Abstract

Background: Postural orthostatic tachycardia syndrome (POTS) is an autonomic neuropathy (dysautonomia) affecting at least 0.2-1% of the US population. POTS symptoms include rapid heart rate, dizziness, and fatigue, and can substantially impair activities of daily living. POTS is often secondary to autoimmune or infectious diseases including COVID-19. In Oct. 2022, the first diagnosis code specific to POTS was introduced. This code presents an opportunity to more accurately identify and characterize patients with POTS.

Objectives: To monitor the uptake of the new POTS diagnosis code and to describe the demographic and clinical characteristics of POTS patients.

Methods: We used the Healthcare Integrated Research Database (HIRD), a large U.S. claims database. We identified patients with at least one claim with a POTS diagnosis code (ICD-10-CM G90.A) in any position or care setting from 10/1/2022 through 9/30/2023. Data were available from 1/1/2006.

Results: A total of 14,603 patients were diagnosed with POTS. Patients were mostly female (89%), aged 20-40 years (75%). Many (47%) had a previous claim for “other cardiac arrhythmias,” and 22% had a procedure code for a tilt table test. Comorbidities potentially related to POTS included hypertension (27%), Ehlers-Danlos syndrome (EDS; 20%), mast cell activation syndrome (11%), Type 1 or 2 diabetes (9%), gastroparesis (8%), post COVID-19 condition (7%), rheumatoid arthritis (7%), adrenal insufficiency (6%), Sjögren's syndrome (5%), mitral valve prolapse (5%), post-viral fatigue (4%), lupus (3%), narcolepsy (3%), and multiple sclerosis (2%). Conditions that may represent misdiagnosis of dysautonomia symptoms included migraine (46%), generalized anxiety disorder (GAD; 45%), irritable bowel syndrome (21%), and panic disorder (15%). Many had at least one fill of a drug that is commonly used off-label for POTS: beta-blocker (50%), fludrocortisone (22%), midodrine (22%), pyridostigmine (8%).

Conclusions: The new POTS diagnosis code was widely used. Conditions that are rare in the general population were not rare in patients with POTS: prevalence of EDS was >20-fold greater than prevalence in the HIRD and published global estimates. Misdiagnosis of POTS as anxiety or other disorders may be common (45% had a diagnosis of GAD). While no drug is indicated for POTS, many patients filled prescriptions for possible off-label treatments, especially beta-blockers. Further research is needed to examine the sensitivity/specificity of the POTS diagnosis code so that patients can be accurately identified for further etiologic and interventional research.