cardiovascular-agents and Amyloidosis

When amyloidosis affects the heart, a devastating and progressive process can lead to congestive heart failure, arrhythmias, conduction abnormalities, angina, and death. The signs and symptoms of cardiac amyloidosis are generally dominated by diastolic heart failure resulting from restrictive cardiomyopathy. Amyloid infiltration of the heart initially causes mild diastolic dysfunction, but late disease produces a thickened heart wall with a firm and rubbery consistency, which worsens cardiac relaxation and diastolic compliance. Patients usually complain of progressive dyspnea from congestive heart failure, chest discomfort secondary to microvascular involvement, and weight loss, which might be a manifestation of cardiac cachexia. Echocardiographic findings include nondilated ventricles with concentric left ventricular thickening, right ventricular thickening, prominent valves, dilated atria, and thickening of the interatrial septum. Recent advances in our understanding of the pathophysiology of amyloid have allowed the various types to be differentiated, which has led to targeted therapy for each unique pathophysiologic process.

Topics: Amyloidosis; Anti-Infective Agents; Biomarkers; Cardiac Catheterization; Cardiomyopathy, Restrictive; Cardiovascular Agents; Drugs, Investigational; Echocardiography; Electrocardiography; Heart Diseases; Heart Failure; Heart Transplantation; Humans; Liver Transplantation; Magnetic Resonance Imaging; Stem Cell Transplantation; Treatment Outcome

Cardiac amyloidosis is a well-known but clinically rare cause of heart failure that has historically been associated with a poor prognosis. Cardiac amyloidosis involves fibril formation from one of several underlying conditions, and the course of illness and prognosis varies among these conditions. Evolving treatment strategies for patients with primary systemic amyloidosis have given this subset of cardiac amyloidosis patients a cause for hope. The identification and appropriate referral of patients in whom this condition is suspected will help to improve the likelihood of successful therapy and long-term survival.

Topics: Amyloidosis; Cardiomyopathies; Cardiovascular Agents; Clinical Trials as Topic; Heart Failure; Humans; Prognosis

Topics: Aged; Amyloidosis; Antihypertensive Agents; Arrhythmias, Cardiac; Body Weight; Cardiac Glycosides; Cardiovascular Agents; Cardiovascular Diseases; Cardiovascular System; Coronary Circulation; Coronary Disease; Heart Valve Diseases; Hemodynamics; Humans; Hypertension; Myocardial Contraction; Organ Size