๐ฉบ Postural orthostatic tachycardia syndrome (POTS) is the final common pathway of a heterogeneous group of underlying disorders that share similar clinical features.
๐ About
- POTS is not a single disease but a syndrome โ a collection of disorders leading to the same clinical picture.
- ๐ง Autonomic dysfunction is central to its pathophysiology.
๐งฌ Aetiology (Unclear cause)
- โ๏ธ Neuroendocrine dysfunction
- ๐ฆ Small fibre neuropathy
- ๐ก๏ธ Mast cell activation
- โฌ๏ธ High plasma norepinephrine levels
๐ Definition: Diagnostic Criteria
- โค๏ธ Heart rate increase โฅ +30 bpm on standing (within 10 min)
- ๐ง Symptoms worsen with standing, improve with lying down
- โณ Symptoms persist > 6 months
- ๐ซ No other overt cause of orthostatic tachycardia (e.g. bleeding, dehydration, meds)
๐ฉบ Clinical Features
- ๐ฉ Female:Male ratio โ 5:1
- ๐ฆ Often triggered by viral illness, COVID, pregnancy, immunisation, sepsis, surgery, or trauma
- ๐ Orthostatic intolerance with palpitations, fatigue
- ๐ต Frequent lightheadedness, presyncope
- ๐ Exercise intolerance, dyspnoea on exertion
- ๐ Symptoms worse around menstruation
- ๐ Fatigue, poor sleep, daytime somnolence
- ๐ IBS, joint hypermobility, abnormal sweating
- ๐ฆต ~50% show dependent acrocyanosis (red-blue discolouration of legs, cold to touch)
๐ฆต Dependent Acrocyanosis
๐ Investigations
- ๐งช Bloods: FBC, U&E, LFTs, CRP โ typically normal
- ๐ ECG: usually normal at rest
- ๐ซ Echo: normal
- ๐ 24h Holter: episodic tachycardia
- ๐ช Tilt-table test: diagnostic, reproduces HR rise and symptoms
๐ Management
- ๐ซ Stop contributing drugs: antidepressants, antihypertensives, alcohol, opiates, sildenafil
- ๐โโ๏ธ Reconditioning: gradual aerobic exercise (20 min, 3ร/week)
- ๐ง IV saline (1L over 1โ2h) for short-term/emergency benefit
- ๐งฆ Compression stockings (30โ40 mmHg)
- ๐ฅค Hydration: 2 L/day
- ๐ง Salt loading: +2โ4 g/day (monitor for oedema)
- ๐ Fludrocortisone 0.1โ0.2 mg OD (risk: hypoK, hypoMg, oedema)
- ๐ Desmopressin (occasional use; risk: hyponatraemia)
- ๐ Midodrine 5โ10 mg TDS (risk: nausea, scalp pruritus, supine HTN)
- ๐ Beta-blockers (metoprolol) in adolescents
- ๐ Erythropoietin in severe refractory cases (โ blood volume & vascular tone)
- โจ Management is trial and error โ goal is symptom relief and quality of life.
๐ References