Ehlers Danlos Syndrome and POTS

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Submitted by Dr T on August 22, 2013 – 9:18am

Hi Dr T,
My daughter is 21 years old and has a Connective Tissue Disorder called Ehlers Danlos Syndrome. She has had ongoing gastrointestinal issues since infancy, which have been diagnosed recently as Irritable Bowel Syndrome. She has also had 2 episodes of syncope, and multiple episodes of pre-syncope ( several times a week) since about 10 years of age. She also has chronic migraines/headaches, severe fatigue since infancy, altered sweating patterns, poor night vision, very cold, pale hands and feet (often with acrocyanosis), frequent shortness of breath and chest discomfort. We took HR and BP measurements after 10 mins supine, and during 3 minutes standing over a 5 month period. Measurements showed a consistent HR increase after 3 mins standing of >30 bpm (often around 56 BPM). Diastolic BP also dropped 10-19 on many occasions, whereas SBP dropped >20 only very rarely. She had a HUT test recently. Her supine rates were HR 77, BP 115/75. The HUT which showed a sustained drop of 25 SBP & of 5 DBP over 5 mins of HUT, with a fluctuating HR increase between 13 – 22 BPM. After 5 mins HUT sublingual Nitroglycerine was administered, and  HR increased over the next 15 mins to 133 (increase of 56 over supine reading), SBP fell to 80 for the next 5 mins, but increased slowly after that to 83 for remainder of test. DBP initial fell to 60( a decrease of 17 from supine reading) but rose after 2 mins back to 70 (the same reading prior to the Nitro) and remained at that level for remainder of HUT. Recovery rate after HUT was HR 90, and BP 110/80. The conclusion noted on the HUT results was Orthostatic Hypotension. Excessive blood pooling was given as the reason. I have 2 queries I hoped you might be able to shed some light on. Firstly, even though we followed best practice guidelines for doing the 5 months of home measurements of BP and HR readings supine and standing, the results of the HUT were quite different to our readings, and seem to indicate a very different picture of what is going on to what we had first thought. I am wondering if there is any obvious reason for the two very different sets of results. Secondly, in trying to research types of Orthostatic Intolerance, I have come across many inconsistencies and contradictions in how Orthostatic Hypotension and Postural Orthostatic Tachycardia are defined. The most confusing is that Chronic Orthostatic Hypotension is often not mentioned, or if mentioned is not associated with any significant rise in HR. Also, with POTS, many sources state it is a sustained  increase of ?30 BPM after 3 mins standing, with NO orthostatic hypotension. Other sources state that there may be an abnormal drop in BP as well, but this is less common in POTS. I read with interest the information on your website which states that a significant drop in BP should be part of the signs indicative of POTS. I would greatly appreciate any information on why there are so many inconsistencies and contradictions from so many reputable sources regarding the diagnostic criteria for POTS, and how do doctors and patients make sense of this in order to diagnose whether someone has POTS or not?

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