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Background: Hydroxychloroquine (HCQ) is a disease-modifying antirheumatic used in rheumatological diseases such as systemic lupus erythematosus. Long-term exposure to HCQ results in drug accumulation and predisposes to adverse effects. Case summary: We present the case of a 45-year-old woman with long-term treatment with HCQ who presented to the Emergency Department with acute heart failure. Transthoracic echocardiogram, previously normal, showed severe biventricular hypertrophy and biventricular systolic dysfunction. Cardiac magnetic resonance (CMR) confirmed the previous findings and showed elevated native T1 and T2 values, elevated extracellular volume, and extensive mid-wall late gadolinium enhancement (LGE). Infiltrative cardiomyopathy was suspected, and endomyocardial biopsy performed. Light microscopy showed myocyte hypertrophy and vacuolar change and absence of lymphocytic inflammatory infiltrates. The diagnosis of HCQ-induced cardiomyopathy was established, and the drug was withdrawn. A CMR performed 1 year later showed normal systolic function of both ventricles and normalization of T2 values, reflecting resolution of myocardial oedema. However, severe hypertrophy, elevated native T1 values, and LGE persisted. Discussion: Our case shows that although discontinuation of the drug stops the progression of the disease, established myocardial structural damage persists. Early diagnosis of this entity is therefore essential to improve prognosis.
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In this article we describe two cases that presented with persistent fever and a hyperinflammatory state in association with severe acute respiratory syndrome-coronavirus-2 infection with various negative reverse transcription-polymerase chain reaction results. These cases subsequently developed myocarditis with cardiogenic shock that required vasoactive drugs and had a good response to corticosteroid treatment. All cases met criteria for a definitive case of multisystemic inflammatory syndrome in adults, a recently described entity associated with coronavirus disease 2019, which has a good response to immunomodulators and a good prognosis in most cases.
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AIMS: The aim of the study was to validate a novel formula for aortic area, based on the principle of continuity equation (CE), that substitutes Doppler-derived stroke volume (SV) by SV directly measured with real-time three-dimensional (RT3D) echo and semi-automated border detection. RT3D has proved outstanding accuracy for left ventricular volume calculation. So far, however, neither this potential has been applied to haemodynamic assessment, nor RT3D has succeeded in the evaluation of aortic valve disease. METHODS AND RESULTS: Aortic area was measured in 41 patients with aortic stenosis using Gorlin's equation, Hakki's formula, Doppler CE, two-dimensional Simpson's volumetric method, and by the novel RT3D method. RT3D has the best linear association and absolute agreement with Gorlin of all non-invasive methods r = 0.902, intraclass correlation coefficient (ICC) = 0.846, better than CE (r = 0.646, ICC = 0.626) and two-dimensional volumetric method (r = 0.627, ICC = 0.378). Linear and Passing-Bablok regression show that RT3D fits better to Gorlin (r(2) = 0.814) than CE (r(2) = 0.417) and two-dimensional method (r(2) = 0.393). Its accuracy is comparable to Hakki's formula, routinely employed in catheter laboratories. Inter- and intraobserver agreements (ICC) were, respectively, 0.732 and 0.985, better than CE (0.662, 0.857). RT3D also grades most efficiently the severity of aortic stenosis as mild, moderate, or severe (weighted kappa = 0.932). RT3D underestimates aortic area (95% CI 0.084-0.193). ROC curves, however, show that the optimal cutoff point to consider aortic stenosis severity remains close to 1 cm(2) (1.06 cm(2)). CONCLUSIONS: RT3D is more accurate than CE and than two-dimensional volumetric methods to calculate area and to grade the severity of aortic stenosis. Area obtained by three-dimensional echo is slightly underestimated, but its range is clinically negligible.