ABSTRACT
Introduction: During SARS-COV-2 pandemic, different reports have been published regarding children who developed hyperinflammatory syndrome with certain or probable relationship with SARS-COV-2. These patients presented incomplete or atypical manifestations of Kawasaki disease (KD), particularly abdominal pain, myocarditis and macrophage activation syndrome features. Objectives: To report a case of SARS-COV-2-related Kawasaki-like disease with severe cardiac involvement. Methods: case report description. Results: A 10-year-old previously healthy girl presented progressively worsening abdominal pain, high grade fever for 3 days and vomiting. Lab tests showed WBC 11680/mmc, N 9370/mmc, Creactive protein (CRP) 329 mg/L, procalcitonin (PCT) 0,74 ug/L, PT-INR 1,35 and elevated D-dimer and fibrinogen levels (817 ug/L and 9,45 g/L respectively). Abdomen ultrasound revealed lymphadenopathies and hyperechogenic mesentery in the right lower quadrant, although the appendix was not visualized. She underwent laparoscopy showing moderate quantity of free fluid and appendectomy was performed. Thereafter she continued to complain of high-grade fever, abdominal pain and diarrhoea, despite broad-spectrum antibiotics. Blood, urine and stool cultures were negative. Bilateral non-exudative conjunctivitis was present. Moreover, the lab tests showed persistent marked elevation of CRP (370 mg/L), WBC 15590/mmc, N 14070/mmc, hypoalbuminemia (23 g/L), elevated ferritin and triglycerides (458 ug/L and 221 mg/dl). By taking into consideration the concomitant SARS-COV-2 pandemic, nasopharyngeal and rectal swabs were taken with negative results. Conversely, serological test showed anti-SARS-COV-2 IgG antibodies and absence of IgM. The family medical history showed that the mother had presented fever, cough, ageusia and anosmia one month before, preceded by a contact with a SARS-COV-2 positive case, while the patient was asymptomatic at that time. Suspecting a KD-like disease she was referred to our Paediatric Rheumatology Unit: cardiological assessment revealed negative Twaves in V4-V5-V6 on EKG while standard and advanced echocardiography showed mild mitral and tricuspid insufficiencies, mild dilatation of the left main coronary artery (LMCA, z score +2), normal global function (FEVS 2D 58%) but reduced longitudinal strain (GLS-16%). Lab tests confirmed myocardial injury with troponin (TnI) 100,1 ng/l and brain natriuretic peptide (P-BNP) 593 ng/L. A single infusion of intravenous immunoglobulin 2 g/kg associated with methylprednisolone (1 mg/kg/day) led to a rapid clinical improvement with apyrexia and resolution of abdominal pain and conjunctivitis. Blood test confirmed gradual normalization of inflammatory markers, ferritin, troponin and BNP and EKG showed positive T-waves. Shortly after the discharge, while she was on prednisone 0.5 mg/kg/day and acetylsalicylic acid 100 mg/day, she referred some episodes of heart pounding, lasting about ten minutes with spontaneous resolution. Three weeks after onset, cardiac MRI was normal, however, speckle tracking echocardiography showed persistent dilatation of LMCA and reduction of global longitudinal strain (GLS-14%). 24-hour EKG-Holter detected episodes of supraventricular tachycardia and several ventricular and supraventricular extrasystoles. Thus, oral atenolol therapy was started. Conclusion: In our patient SARS-COV-2 induced a possible postinfectious antibody or immune-complex mediated reaction that led to KD-like disease with acute surgical abdomen presentation and persistent myocardial damage and arrythmias. Speckle tracking echocardiography appears more reliable than MRI in early detection of myocardial damage in patients with preserved left ventricular ejection fraction.