From the Guidelines
Dysautonomia is a disorder of the autonomic nervous system that controls involuntary bodily functions, and its management should prioritize addressing specific symptoms to improve morbidity, mortality, and quality of life.
Definition and Causes
Dysautonomia occurs when the nerves that carry information from the brain to these organs are damaged or malfunction, leading to a range of symptoms including dizziness, fatigue, and fainting. The condition can be primary, such as Postural Orthostatic Tachycardia Syndrome (POTS) or Pure Autonomic Failure, or secondary to conditions like diabetes, Parkinson's disease, or autoimmune disorders 1.
Treatment Approaches
Treatment typically involves addressing specific symptoms and may include:
- Medications such as fludrocortisone (Florinef) at 0.1-0.2 mg daily to increase blood volume, midodrine (ProAmatine) at 2.5-10 mg three times daily to raise blood pressure, and beta-blockers like propranolol (10-40 mg twice daily) to manage tachycardia 1.
- Non-pharmacological approaches, including:
- Increasing salt intake to 3-10 grams daily
- Maintaining hydration with 2-3 liters of fluid daily
- Wearing compression stockings
- Performing recumbent exercises
- Using techniques like counter-maneuvers when changing positions
These interventions help because dysautonomia often causes blood pooling in the lower extremities and inadequate blood return to the heart, leading to symptoms like dizziness, fatigue, and fainting 1.
Recent Guidelines
The 2019 ACC/AHA/HRS versus ESC guidelines for the diagnosis and management of syncope provide updated definitions and recommendations for orthostatic hypotension, orthostatic intolerance, and postural orthostatic tachycardia syndrome (POTS) 1. The 2018 ESC guidelines for the diagnosis and management of syncope also provide practical instructions for the diagnosis and management of dysautonomia 1.
Key Considerations
When managing dysautonomia, it is essential to prioritize addressing specific symptoms to improve morbidity, mortality, and quality of life. A comprehensive treatment plan should include a combination of pharmacological and non-pharmacological approaches, tailored to the individual patient's needs and medical history. Regular monitoring and follow-up are crucial to adjust the treatment plan as needed and ensure optimal symptom management.
From the Research
Definition and Classification of Dysautonomia
- Dysautonomia covers a range of clinical conditions with different characteristics and prognoses, including Reflex Syndromes, Postural Orthostatic Tachycardia Syndrome (POTS), Chronic Fatigue Syndrome, Neurogenic Orthostatic Hypotension (nOH), and Carotid Sinus Hypersensitivity Syndrome 2
- Cardiovascular Autonomic Neuropathy (CAN) is a term used to define dysautonomia with impairment of the sympathetic and/or parasympathetic cardiovascular autonomic nervous system 2
Diagnosis and Investigation of Dysautonomia
- Detection of Orthostatic Hypotension (OH) is a late sign and means greater severity in the context of dysautonomia, defined as Neurogenic Orthostatic Hypotension (nOH) 2
- Valsalva, Respiratory and Orthostatic tests (30:15) are the gold standard methods for the diagnosis of CAN, and can be associated with RR Variability tests to increase sensitivity 2
- The Tilt Test should not be the test of choice for investigating CAN at an early stage, as it detects cases at more advanced stages 2
Treatment and Management of Dysautonomia
- Treatment of patients at moderate to advanced stages of dysautonomia is quite complex and often refractory, requiring specialized and multidisciplinary evaluation 2
- Midodrine, droxidopa, fludrocortisone, and domperidone may be used for OH treatment 3, 4
- Sialorrhea can be managed with botulin toxin injections and oral glycopyrrolate, while erectile dysfunction can benefit from sildenafil treatment 3, 4
- Preventive measures such as postural care, good hydration, higher salt intake, use of compression stockings and abdominal straps, portioned meals, and supervised physical activity are important treatment steps 2
Specific Conditions and Their Management
- Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia characterized by sustained elevation of heart rate in an orthostatic position, and can be treated with propranolol, bisoprolol, and pyridostigmine 5
- Chronic Fatigue Syndrome represents a form of Dysautonomia, and has been renamed as a systemic disease of exercise intolerance, with new diagnostic criteria 2
- Fludrocortisone is a mineralocorticoid that increases blood volume and blood pressure, and is considered the first- or second-line pharmacological therapy for orthostatic hypotension alongside mechanical and positional measures 6