Postural orthostatic tachycardia syndrome is a from of dysautonomia. EDS patients can have a variety of neurological issues and disorders of the autonomous nerve system and POTS seems to be one of the most common.
The diagnosis of POTS is usually verified through a TILT table test by a cardiologist or through neurovegetative diagnose (mostly part of neurology). The Schellong Test can also help and show early results while the symptoms are acute.
POTS leads to a rapid increase of the heart rate if the patient is moved from supine to an upright position. An increase of more than 30 bpm (beats per minute) or > 120 bpm from supine to upright is pathologic. During the TILT table test the blood pressure and heart rate is continuous observed while the patient is brought from a supine in an upright position. Furthermore other additional tests during the TILT table test are recommended.
POTS can occur with a variety of different diseases or just alone without any other disorders. A connection between POTS and Fibromyalgia and between POTS and Chiari Malformation was also found. Furthermore it seems like a lot of EDS patients (especially with the hypermobile type) would develop POTS. Viral or bacterial infections (such as lyme) were also described as causative. Moreover another EDS related disease called mast cell activation syndrome (MCAS) was found to be a reason for POTS episodes. There is a lot more research needed here and in case of patients which were diagnosed with POTS it should be thought about a potential underlying disease.
The reason why POTS patients have this problems when standing upright seems to be the blood which is pooling in the vessels of the legs and abdomen, which leads to an increased secretion of norepinephrin and epinephrin. This in turn leads to the increase in heart rate.
De Wandele et al. (see EDS Publications) described in a couple of publications the mechanism of POTS in the EDS population. They suggest that Dysautonomia develops through different factors like cardiovascular, sudomotor dysfunction, neuropathy, laxity of connective tissue and under the influence of different medication.
high heart rate
shortness of breath
POTS symptoms can be triggered through:
after physical activity
Alcohol and sometimes coffee
warm environment (like a hot shower or warm temperatures)
some people have problems after eating because of the blood pooling during digestion
The therapy of POTS is a stepwise approach. In the beginning the avoidance of trigger factors should be first priority. Additional patients can use compression garmets or compression stockings. If long standing cannot be avoided, maneuvers like standing on the toes and rolling back to contract the leg muscles can be helpful to help with blood flow. After these therapies it should be tried to start again slowly with physical activity (like aerobic or cycling). If all these therapies do not help the use of medications can be helpful. There are drugs which increase the blood volume, interfere with the release and responds of epinephrine or norepinephrine or drugs which help with vasoconstriction.
Emergency information and surgery in Dysautonomia patients:
In emergency situations you should take into account that some medication is not well tolerated in Dysautonomia patients.
For example: Drugs like vasodilators, narcotics (like Codeine, Morphine, Oxycodone) and Phenothiazine, antiemetics (like Phenergan or Compazine), Niacin, Nortriptyline, Amitryptiline or tricyclic antidepressants (TCA), Epinephrine and Adrenaline.
You should always drink lots of fluid and have some electrolyts with you. They can help after acute fainting.
In case of surgery:
Because of the autonomous nerve system dysfunction our bodies cannot well regulate our bodies temperature. This means hyper- as well as hypothermia can occur frequently. There should be a constant temperature control.
HF and BP should be controlled more often than in healthy individuals especially when changing the patients position.
Epinephrine should be avoided because it causes increased tachycardia (but it might be necessary in patients with MCAS).
Vasopressors and extra fluid should be close in case of prolonged hypotonia.
Avoid Thiopental and Ketamine; Propofol might be better but caution: hypotonia.
Mustafa HI, Fessel JP, Barwise J, Shannon JR, Raj SR, Diedrich A, Biaggioni I, Robertson D. DysautonomiaPerioperative Implications. The Journal of the American Society of Anesthesiologists. 2012 Jan 1;116(1):205-15.
Corbett WL, Reiter CM, Schultz JR, Kanter RJ, Habib AS. Anaesthetic management of a parturient with the postural orthostatic tachycardia syndrome: a case report. British journal of anaesthesia. 2006 Aug 1;97(2):196-9.
Rabbitts JA, Groenewald CB, Jacob AK, Low PA, Curry TB. Postural orthostatic tachycardia syndrome and general anesthesia: a series of 13 cases. Journal of clinical anesthesia. 2011 Aug 31;23(5):384-92.