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"Heart failure", "Pulmonary hypertension", "Acute heart failure", Cardiomyopathy

Adam Golda, Agnieszka Jurecka, Anna Tylki-Szymanska
The aim of the article is to gather and summarize the published data about the incidence, course of illness, treatment possibilities and complications of cardiovascular disorders in patients with mucopolysaccharidosis type VI (MPS VI) also known as Maroteaux-Lamy syndrome. MPS VI is a lysosomal storage disorder caused by deficient activity of N-acetylogalactosamine-4-sulfatase leading to progressive intracellular accumulation of glycosaminoglycans. The relatively low birth prevalence ranging from 1 in 43,000 to 1 in 1...
June 28, 2012: International Journal of Cardiology
Avihu Z Gazit, Phineas P Oren
Prompt initiation of appropriate and intensive treatment in children with decompensated heart failure is crucial to avoid irreversible end-organ dysfunction. Initial management of these children includes transfer to the pediatric cardiac intensive care unit, basic hemodynamic monitoring, and establishment of intravenous access. Inotropic support should be instituted peripherally before obtaining central venous and arterial access. The team should be prepared for emergent intubation and initiation of mechanical circulatory support...
October 2009: Current Treatment Options in Cardiovascular Medicine
Magdalena Dziadzio, Andrea Giovagnoni, Giovanni Pomponio, Andrea Recanatini, Osmy Paci della Costanza, Aldo Manzin, Walter Casagrande, Armando Gabrielli
We describe a 40-year-old man with limited scleroderma who presented with acute heart failure following a flu-like illness. He was known to have incomplete left anterior bundle branch block, initial isolated pulmonary hypertension with enlarged right atrium, and no pulmonary fibrosis. He received therapy for acute heart failure and was transferred to a scleroderma centre for specific treatment of scleroderma cardiomyopathy. Investigations showed raised inflammatory markers and diffuse hyperechogenic thickening of the myocardium on echocardiography...
December 2003: Clinical Rheumatology
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