Where Neurosurgery Meets Heart Failure: A Case Report of a Patient with Amyloid Transthyretin Wild Type (ATTRwt) in the Ligamentum Flavum and Cardiac Tissue with Bilateral Carpal Tunnel Syndrome.

Transthyretin wild type (ATTRwt) amyloidosis is a systemic process resulting in deposition of misfolded transthyretin protein in several different tissues throughout the body. It is known to be a cause of progressive, life-threatening cardiomyopathy as well as lumbar spinal stenosis and carpal tunnel syndrome. Here we present the case of a 71yo male who has clinical manifestations of all three entities over several years, providing a picture of the natural history of ATTRwt amyloidosis. This patient eventually underwent a heart transplant due to progressive cardiac amyloidosis (CA) resulting in end stage heart failure. However, his symptoms in his carpal tunnel and lumbar spine manifested years before the symptoms of heart failure. ATTRwt amyloidosis may present as a clinical triad of lumbar stenosis, carpal tunnel syndrome, and heart failure. Recently developed medications have shown efficacy in slowing the progress of systemic and cardiac amyloidosis. Knowing that extra-cardiac symptoms may occur first, we propose that sending ligamentum flavum and flexor tenosynovium for pathologic evaluation may be a unique opportunity to screen and treat a population of patients at risk for developing CA and heart failure.