BACKGROUND: Hypermobile Ehlers-Danlos Syndrome (hEDS) is a connective tissue disorder characterized by joint hypermobility and other systemic manifestations, such as cardiovascular symptoms, musculoskeletal pain, and joint instability. Cardiovascular symptoms, such as lightheadedness and palpitations, and types of dysautonomia, including postural orthostatic tachycardia syndrome (POTS), are frequently reported in adults with hEDS and have been shown to negatively impact quality of life (QoL).
OBJECTIVE: This brief review will be an overview of co-occurring symptoms in POTS and hEDS to inform potential cardiovascular screening procedures.
RESULTS: While many patients with hEDS report cardiovascular symptoms, few have structural abnormalities, suggesting that dysautonomia is likely responsible for these symptoms. One validated screening measure for dysautonomia symptom burden is the Composite Autonomic Symptom Scale (COMPASS-31). Studies have found that adults with POTS, hEDS, and both POTS and hEDS have higher COMPASS-31 scores than the general population, suggesting a high symptom burden due to dysautonomia, which leads to impaired QoL.
CONCLUSIONS: While studies have examined cardiovascular symptoms and the impact of dysautonomia in adults with and without hEDS, there is scant literature on dysautonomia in pediatric patients with hEDS. Therefore, more studies on cardiovascular symptoms and dysautonomia, as they relate to the quality of life in pediatric patients with hEDS, are needed. This brief review summarizes the current literature on dysautonomia and cardiovascular symptoms in pediatric and adult populations with hEDS.

BACKGROUND: There are few tools to detect the diabetic autonomic neuropathy at an earlier stage. This study was conducted to investigate the association between symptoms of autonomic neuropathy and the heart rate variability (HRV) in diabetics.
METHODS: Study subjects consisted of 50 diabetic patients and 30 outpatient hospital control patients at a university family medicine department. The patients completed a Korean version of composite autonomic symptom scale (COMPASS). Electrocardiography was recorded in the supine position, on standing, and during deep breathing, for 5 minutes each. HRV of frequency domain was calculated by power spectral analysis.
RESULTS: The COMPASS score was higher in female diabetic patients compared with that in controls. Among 50 diabetic patients, the total COMPASS score correlated positively with normalized low frequency (LF) score (normalized units, n.u.) (r = 0.62, P < 0 .001) and low frequency/high frequency (LF/HF) (r = 0.77, P < 0.001), negatively with normalized HF score (n.u.) (r = -0.59, P < 0.001) and RMSSD (square root of the mean of the sum of the square of differences between adjacent NN interval; r = -0.33, P = 0.031). The decrease in LF (n.u) and the increase in HF (n.u) by deep breathing from the supine position were higher in diabetic patients compared with those in controls. The increase in LF (n.u) and the decrease in HF (n.u) by standing from the supine position were lower in diabetic patients compared with those in controls.
CONCLUSIONS: The COMPASS score correlated with some component score of the HRV in diabetics. The HRV may be used as a tool to detect diabetic autonomic neuropathy by augmentation with position change.