Overview
Primary orthostatic hypotension is a rare autonomic nervous system disorder characterized by a significant drop in blood pressure upon standing from a seated or lying position, occurring as a primary (idiopathic) condition rather than secondary to another disease. This condition results from a failure of the autonomic nervous system to appropriately regulate blood pressure during postural changes. The underlying defect involves impaired norepinephrine release from sympathetic nerve endings or deficient autonomic reflex pathways that normally constrict blood vessels and increase heart rate when a person assumes an upright posture. Key symptoms include dizziness, lightheadedness, blurred vision, weakness, fatigue, and syncope (fainting) upon standing. Some patients may also experience neck and shoulder pain (coat-hanger pain), cognitive difficulties during upright posture, and exercise intolerance. In severe cases, recurrent falls and loss of consciousness can significantly impair quality of life and daily functioning. The condition primarily affects the cardiovascular and autonomic nervous systems. Treatment is largely supportive and symptomatic. Non-pharmacological measures include increased fluid and salt intake, wearing compression garments, sleeping with the head of the bed elevated, and avoiding prolonged standing or rapid postural changes. Pharmacological options may include fludrocortisone (a mineralocorticoid to expand blood volume), midodrine (an alpha-adrenergic agonist to increase vascular tone), and droxidopa (a norepinephrine precursor). Management is individualized based on symptom severity and patient response.
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Primary orthostatic hypotension.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Primary orthostatic hypotension.
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Common questions about Primary orthostatic hypotension
What is Primary orthostatic hypotension?
Primary orthostatic hypotension is a rare autonomic nervous system disorder characterized by a significant drop in blood pressure upon standing from a seated or lying position, occurring as a primary (idiopathic) condition rather than secondary to another disease. This condition results from a failure of the autonomic nervous system to appropriately regulate blood pressure during postural changes. The underlying defect involves impaired norepinephrine release from sympathetic nerve endings or deficient autonomic reflex pathways that normally constrict blood vessels and increase heart rate when
At what age does Primary orthostatic hypotension typically begin?
Typical onset of Primary orthostatic hypotension is adult. Age of onset can vary across affected individuals.
Which specialists treat Primary orthostatic hypotension?
22 specialists and care centers treating Primary orthostatic hypotension are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.