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• W1549005598 abstract "Amyloidosis is a group of diseases characterized by the extracellular deposition of a proteinaceous material in various organs and tissues, leading to progressive multiple organ failure and death. The presence of cardiac involvement and its relative predominance varies with the type of amyloidosis, tends to progress rapidly and has a very poor prognosis. AL amyloidosis is the most common form of the disease. The heart in AL amyloidosis is affected in close to 50% of cases and amyloid deposition can be very rapidly progressive with increased untreated myocardial wall thickening at rates of up to 1.45–2.16 mm/month (Kristen et al., 2007). Heart failure (HF) is the presenting clinical manifestation in about half of these patients (Dubrey et al., 1998). Even among patients in whom another organ system dysfunction predominates, the presence of cardiac amyloidosis is frequently the worst prognostic factor (Kyle et al., 1995). Once HF occurs, the median survival is less then six months in untreated patients (Dubrey et al., 1998; Kyle et al., 1995). Although the overall survival of AL amyloidosis patients can be improved by the use of high dose chemotherapy and autologous stem-cell transplantation (ASCT) (Dey et al., 2010), the advanced cardiac disease at the time of diagnosis place these patients at a risk of 30% peri-treatment mortality (Falk et al., 1998). Marked wall thickening, elevated brain natriuretic peptide or elevated troponin predict poor outcomes and an ejection fraction < 40% is considered an absolute contraindication to high-dose chemotherapy and ASCT (Dispenzieri et al., 2003; Dispenzieri et al., 2004; Falk et al., 2005). However, it has been suggested that cardiac transplantation can restore health and permit subsequent administration of intensive chemotherapy." @default.
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• W1549005598 date "2011-11-16" @default.
• W1549005598 modified "2023-10-16" @default.
• W1549005598 title "Cardiac and Multi-Organ Transplantation in Patients with Amyloidosis" @default.
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• W1549005598 doi "https://doi.org/10.5772/18880" @default.
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