Orthostatic tachycardia is common but its specificity remains uncertai
n. Our preliminary work suggested that using autonomic function testin
g in conjunction with time-frequency mapping (TFM), it might be possib
le to characterize a subset of the postural tachycardia syndrome (POTS
), that is due to a restricted autonomic neuropathy. We describe 20 pa
tients (17 women and 3 men, aged 14-43 years) with florid POTS and 20
controls (14 women and 6 men, aged 20-41 years). Autonomic failure was
quantified by its distribution (cardiovagal, adrenergic and sudomotor
) and severity. a symptom profile was generated, and spectral indices,
based on modified Wigner distribution during rest and head-up tilt (8
0 degrees), were evaluated. During tilt-up POTS patients differed from
controls by an excessive heart rate (> 130 bpm) (P < 0.001), and high
er diastolic pressure (P < 0.01). During rest, cardiovagal oscillation
s (at respiratory frequencies [RF]) and slow rhythms at nonrespiratory
frequencies (NONRF) (from 0.01 to 0.07 Hz) in R-R intervals (RRI) (P
< 0.01) were reduced. Both RF and NONRF rhythms in RRI were further bl
unted with tilt-up (P < 0.001). Slow adrenergic vasomotor rhythms in b
lood (NONRF-Systolic BP (SBP)/RF-RRI) was dramatically increased in PO
TS (P < 0.001). Distal postganglionic sudomotor failure was observed,
and impairment of the BP responses to the Valsalva maneuver (phase II)
suggested peripheral adrenergic dysfunction. Persistent orthostatic d
izziness, tiredness, gastrointestinal symptoms and palpitations were c
ommon in POTS patients. It is possible to identify a subset of POTS pa
tients who have a length-dependent autonomic neuropathy, affecting the
peripheral adrenergic and cardiovagal fibers, with relative preservat
ion of cardiac adrenergic fibers.