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ST - segment Elevation Myocardial Infarction complicating an atypical Kawasaki disease Raluca PRISECARU, Marc VINCENT, Steven VERCAUTEREN Brussels Heart Center, Brussels, Belgium Disclosure None Clinical case • • • • • • 20 years old female for typical chest pain (<2h) Aseptic meningoencephalitis 4 weeks ago No risk factors for coronary artery disease Absence of recent trauma/ contusion Deny cocaine abuse/ contraceptive medication Physical examination: normal BP and tachycardia Clinical case • ECG: anterior ST-elevation. • Troponine level: 19.97 ng/ml (N:0 – 0.1 ng/ml) • TTE: - systolic dysfunction- LVEF at 40% - antero septal akinesia On the way to the Cathlab… • • • • • • • Hematological disorders Dissection of coronary artery Coronary spasm Tako Tsubo cardiomyopathy Congenital abnormality of coronary arteries Diseases involving intimal proliferation Arteritis occlusion at the mid segment of LAD Stenosis at mid and distal right coronary artery (RCA). Fusiforme coronary aneurysms. Coronary arteriography • occlusion at the mid segment of LAD (left anterior descending artery) • non significant stenosis at proximal circumflex artery, mid and distal right coronary artery • multiple fusiforme coronary aneurysms with internal diameter between 2-7 mm. An intracoronary thrombus aspiration was performed and the left anterior descending artery was recanalized with restoration of a TIMI 2 flow Coronary aneurysms 1. Congenital aneurisms 2. Acquired aneurisms No RF for atherosclerosis No trauma No cocaine abuse Vasculitis ?? Vasculitis? - Polyarteritis nodosa - Giant cell arteritis - Takayasu arteritis - Kawasaki disease - Churg-Strauss syndrome - Granulomatosis with polyangiitis (Wegener’s) - Hypersensitivity vasculitis - Henoch-Schönlein purpura - Vasculitis secondary to connective tissue disorders - Vasculitis secondary to viral infection….. It’s time for a diagnosis… The diagnosis of ST - segment Elevation Myocardial Infarction complicating a Kawasaki disease was established, based on: - multiple aneurysms - multiple stenosis - aseptic meningo encephalitis A 48 occlusion LAD h coronary and dissection arteriography: occlusion of LAD associated with dissection LAD occlusion and dissection Treatement: drug eluting stent placement. A 48 h coronary arteriography: After drug eluting stent placement. She was discharged with the following treatment prescription: - long-term duble antiplatelet therapy with aspirine and clopidogrel - β-adrenergic–blocking drug - ACE inhibitor - statine. After 1 month • The patient was asymptomatic • The ultrafast computed tomography (CT): persistent aneurysmatic lesions on both principal and secondary arteries. • The ETT: - persistent systolic dysfunction (LVEF at 40%) antero septal wall akinesia. The Kawasaki disease • Acute, small-to-medium-vessel vasculitis of unknown etiology • Preferentially affects children • Meningitis may be in seldom cases a feature of KD (subacute phase). • No specific laboratory tests Major clinical criterias • • • • • Fever > 5 days Bilateral conjunctivitis Rash Erythema of the lips and oral mucosa Cervical lymphadenopathy. Diagnosis in KD • >=4/5 of main clinical criteria • <4/5 of main clinical features + coronary artery disease detected on angiography. • <4/5 of main clinical features + coronary artery disease detected on ETT Cardiac complications of KD • Coronary aneurysms leading to - myocardial ischemia - myocardial infarction - sudden cardiac death • Decreased myocardial contractility • Coronary arteritis without aneurysms • Mild valvular regurgitation • Pericardial effusion Coronary artery aneurysms • Develop in 15% to 25% of children with untreated KD • Evolves during the subacute phase (D 10 to 40 after onset of fever) when dinamics and mortality are highest • Involve multiple vessels - proximal segments and bifurcations Extracardiac complications • • • • • Peripheral arterial obstruction Urinary abnormalities and renal disease wide variety of gastrointestinal manifestations Persistent sensorineural hearing loss Behavioral changes Prognosis • Mortality rate of KD is low (0.1 to 0.3%) • Low rate of recurrence for KD (commonly occurred within the first 12 months after the initial episode of KD ) • Long-term morbidity related to CA involvement: – best prognosis: fusiform aneurysms <8 mm in diameter (generally regresse over time ) – giant aneurysms - highest risk of morbidity and mortality (thrombosis and stenoses at the proximal and/or distal ends of the aneurysms). Problems in management of KD 1. Onset of KD in adults: - rare - frequently atypical 2. The diagnosis presents more difficulties and can easily be missed 3. Atypical forms of KD seem to predict a higher risk to coronary abnormalities Conclusion • Major complication of KD: CA aneurysms • Myocardial infarction- main cause of death in KD • Although the incidence of acute myocardial infarction in pediatric or young patients is low , the diagnosis of KD should be considered as an underlying cause. Thank you!