Quantitative assessment of autonomic symptom burden in Postural tachycardia syndrome (POTS).

Postural tachycardia syndrome (PoTS) is a poorly understood disorder characterized by excessive tachycardia in the upright position. In addition, patients with PoTS often complain of non-postural symptoms, including fatigue, gastrointestinal and vasomotor fluctuations. The present study quantitatively assessed autonomic symptom burden in PoTS patients (n=32) using the COMPASS-31, compared to that of autonomic failure/neuropathy (AF/N; n=47) and asymptomatic, healthy controls (n=32). Using AIC model selection and regression analysis, we found differences in the contribution of individual COMPASS-31 domains, depending on the autonomic disorder. In PoTS, fatigue severity, orthostatic intolerance and pupillomotor symptom domains, contributed significantly to differences in COMPASS-31 scores compared to controls. In contrast, the secretomotor, gastrointestinal, bladder and vasomotor domains, contributed significantly to the AF/N model. Our results confirm an increase in autonomic symptoms across all functional domains in PoTS compared to controls, and with similar severity to AF/N, though with differing significant domain contributions. Our findings provide additional support that PoTS is indeed a syndrome of autonomic dysfunction beyond orthostatic intolerance, but also indicates the likelihood of disease-specific contributions to symptom burden, highlighting the need for application of expanded physiological assessment beyond orthostatic challenge, as well as disease-specific symptom assessment tools for use in PoTS.

Assessment of prevalence and pathological response to orthostatic provocation in patients with multiple sclerosis.

OBJECTIVE: The aim of this study was to determine the prevalence of pathologic response to orthostatic challenge in patients with relapsing remitting multiple sclerosis (RRMS) and the difference of the response in patients in relapse and remission. PATIENTS AND METHODS: We included 112 RRMS patients; group 1 included 53 patients in a relapse and group 2, 59 patients in remission. The head up tilt table test was used to provoke an orthostatic reaction. RESULTS: 71 (63%) patients (60.4% and 66% of relapse and remission subjects respectively) had a pathological response to orthostatic provocation. Syncope was found in 9 (17%) patients in group 1 compared to 22 (37.3%) in group 2 (p=0.014). Postural orthostatic tachycardia syndrome (POTS) was found in 17 (32%) patients in group 1 compared to 4 (6.8%) in group 2 (p=0.001). There was a significantly negative correlation between the Expanded Disability Status Scale (EDSS) and POTS (-0.201; p=0.034) and a positive correlation between the EDSS and syncope (0.190; p=0.044). CONCLUSION: The prevalence of distinct types of orthostatic autonomic dysfunction in different phases of RRMS seems to be in direct correlation with the EDSS. Furthermore, certain autonomic dysfunctions of orthostasis, more specifically syncope and POTS, tend to be increased in remission and relapse respectively.

What is the optimal duration of tilt testing for the assessment of patients with suspected postural tachycardia syndrome?

AIMS: The aim of this study is to define the optimal duration of tilt testing for the assessment of patients with suspected postural tachycardia syndrome (POTS). METHODS AND RESULTS: This was a case-control study. Cases were identified retrospectively from a database of patients referred with orthostatic intolerance (OI). All met the diagnostic criteria for POTS. Controls were enrolled prospectively. All subjects underwent tilting to 70 degrees for 40 min if tolerated. Continuous monitoring was provided by a Finometer. Analysis of responses to tilting was performed on 28 cases and 28 controls. The mean age in the case group was 23.6 and in the control group was 26.2. The majority was female in both groups (cases = 4F:3M, controls = 2F:1M). All cases met the criteria for POTS within 7 min of orthostasis. No controls demonstrated a sustained tachycardia. The prevalence of vasovagal syncope (VVS) was 36% in cases vs. 7% in controls (P = 0.02) and 25% in the remaining patients (n = 233) on the OI database (P = 0.259). CONCLUSION: A 10 min tilt will diagnose POTS in the majority of patients. It will not, however, be sufficient to identify the overlap that exists between POTS and VVS. The optimal duration of tilt testing in patients suspected of POTS is 40 min.