Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations

Abstract

Background Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware. Case Description We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin. Literature Review POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation. Clinical Relevance These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

A Pilot Study using the Compensatory Reserve Index to evaluate individuals with Postural Orthostatic Tachycardia syndrome

“…Patients in the physiologic Postural Orthostatic Tachycardia syndrome and no physiologic Postural Orthostatic Tachycardia syndrome groups also had similar symptom severity scores (p = 0.260). 1 The remaining findings are summarised in Table 2.…”

“…The Functional Disability Inventory, a well-validated measure of perceived activity limitations in children and adults, 30 evaluates the difficulty of 15 daily tasks with a scale of impossible (4), a lot of trouble (3), some trouble (2), a little trouble (1) and no trouble (0). 30 This measure was adapted very slightly to reflect the ubiquity of electronic media for today's adolescents, e.g., modified from "Watching TV" to "Watching TV and other screens."…”

“…Postural Orthostatic Tachycardia syndrome is a condition that affects 1-3 million patients per year in the United States with a predominance in adolescent Caucasian females. 1,2 Postural Orthostatic Tachycardia syndrome is characterised by autonomic dysfunction, with an increase in heart rate (≥30 beats/minute) when moving from supine to standing. 3 Many patients also experience non-specific symptoms that may or may not be related to autonomic dysfunction, such as dizziness, nausea, vomiting, fatigue, headaches, abdominal discomfort and sleep disturbances.…”

Hypermobile Ehlers-Danlos Syndrome during pregnancy, birth and beyond

“…Women with hEDS often also experience postural orthostatic tachycardia syndrome (POTS) (Grigoriou et al, 2015), which could affect up to 78% of women (Gazit et al, 2003). POTS is defined by a rise in heart rate of >30 beats/min, or a heart rate of >120 beats/min reached within 10 min of head-up tilt when moving from supine to an upright position (Kanjwal et al, 2003).…”

Ehlers–Danlos hypermobility type in an adult with chronic pain and fatigue: a case study

“…Unrelated previous diagnoses included diabetes mellitus type 2, hyperlipidemia, and depression. The patient's family history was noncontributory with the exception of her daughter, who is currently being tested for POTS, which has a high concurrence rate with EDS‐HT .…”