Cardiac Origins of the Postural Orthostatic Tachycardia Syndrome

Objectives To test the hypothesis that a small heart coupled with reduced blood volume contributes to the Postural Tachycardia Syndrome (POTS), while exercise training improves this syndrome. Background Patients with POTS have marked increases in heart rate during orthostasis. However, the underlying mechanisms are unknown and the effective therapy is uncertain. Methods Twenty-seven POTS patients underwent autonomic function tests, cardiac MRI, and blood volume measurements. Twenty-five of them participated in a 3-mo specially designed exercise training program with 19 completing the program; these patients were reevaluated after training. Results were compared with those of 16 healthy controls. Results Upright heart rate and total peripheral resistance were greater, while stroke volume and cardiac output were smaller in patients than controls. Baroreflex function was similar between groups. Left ventricular mass (median [25%, 75%], 1.26 [1.12, 1.37] vs 1.45 [1.34, 1.57] g/kg; P<0.01) and blood volume (60 [54, 64] vs 71 [65, 78] ml/kg; P<0.01) were smaller in patients than controls. Exercise training increased left ventricular mass and blood volume by ~12% and ~7%, and decreased upright heart rate by 9 [1, 17] bpm. Ten out of 19 patients no longer met POTS criteria after training, while patients’ Quality of Life assessed by Short-Form 36 was improved in all patients after training. Conclusions Autonomic function was intact in POTS patients. The marked tachycardia during orthostasis was attributable to a small heart coupled with reduced blood volume. Exercise training improved or even cured this syndrome in the majority of patients. It seems reasonable to offer POTS a new name based on its underlying pathophysiology – “The Grinch Syndrome”, because in this famous children’s book by Dr. Seuss, the main character had a heart that was “two sizes too small.”