History

Fact Explanation
Introduction Orthostatic hypotension (OH) is defined as a persistent, consistent, orthostatic fall in systolic blood pressure of ≥20 mm Hg or diastolic pressure of ≥10 mm Hg by 3 minutes of standing up.1 Acute, unexpected, episodic falls in blood pressure while standing, as in neurocardiogenic syncope, do not satisfy criteria for OH. OH can be due to nurogenic as well as non neurogenic causes. Neurogenic orthostatic hypotension is caused by failure of noradrenergic neurotransmission which can arise from primary neurodegenerative disorders( pure autonomic failure, Parkinson's disease with autonomic failure, multiple system atrophy ) or can be secondary to systemic conditions that influence peripheral nerve function(diabetic and nondiabetic autonomic neuropathies) In baroreflex failure (BF) there is loss of afferent baroreflex engagement of central mechanisms of blood pressure control. However, central stimuli (such as anxiety, pain, anger, or excitement) can still engage an otherwise functional peripheral sympathetic system. BF is generally caused by bilateral structural lesions in the carotid sinuses, the glossopharyngeal nerves or brainstem due to tumor, injury, or other damage to afferent pathways.[2][5]
Dizziness, lightheadedness,syncope Normally consciousness is highly dependant on cerebral perfusion which begins to alter within 6 seconds after shutdown of cerebral blood flow, this will result in range of symptoms like dizziness, light headedness, presyncope or syncope.[1][2][3][4]
Chest pain Reduced blood pressure result in cardiac hypoperfusion causes cardiac muscle ischaemia.[1][2][3][4]
Oliguria When the systemic low blood pressure exceeds the compensatory mechanisms by glomerular afferent and efferent arteriolar contraction,glomerular filtration is reduced thus affecting urine output.<0.5ml/Kg/hr[1][2][3][4]
Blurred vision In orthostatic hypotension occipital cortex hypoperfusion can result in ischaemia to the specific neuronal cells.[1][2][3][4]
Amaurosis fugax, aphasia, dysarthria, unilateral sensory and motor symptoms Stroke can be a cause for orthostatic hypotension(OH)[1][2][3][4]
Any history of burns abnormal uterine bleeding, rectal bleeding,diarrhoea, vomitting Volume depletion can cause OH.[1][2][3][4]
Chest pain, palpitations, shortness of breath Congestive heart failure, myocardial infarction, myocarditis, pericarditis could cause OH.[1][2][3][4]
Extremity swelling Congestive heart failure, venous insufficiency can cause OH.[1][2][3][4]
Bradykinesia, pill-rolling tremor, shuffling gait Parkinson disease can be a cause for OH.[1][2][3][4]
Progressive motor weakness Guillain-Barré syndrome, multiple system atrophy can cause OH.[1][2][3][4]
Any history of medication such as diuretics, α-antagonists for prostatism,antihypertensive drugs, and calcium channel entry blocking drugs. Insulin, levodopa, or tricyclic antidepressants can also cause vasodilatation and OH in predisposed subjects. These drugs can cause OH.[1][2][3][4]
Symptoms after a meal Postprandial hypotension caused by splanchanic vaso dilation. [1][2]
Decreased libido, impotence in men; urinary retention and incontinence in women If OH is due to pure autonomic faliure the symptoms are seen.[1][2][3][4]
High-risk sexual behaviour AIDS, neurosyphilis can cause OH.[1][2][3][4]
Polyuria and polydipsia Diabetes insipidus and DM can present in this way.[1][2][3][4]
References
  1. FIGUEROA J. J., BASFORD J. R., LOW P. A.. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic Journal of Medicine [online] December, 77(5):298-306 [viewed 24 September 2014] Available from: doi:10.3949/ccjm.77a.09118
  2. GOLDSTEIN D. S., SHARABI Y.. Neurogenic Orthostatic Hypotension: A Pathophysiological Approach. Circulation [online] December, 119(1):139-146 [viewed 24 September 2014] Available from: doi:10.1161/CIRCULATIONAHA.108.805887
  3. LOW PA, SINGER W. Update on Management of Neurogenic Orthostatic Hypotension Lancet Neurol [online] 2008 May, 7(5):451-458 [viewed 24 September 2014] Available from: doi:10.1016/S1474-4422(08)70088-7
  4. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303
  5. METZLER MANUELA, DUERR SUSANNE, GRANATA ROBERTA, KRISMER FLORIAN, ROBERTSON DAVID, WENNING GREGOR K.. Neurogenic orthostatic hypotension: pathophysiology, evaluation, and management. J Neurol [online] December, 260(9):2212-2219 [viewed 30 September 2014] Available from: doi:10.1007/s00415-012-6736-7

Examination

Fact Explanation
Supine and standing blood pressure In orthostatic hypotension there is a reduction of systolic blood pressure (SBP) of at least 20 mm Hg or diastolic blood pressure (DBP) of at least 10 mm Hg within 3 minutes of standing up.[1][2]
Confusion, dry mucous membranes, dry tongue, longitudinal tongue furrows, speech difficulty, sunken eyes If the cause for OH is blood loss or dehydration these signs will be seen.[1][2]
Increased pigmentation in the skin, buccal mucosa,palm and soles If OH was due to addisons disease these features will be seen.[1][2]
Cardiac murmur or gallop In Congestive heart failure and myocardial infarction these signs are seen.[1][2]
Dependent lower extremity edema, stasis dermatitis In right-sided congestive heart failure and venous insufficiency these signs will be present.[1][2]
Aphasia, dysarthria, facial droop, hemiparesis These signs are seen in a stroke.[1][2]
Cogwheel rigidity, festinating gait, lack of truncal rotation while turning, masked facies These signs are seen in Parkinson disease.[1][2]
Partial ptosis, anhydrosis, miosis Horner syndrome can present in pure autonomic faliure.[1]
Rigidity and bradykinesia are out of proportion to tremor Speech is affected severely (dysarthrophonia, severe dysarthria) Multiple system atrophy.[1]
References
  1. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303
  2. SHIBAO CYNDYA, LIPSITZ LEWIS ARNOLD, BIAGGIONI ITALO. ASH Position Paper: Evaluation and Treatment of Orthostatic Hypotension. [online] December, 15(3):147-153 [viewed 24 September 2014] Available from: doi:10.1111/jch.12062

Differential Diagnoses

Fact Explanation
Cardiovascular Disorders These are cardiac arrhythmia, congestive heart failure, myocardial infarction, myocarditis,pericarditis, valvular heart disease and venous insufficiency. shortness of breath,basal crackles on auscultation,JVP elevation,ankle odema(heart failure), anginal symptoms,ECG changes(myocardial infarction,myocarditis),echocardiography(valvular heart disease) can diagnose these conditions.[1][2][3]
Endocrine Disorders These are adrenal insufficiency (ACTH stimulation test),diabetes insipidus(urine osmolality and electrolyte levels) hyperglycemia(fasting blood sugar,HbA1c), acute hypoaldosteronism(serum electrolytes) hypokalemia, hypothyroidism(TSH and T4 levels), pheochromocytoma(urine vanillylmandelic acid, and metanephrines) History and above investigations can differentiate these conditions.[1][2][3]
Intravascular volume depletion -blood loss, dehydration History of blood loss,burns,diarrhoea,vomiting,diuretic use,features of dehydration can diagnose these conditions.[1][2][3]
Afferent or central lesion of baroreceptor faliure. Causes such as bilateral structural lesions in the carotid sinuses, the glossopharyngeal nerves or brainstem due to tumor, injury, or other damage to afferent pathways can be identified by imaging studies.[4]
Parkinsons Disease,Pure autonomic faliure,Multiple system atrophy These are diagnosed mainly by the clinical history and examination described in the earlier sections.[4]
Non diabetic neuropathies Consists of the following. HIV (HIV IGg Ab testing), Syphilis(VDRL), Amyloidosis(urine and protein electrophoresis), Paraneoplastic syndrome(specific tests for suspected tumors), Renal dysfunction(serum electrolytes), Guillain-Barré syndrome (albuminocytologic dissociation, known as the elevation in CSF protein more than>0.55 g/L without an elevation in white blood cells Autoimmune diseases(specific tests like rheumatoid factor,ANA,d.s DNA)[1]
References
  1. FIGUEROA J. J., BASFORD J. R., LOW P. A.. Preventing and treating orthostatic hypotension: As easy as A, B, C. Cleveland Clinic Journal of Medicine [online] December, 77(5):298-306 [viewed 24 September 2014] Available from: doi:10.3949/ccjm.77a.09118
  2. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303
  3. LAHRMANN H., CORTELLI P., HILZ M., MATHIAS C. J., STRUHAL W., TASSINARI M.. EFNS guidelines on the diagnosis and management of orthostatic hypotension. Eur J Neurol [online] 2006 September, 13(9):930-936 [viewed 24 September 2014] Available from: doi:10.1111/j.1468-1331.2006.01512.x
  4. METZLER MANUELA, DUERR SUSANNE, GRANATA ROBERTA, KRISMER FLORIAN, ROBERTSON DAVID, WENNING GREGOR K.. Neurogenic orthostatic hypotension: pathophysiology, evaluation, and management. J Neurol [online] December, 260(9):2212-2219 [viewed 30 September 2014] Available from: doi:10.1007/s00415-012-6736-7

Investigations - for Diagnosis

Fact Explanation
Head-up tilt-table testing Head-up tilt-table testing should be ordered if there is a high index of suspicion for orthostatic hypotension despite normal orthostatic vital signs, and it may be considered in patients who are unable to stand for orthostatic vital sign measurements. The procedure is generally considered safe, but serious adverse events such as syncope and arrhythmias have been reported. [1]
Serum and urine protein electrophoresis and genetic testing electrophoresis for primary amyloidosis and genetic testing for hereditary amyloidosis.[1]
Magnetic resonance imaging In Multisystem atrophy (Shy-Drager syndrome) brain shows changes in putamen, pons, middle cerebellar peduncle, and cerebellum.Spinal cord injuries ,changes in transverse myelitis,changes in syringomyelia and spinal cord tumors can be observed from MRI if they are the cause for OH.[1]
Fasting blood glucose, ,Hb A1C Diabetic autonomic neuropathy can cause OH.[1]
Cardiac SPECT In Parkinson disease, pure autonomic failure and, Lewy body dementia it shows impaired uptake of iobenguane I 123[1]
Serum electrolytes and urea Chronic renal faliure can cause neuropathy resulting in OH.[1]
ECG,Echocardiography If heart diseases are the cause for OH these investigations can detect these.[1]
Rapid plasma reagent (RPR) or Venereal Disease Research Laboratory test (VDRL) and HIV testing These are needed to test for syphilis and HIV.[1]
Antinuclear antibody levels, erythrocyte sedimentation rate, and other autoimmune tests (eg, rheumatoid factor, SS-A and SS-B antibodies) Autoimmune screening helps to evaluate for collagen-vascular disease. [1]
References
  1. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303

Management - General Measures

Fact Explanation
Management of offending drugs. If possible withdraw the offending agent other wise potentially contributing medications should be instructed to take at bedtime when possible, particularly antihypertensives.If OH is caused by drugs these can minimise the symptoms of OH. [1][2]
Dietary modification Patients should avoid large carbohydrate-rich meals since this can cause splanchanic vaso dilation resulting in OH. (to prevent postprandial hypotension),Limit alcohol intake since this also cause vasodilation, and ensure adequate hydration.Older patients should consume a minimum of 1.25 to 2.50 L of fluid per day to prevent OH.[1][2]
Water boluses One 480-mL glass of tap water in one study and two 250-mL glasses of water in rapid succession in another study) have been shown to increase standing systolic blood pressure by more than 20 mm Hg for approximately two hours.[1][2]
Sodium supplements This can be done by adding extra salt to food or taking 0.5- to 1.0-g salt tablets. A 24-hour urine sodium level can aid in treatment. Patients with a value of less than 170 mmol per 24 hours should be placed on 1 to 2 g of supplemental sodium three times a day and be re-evaluated in one to two weeks, with the goal of raising urine sodium to between 150 and 200 mEq.[1][2]
Lower-extremity elastic stockings and abdominal binders An abdominal bandage may also be useful in attenuating orthostatic dysregulation by restricting splanchnic blood pooling.Elastic sockings cause venous compression and increase cerebral circulation alleviating the OH symptoms.[1][2]
Judicious exercise and certain physical maneuvers to avoid orthostatic hypotension. Isometric exercises involving the arms, legs, and abdominal muscles during positional changes or prolonged standing.Toe raises, thigh contractions, and bending over at the waist are recommended. Patients should actively stand with legs crossed, with or without leaning forward. Squatting has been used to alleviate symptomatic orthostatic hypotension These exercises and physical maneuvers act by reducing venous capacity and by increasing the total peripheral resistance. [1][2]
Avoid OH precipitating conditions. Avoid sudden head-up postural change,exposure to warm weather, hot baths( cutaneous vasodilatation), straining during micturition or defecation and valsalva manoeuvre.[1][2]
Head tilt up at night This is done by raising the bedhead by blocks or a polystyrene wedge beneath the mattress. Benefit may result from activation of the renin-angiotensin-aldosterone system, and other mechanisms that reduce recumbency induced diuresis. An improvement in cerebrovascular autoregulation may occur with time due to recurrent exposure to low blood pressure.[1][2]
Education Educate the patient and the family regarding posture,dietary modification,exercises to minimize the effects of OH.[1]
References
  1. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303
  2. MATHIAS C. J, KIMBER J. R. Treatment of postural hypotension. Journal of Neurology, Neurosurgery & Psychiatry [online] 1998 September, 65(3):285-289 [viewed 24 September 2014] Available from: doi:10.1136/jnnp.65.3.285

Management - Specific Treatments

Fact Explanation
Fludrocortisone. This is considered first-line therapy for the treatment of orthostatic hypotension and it acts by reducing salt and water loss, and possibly increases α-adrenoceptor sensitivity. Dosing should be titrated within the therapeutic range until symptoms are relieved, or until the patient develops peripheral edema or has a weight gain of 4 to 8 lb (1.8 to 3.6 kg). Adverse effects include headache, supine hypertension, and congestive heart failure. Hypokalemia, which is dose-dependent and can appear within one to two weeks .[1][2][3][4]
Ephedrine/Midodrine Ephedrine has both direct and indirect actions, and is of value in central autonomic failure, such as that due to multiple system atrophy. The dose is 15–45 mg thrice daily. It is best taken on waking up, with further doses before lunch and dinner. Its is not recommended at night, when its pressor effects are not needed; furthermore, it may cause insomnia. Other side effects, with higher doses, include tremulousness, a reduction in appetite, and, in males, urinary retention due to its effects on the urethral sphincter.[3][4] Midodrine is a peripheral selective alpha-1-adrenergic agonist, significantly increases standing systolic blood pressure and improves symptoms in patients with neurogenic orthostatic hypotension.26 Patients should not take the last dose after 6:00 p.m. to avoid supine hypertension. Adverse effects include piloerection, pruritus, and paresthesia. Its use is contra-indicated in patients with coronary heart disease, urinary retention, thyrotoxicosis, or acute renal failure. [1][2]
Pyridostigmine (Mestinon). It's a cholinesterase inhibitor that improves neurotransmission at acetylcholine-mediated neurons of the autonomic nervous system[1][2]. Adverse effects include loose stools, diaphoresis, hypersalivation, and fasciculations[3][4][5]
Erythropoietin This is used to treat OH in anemia.[4]
Octreotide This is used to treat OH in postprandial hypotension. [4]
References
  1. LANIER JB, MOTE MB, CLAY EC. Evaluation and management of orthostatic hypotension. Am Fam Physician [online] 2011 Sep 1, 84(5):527-36 [viewed 24 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21888303
  2. MATHIAS C. J, KIMBER J. R. Treatment of postural hypotension. Journal of Neurology, Neurosurgery & Psychiatry [online] 1998 September, 65(3):285-289 [viewed 24 September 2014] Available from: doi:10.1136/jnnp.65.3.285
  3. FERESHTEHNEJAD SEYED-MOHAMMAD, LöKK JOHAN. Orthostatic Hypotension in Patients with Parkinson’s Disease and Atypical Parkinsonism. Parkinson's Disease [online] 2014 December, 2014:1-10 [viewed 24 September 2014] Available from: doi:10.1155/2014/475854
  4. LOW PA, SINGER W. Update on Management of Neurogenic Orthostatic Hypotension Lancet Neurol [online] 2008 May, 7(5):451-458 [viewed 24 September 2014] Available from: doi:10.1016/S1474-4422(08)70088-7
  5. SINGER W. Acetylcholinesterase inhibition: a novel approach in the treatment of neurogenic orthostatic hypotension. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 September, 74(9):1294-1298 [viewed 24 September 2014] Available from: doi:10.1136/jnnp.74.9.1294