Autonomic nervous system dysfunction has a major role in the blood pressure (BP) decrease associated with orthostatic hypotension and syncope. The clinical picture of Bartter and Gitelman syndromes includes reduced extracellular fluid volume and normotension or hypotension, but no study has explored autonomic nervous system status in patients with hypotensive episodes associated with these diseases. We tested sympathetic and parasympathetic nervous system function in 4 patients with Bartter and Gitelman syndromes with chronic hypotension. Each patient underwent a battery of autonomic reflex tests, including BP and heart rate response to orthostatism, Valsalva maneuver, cold-pressor test, hand-grip test, and deep breathing. Plasma catecholamines also were measured. BP was monitored during tests by means of continuous noninvasive finger BP recording. Orthostatic hypotension was observed in 1 patient who experienced syncope episodes. Valsalva ratio ranged from 1.21 to 1.61. During the cold-pressor test, the range of systolic and diastolic BP increases were 8 to 31 and 6 to 24 mm Hg, respectively. During the hand-grip test, systolic and diastolic BP increases ranged from 10 to 39 and 8 to 32 mm Hg, respectively. During hyperventilation, the difference between the highest and lowest heart rates was 12 or more beats/min in all patients. Patients' plasma norepinephrine concentrations increased during standing. Our preliminary results suggest that chronic hypotension in patients with Bartter and Gitelman syndromes is not associated with sympathetic and parasympathetic nervous system dysfunction, even when orthostatic hypotension is present. This normal autonomic function suggests that other pathophysiological mechanisms, such as the characteristic vasoconstrictor abnormal cell signaling, may account for hypotension in patients with Bartter and Gitelman syndromes.