title: Contemporary treatment of amyloid heart disease.
abstract: The amyloidoses represent a group of diseases characterized by extracellular deposition of abnormal protein, amyloid, which is formed by insoluble extracellular fibrils in β-pleated sheets. Although cardiac involvement may occur in all types of amyloidoses, clinically relevant amyloid cardiomyopathy is a typical feature of AL amyloidosis and transthyretin-related amyloidoses. Congestive heart failure represents the commonest manifestation of amyloid heart disease. Noninvasive imaging techniques, especially echocardiography and cardiac magnetic resonance, play a major role in the diagnosis of amyloid cardiomyopathy; however, histological confirmation and exact typing of amyloid deposits is necessary whether in extracardiac location or directly in the myocardium. Early diagnosis of amyloid heart disease is of utmost importance as the presence and especially the severity of cardiac involvement generally drives the prognosis of affected subjects and plays a major role in determining the intensity of specific treatment, namely in AL amyloidosis. The management of patients with amyloid heart disease is complex. Loop diuretics together with aldosterone antagonists represent the basis for influencing signs of congestion. In AL amyloidosis, high-dose chemotherapy followed by autologous stem cell transplantation is generally considered to be a front-line treatment option, if the disease is diagnosed at its early stage. The combination of mephalan with dexamethasone has been the standard therapy for severely affected individuals; however, the combinations with several novel agents including immunomodulatory drugs and bortezomibe have been tested in clinical trials with promising results. New therapeutic substances with the potential to slow or even stop the progression of transthyretin-related amyloidosis are also extensively studied.
authors.forename: Tomas
authors.initials: T
authors.lastname: Palecek
authors.forename: Michal
authors.initials: M
authors.lastname: Fikrle
authors.forename: Eduard
authors.initials: E
authors.lastname: Nemecek
authors.forename: Lenka
authors.initials: L
authors.lastname: Bauerova
authors.forename: Petr
authors.initials: P
authors.lastname: Kuchynka
authors.forename: William E
authors.initials: WE
authors.lastname: Louch
authors.forename: Ivan
authors.initials: I
authors.lastname: Spicka
authors.affiliation: 2nd Department of Medicine - Department of Cardiovascular Medicine, General University Hospital in Prague, First Faculty of Medicine, Charles University in Prague, U Nemocnice 2, 128 08 Prague 2, Czech Republic. tpalec@lf1.cuni.cz.
authors.forename: Romana
authors.initials: R
authors.lastname: Rysava
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D006331:Heart Diseases / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D014182:Transplantation, Autologous
chemical_list: D000682:Amyloid