Cardiac amyloidosis has high associated morbidity and, until recently, limited treatment options.
This review discusses the mechanism and clinical trial performance of multiple emerging therapies.
Optimal treatment paradigms and biomarker strategies for cardiac response to therapy are being identified.
Amyloidosis is a difficult diagnostic conundrum in many situations and it is well known that there can be long delays in confirming the accurate diagnosis. The treatment options for this condition are rapidly developing and the field is energized with new successful treatments. Dr Lenihan can assist in facilitating the diagnosis and getting each patient on the best possible therapy.
AL amyloidosis results from clonal production of immunoglobulin light chains, most commonly arising from a clonal plasma cell disorder. Once considered a nearly uniformly fatal disease, prognosis has improved markedly over the past 15 years, predominantly because of advances in light chain suppressive therapies. Cardiac deposition of amyloid fibrils is common, and the severity of cardiac involvement remains the primary driver of prognosis.