Shaima Al Maeeni, MD
Pediatric Intensivist
Sheikh Khalifa Medical City
Abu Dhabi, United Arab Emirates
Disclosure information not submitted.
musaab al ramsi, MD
Pediatric Intensivist- Division Chief
Sheikh Khalifa Medical City, United Arab Emirates
Disclosure information not submitted.
Alyaa Al Ali, MD
Pediatric intensivist
Sheikh Khalifa Medical City
Abu Dhabi, United Arab Emirates
Disclosure information not submitted.
Title: Systemic thrombolysis and ECPR for fatal MIS-C with extensive coronary thrombosis and cardiac arrest
Case Report Body:
Introduction: Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe complication of coronavirus disease 2019 in children. These children present with symptomatology similar to Kawasaki disease (KD) or toxic shock syndrome. Compared to KD, MIS-C is associated with a greater degree of cardiac involvement, with variable severity. Herein, we report a case of fatal MIS-C with giant coronary artery aneurysms complicated myocardial infraction and extensive thrombosis, despite intravenous immunoglobulin (IVIG), steroids, and immunomodulators. Extracorporeal cardiopulmonary resuscitation (ECPR) and thrombolytic therapy were utilized to re-establish myocardial perfusion and prevent death.
Description: A 7-month-old previously healthy boy, presented with a fever, cough, rash and diarrhea for 3 days during the COVID pandemic. Clinical examinations showed bilateral bulbar conjunctival injection, maculopapular rashes, cracking of lips, strawberry tongue, erythema and edema of the hands and feet. The clinical and biochemical findings were consistent with MIS-C and fulfilled the CDC MIS-C diagnostic criteria. He was treated with IVIG and high dose aspirin. Initial Echocardiogram was normal. On day 7, echocardiogram demonstrated a small aneurysm in RCA, LCA and normal cardiac function. Thus, he was started on pulse steroids, enoxaparin and clopidogrel. A follow up echocardiogram after 48 hours showed a giant aneurysm in RCA, LCA and an impaired LV function with no flow in LCA. Infliximab was added. ECG showed elevated ST segments. Urgent aggressive thrombolysis was initiated. Within 6 hours, he developed junctional arrhythmia and sudden cardiac arrest, so ECPR was commenced. Course was complicated with a stroke and acute renal failure requiring CRRT for 6 weeks. On a 6 month follow up, he had stable ischemic cardiomyopathy with an ejection fraction of 15% on oral afterload agents, normal renal function, and a score 4 of Modified Rankin Scale severe disability.
Conclusion: To date, there have only been a few reported cases of giant coronary artery aneurysms complicated with extensive thrombosis. The incidence of coronary artery abnormalities varies in most reports with mostly small aneurysms. Until further evidence emerges, timely high dose IVIG and steroids are imperative to avoid such a dreadful complication of MIS-C.