RESUMO
Double-outlet right atrium is an extremely rare congenital cardiac defect, in which the right atrium drains into both ventricles. Here in, we present a peculiar case involving an 8-year-old female with double-outlet right atrium characterized by three distinct atrioventricular valves and a membranous ventricular septal defect. This is associated with right atrium to left ventricular flow through the accessory atrioventricular valve while maintaining adequate size of the right ventricle. Additionally, we include a comprehensive review of the literature to underscore the variability in clinical presentation and management strategies.
RESUMO
We herein report on a series of four patients presented to our tertiary care centre with features of multisystem inflammatory syndrome in children and cardiac involvement. Two of our patients had recent exposure to a COVID-19-positive patient, one had recent documented infection, and another had no known positive contact. All the patients were tested positive for severe acute respiratory syndrome coronavirus 2 immunoglobulin G antibody at the time of presentation. All of them fulfilled the diagnostic criteria according to the World Health Organization Centers for Disease Control or the British guidelines for MIS-C (fever for ≥3 days, multisystem involvement (at least two), elevated markers of inflammation, and no other alternative diagnosis).Cardiac involvement was variable ranging from isolated ectasia of the coronary arteries to full-blown pancarditis such as severe biventricular dysfunction, multi-valvar involvement, and pericardial effusion.All our patients received intravenous immunoglobulin (2 g/kg), methylprednisolone, and aspirin and some required inotropic support and ICU admission.Remarkably, all our patients showed significant improvement in their cardiac disease within a few days as evident on serial echocardiographic evaluation. However, we stress the need for long-term follow-up as one of our patients demonstrated mild LV myocardial scarring as evident by gadolinium late enhancement on a cardiac MRI.