RESUMO
Introduction: We want to present the cardiac implication in PMIS, a new and serious SARS COV2 pandemic entity, that has similar characteristics with Kawasaki disease(KD) or Toxic Shock Syndrome and may develop a severe and potential fatal Macrophage Activation Syndrome(MAS), targeting major organs. Methods: During pandemic, 7 patients were admitted into our clinic with prolonged unremitted fever and different clinical onset, 4 female and 3 males, with ages between 4 mo and 16 yo. All patients underwent clinical examination, ECG, serial Echocardiography, abdominal ultrasound, Cardio-pulmonary X ray or CT, complex lab tests. Two tested positive for COVID antibodies. S66 Cardiology in the Young: Volume 32 Supplement 1 Results: Two patients developed incomplete Kawasaki-like disease with slight dilatation of the coronary arteries and thrombocytosis over 1 million/mm3, treated with IGIV and Aspirin. PIMS with severe MAS, a rare complication was present in 5 patients admitted for fever and: diarrhea(1), rash(2), aseptic meningitis(1), encephalitis, seizures and coma(1), ground glass pneumonia(5), generalized purpura(1), appendicitis(2), ascites(1), colonic abscesses(1), arthritis mimicking Systemic Juvenile Arthritis(1), symptoms presented in different combinations, targeting 2 or 3 organs. Hepato-splenomegaly was palpable in all 5. Cardiac involvement was present at the beginning in 2 cases: pericarditis(2), mitral insufficiency(1), mitral and aortic insufficiency(1), and rapidly after admittance in 3 cases: cardiac tamponade and severe hypotension(1), pericarditis( 1), myocarditis(1). Cardiac biomarkers: NT-proBNP and Troponins T and I were elevated in all cases, with more sensitive Troponin I. All 5 patients had: elevated CRP, Ferritin, D-Dimers, liver enzymes, triglycerides, low fibrinogen, falling ESR, leucopenia and thrombocytopenia(one patient with 5000/mm3 thrombocyte), abnormal coagulation. Interleukin 6(IL6) was high in all 7 patients, but in PIMS with MAS, the cytokine storm syndrome, was confirmed by highly elevated soluble IL2 receptor. This patients were promptly treated with IV corticotherapy, anticoagulants, IGIV and antibiotics with favorable evolution. Conclusions: Cardiac tamponade, pericarditis, myocarditis, mitral and aortic insufficiency and Kawasaki-like disease with coronary artery dilatation were found in PIMS patients. Only two cases were positive for COVID Antibodies. Rapid recognition of PIMS and aggressive treatment of MAS prevent fatalities and determined a favorable evolution.