ABC Heart Fail Cardiomyop 2024; 4(2): e20230096
Pulmonary Embolism and Light Chain Cardiac Amyloidosis with Progression to Heart Transplant
Thales Quintella Drummond, Lígia Lopes Balsalobre Trevizan
, Sandrigo Mangini
, Fernando Bacal
Introduction
Cardiac amyloidosis results from the deposition of amyloid fibrils in the heart’s extracellular space. This chronic and progressive disease significantly reduces quality of life and carries a high mortality rate if not diagnosed and treated early. Myocardial damage occurs due to direct injury by blood peptides, as well as architectural distortion caused by the extracellular infiltrate, leading primarily to diastolic ventricular dysfunction but also systolic dysfunction, as the disease progresses and cardiac arrhythmias emerge. The most common subtypes include abnormal transthyretin peptides (ATTR) and immunoglobulin light chain (AL), with AL having a worse prognosis and heart transplantation being a potential treatment option. In the United States, the estimated incidence of light chain amyloidosis cardiomyopathy is 1 in 100,000 individuals., The primary risk factor for the development of light chain amyloidosis is a personal history of monoclonal gammopathy.
Cardiac manifestations often involve orthostatic hypotension, arrhythmias, ventricular hypertrophy inconsistent with electrically inactive regions on the electrocardiogram, and heart failure with preserved ejection fraction predominantly, though reduced ejection fraction may occur in advanced cases.
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