

  23. Cardiac findings : Imaging • Echocardiography • Unclear sensitivity and specificity • Coronary Angiography • Intravascular Ultrasound (IVUS) • Transesophageal echocardiography • MRA • CTA

  24. Cardiac findings :Imaging • Echocardiogram • 1st at time of diagnosis • Number, location and classification of aneurysms • Small, medium or giant • Repeat echo 2-3 and 6-8 weeks after onset of the disease • If both normal – no further echos needed • If abnormal • Severity determines frequency and type of follow-up

  25. Cardiac findings :Imaging

  26. Management of Aneurysms • Small to medium:long term Aspirin, no activity restriction, stress test in older children if suggests stenosis→ coronary angiography • Multiple, small to medium or giant: Aspirin with/without warfarin, >10y stress test with myocardial perfusion scan if coronary obstruction→ bypass grafting, angioplasty

  27. Summary • Self limited systemic vasculitis • Leading cause of acquired heart disease in the US. • Morbidity and mortality from cardiac involvement. • Imaging plays critical role in diagnosis and management of cardiac involvement.

  28. References • J. W. Newburger, M. Takahashi, M. A. Gerber, M. H. Gewitz, L. Y. Tani, J. C. Burns, S. T. Shulman, A. F. Bolger, P. Ferrieri, R. S. Baltimore, W. R. Wilson, L. M. Baddour, M. E. Levison, T. J. Pallasch, D. A. Falace, and K. A. TaubertDiagnosis, 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 AssociationPediatrics, December 1, 2004; 114(6): 1708 - 1733. • http://www.emedicine.com/EMERG/topic811.htm • http://www.cdc.gov/ncidod/diseases/kawasaki/index.htm • http://www.americanheart.org/presenter.jhtml?identifier=11163

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