Amyloidosis

Cardiac disease dominates - Expect a restrictive cardiomyopathy with conduction block, arrhythmias, and a risk of sudden death; obtain an ECG and echocardiogram, and manage the heart on restrictive principles (preserve preload and rhythm, avoid tachycardia and myocardial depression). A pacemaker or ICD may be present.

Difficult airway - Macroglossia and pharyngeal or laryngeal deposits can obstruct the airway and complicate intubation; do a focused airway exam and get imaging or nasendoscopy when deposits are suspected, and prepare a difficult-airway plan.

Autonomic dysfunction - Orthostatic hypotension and blunted compensatory reflexes make blood pressure labile; position slowly, anticipate exaggerated hypotension, and have vasopressors ready.

Renal and metabolic - Nephrotic-range renal disease with hypoalbuminemia alters drug protein binding and volume status.

Bleeding - AL amyloid can cause an acquired factor X deficiency and vascular fragility (periorbital purpura); anticipate bleeding.

A group of disorders in which misfolded proteins deposit as insoluble amyloid fibrils in tissues. Systemic forms (AL and ATTR) target organs that matter to the anesthesia provider: the heart (a restrictive cardiomyopathy with conduction disease and arrhythmias - the major cause of death), the autonomic and peripheral nervous system (orthostatic hypotension and labile hemodynamics), the kidneys (nephrotic-range disease), the gut, and the tongue (macroglossia). The combination of a stiff heart, an unstable circulation, and a potentially difficult airway defines the risk.