Summary

Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous group of conditions characterised by autonomic dysfunction and an exaggerated sympathetic response to assuming an upright position. Up till recently, it was largely under-recognised as a clinical entity. There is now consensus about the definition of POTS as a greater than 30/min heart rate increase on standing from a supine position (greater than 40/min increase in 12–19-year-old patients) or an absolute heart rate of greater than 120/min within 10 min of standing from a supine position and in the absence of hypotension, arrhythmias, sympathomimetic drugs or other conditions that cause tachycardia. We present two cases of POTS, followed by a discussion of its pathogenesis, pathophysiology, epidemiology and management.