Restrictive cardiomyopathy as a presentation of amyloidosis

Abstract

Cardiac amyloidosis is a rare disease that may occur as a component of systemic amyloidosis. Its symptomatic phase usually presents when cardiac function is highly compromised. Recently, some non-invasive diagnostic tools have emerged improving diagnosis. Treatment for this disease depends on its subtype. In most cases, it consists on supportive measures to improve heart failure and organ function, in addition with oral chemotherapy and autologous stem-cell transplantation. Alongside, new prognostic factors have been introduced. As an example, a clinical case about a patient who develops rapid-progression dyspnea and congestive heart failure is described. The ecocardiographic report stated a left ventricular concentric hypertrophy and ecogenic infiltrate patterns associated with extensive fibrosis. This leaded to the diagnosis of cardiac amyloidosis secondary to systemic amyloidosis. Based on this case, characteristics and new advances in its management are presented