Abstract
Immunoglobulin light chain (AL) amyloidosis may lead to amyloid fibril deposition into peripheral and autonomic nerves, resulting in resting and orthostatic hypotension. While most patients die from progressive heart failure, the most commonly proposed cardiac rhythm associated with sudden death is pulseless electrical activity (PEA). Herein, we describe four patients with severe AL cardiac amyloidosis who had witnessed cardiac arrest with pulseless electrical activity as a result of vasovagal syncope. Healthcare providers should be aware of severe autonomic dysfunction in cardiac amyloidosis and the potential for an abnormal vasovagal response leading to syncope or death.