Notes From the 2023 Dysautonomia International Conference

The conference was a bit different this year as it was in person after a few years of being on Zoom due to the pandemic. Luckily, Dysautonomia International still offered a virtual option where the recordings would be sent at a later date. I chose that option as I live far from DC.

The number of recaps I have is on the light side this year. Not all of the sessions are available in the virtual portal (at least not yet), and of those, only a few piqued my interest. A year is not a long time in terms of research, so topics I have heard covered in the past, I tended to skim to see if there was any new information.

Every Breath You Take: Hyperpnea, Hypocapnia, and POTS. By Glen Cook MD

• The big POTS study showed that 88% of POTS patients have shortness of breath.
• The decreased central blood volume typical in POTS leads to a decreased cardiac output, this causes a drop on the stretch of the baroreceptors in the carotid sinus, the medulla responds, sympathetic nervous system activity increases, ventilation and heart rate increases, the pH of the blood increases, and this causes a constriction of vasculature to the brain.
• The decrease in central blood volume can have various causes depending on the POTS patient.
• Increased ventilation in POTS is different than increased ventilation in panic attacks or self-induced increases in ventilation.
• A research group from Vanderbilt is studying if a device the creates inspiration resistance improves POTS symptoms.
• Different research groups are also studying POTS from this angle with different interventions such as a compressive neck collar or the modulation of P2X3 receptors.
• Dr. Cook wonders if other breathing interventions will prove useful with future study (biofeedback-esque interventions).

Research Updates of POTS. By Satish Raj MD.

• POTS and pregnancy study. Generally, patient’s POTS symptoms got worse during pregnancy (around 60% of patients). The rest were the same or got better. The first trimester symptom burden tended to predict second and third trimester symptoms burdens. 3.6% out of 218 studied report that pregnancy triggered their POTS.
• POTS and EDS (Ehlers-Danlos Syndrome). Around 25% of POTS patients have EDS in their study. 28% of patients that have POTS and EDS also have mast cell activation syndrome (MCAS), while only 5% of patients with POTS and no EDS have MCAS. That is a very significant finding. EDS patients are usually diagnosed earlier in their lives but tend to have a longer diagnostic delay. EDS patients are more likely to have a family history of fainting or POTS (may demonstrate a genetic component). Neurological and GI symptoms rates are slightly higher in the EDS group than the only POTS group.
• POTS and compression garments. The study demonstrated that compression decreased heart rate upon standing and decreased symptom burden. Several areas of compression were studied- full (legs and abdomen), abdomen, legs, and none as a control. Full compression is the most effective followed by abdomen and then drop off to legs which was modestly higher than no compression. Dr. Raj’s lab extended this study to commercial products used at home. In his words, they used “butt ugly” neoprene and velco wraps in the lab study. He’s a hoot. The home study demonstrated the same results- improvement in heart rate and improvement symptoms. They did not control garments used, they just gave the guideline of waist-high compression stockings.
• Autonomic function in long Covid. He said us patients who had POTS before Covid should thank the Covid patients because before “no one gave a crap” (except more colorful language). I appreciate his honesty and agree that Covid has furthered the study of POTS. In this study they did an active stand test with beat-to-beat monitoring. The patients were on average 1 year post-Covid. 73% of the patients in the study had a cardiac autonomic abnormality (CAA). 61% had initial orthostatic hypotension (IOH), 30% had POTS, 2.9% had OH (orthostatic hypotension), and 1.4% had IST (inappropriate sinus tachycardia). Most groups were biological female dominant, except the IOH group which was more evenly split between biological males and biological females. A small percent improved 3 months after the initial study (so roughly 12 months vs 15 months post-Covid).
• Q&A. Dr. Raj has had post-Covid vaccine POTS patients as had a physician who asked a question. This hasn’t been studied yet as it was hard to find enough people to participate in the study. Dr. Raj noted that he found the 3rd shot worsened some of his patients that already had POTS. I found that interesting because it was my 3rd shot that did me dirty. For compression stockings, he doesn’t necessarily recommend a single brand, but he says he usually tells his patients to get waist high compression tights from a sporting goods store. He said some of his patients like the brand 2XU.

Using an Ear Device to Monitor Blood Flow to the Head as a Dysautonomia Management Tool. By Daniel Lee MD and Marat Fudim MD.

• The tool is called STAT, it is a wearable that measures a shallow ear artery.
• It is the equivalent of a continuous glucose monitor, but for cardiovascular disease.
• The device will measure flow changes in the external carotid artery.
• Testing has demonstrated that the device is effective in its measurements compared to TCD ultrasound devices and carotid ultrasound. The testing for this has been small, so they hope to expand it further in the future.
• Other methods of measuring blood flow changes have limitations that this device hopes to remedy by being ambulatory, realistic, discreet, continuous, and user-friendly.
• The device links with an app that can be used as a pacing tool.
• The product is launching its pre-order on October 19th. This is the link to Stat Health’s website.

Brain Fog, Cognitive Dysfunction and Blood Flow in the Dysautonomia Brain. By Amanda Miller PhD.

• Dr. Miller is a Dysautonomia patient.
• Brain fog can be triggered even while lying down.
• Language, executive function, complex attention, learning, and memory are all domains affected in POTS.
• Attention scores for POTS sit between the level of normal controls and people with ADHD.
• Ruff 227 speed scores were worse in POTS patients than controls.
• POTS patients performed worse on identification tasks when upright compared to lying down. When lying down, the POTS patients performed roughly the same as the controls.
• POTS patients performed significantly worse than controls on executive function testing (in this case the stroop color word test).
• Working memory scored lower in POTS and CFS patients than controls (verbal n-back test).
• There is no difference in psychomotor speed or verbal fluency between POTS patients and controls, which suggest a selectivity in the cognitive deficits.
• 1/3 of POTS patients had mild impairment in executive functioning.
• Attention seems to be affected by position.
• Treatment- 70% of patients using stimulants demonstrate improvement. Patients report these as the top 5 treatments for brain fog: IV saline, stimulants, salt tablets, B-12 injections, and midodrine.
• In POTS, mild changes in blood pressure alter cerebral blood flow. Healthy people’s blood pressure can vary, but cerebral blood flow remains constant.
• Decreased brain blood flow precedes tachycardia.
• Dr. Miller is doing a study where fMRIs will be taken during cognitive testing on POTS patients under orthostatic stress (lower body negative pressure).

Dysautonomia After Covid- What Should My Doctor Do? By Liza Fisher and Svetlana Blitshteyn MD.

• Liza mentioned a therapy that I had not heard of that helped her- external counterpulsation therapy (EECP).
• Of Dr. Blitshteyn’s patients with post-Covid dysautonomia, 60% have been unable to return to work.
• A number of physicians can manage long haul Covid including a PCP, physiatrist, neurologist, cardiologist, pulmonologist, PT, psychiatrist/psychologist. The main thing is that they need to be compassionate and knowledgeable.
• Dr. Blitshteyn and colleagues wrote a paper outlining guidelines for the assessment and treatment of post-Covid Dysautonomia.
• This is the link to that paper. It is a beneficial resource for both the patient and the physician to use to collaborate on a treatment plan for post-Covid dysautonomia.
• A lot of the interventions and assessments covered were things that have been staples in this field, but there were a few things that were newer such as the recommendation of a short course of steroids. The paper is also comprehensive, and having all that info in one place is fantastic.

Immunotherapy for POTS. By Tae Chung MD.

• Proposed mechanism of POTS: pathogen, immune system fights it, molecular mimicry occurs, immune system then attacks its own nervous system.
• Volume expansion therapies treat the symptoms, but not the underlying cause. Immunotherapy targets the underlying cause. It’s not a cure, but it still address more of the underlying pathophysiology.
• When to consider immunotherapy: when autoimmunity is clinically suspected such as a post-infectious onset, volume expansion fails to restore function, flares are too frequent and too debilitating, and only when the potential benefit outweighs potential side effects.
• Potential benefits: disease modifying effects, more powerful, may restore function, and may possibly prevent flares.
• Potential concerns: immune suppression, lack of biomarker/outcome, uncertainty, and drug side effects.
• Dr. Chung aims to rule out non-immune mediated POTS via a workup to make sure it truly is the correct path of treatment.
• Immunotherapy drugs: temporary high-dose steroids (sometimes in conjunction with a secondary agent), IVIG, Subcutaneous IG, and agents like plaquenil, methotrexate, azathioprine, mycophenolate mofetil, and rituximab.
• High-dose steroids have a lot of side-effects and are not ideal beyond a short bout.
• IVIG- may take several months for symptoms to respond. IVIG is very expensive and there is a high rate of denial from insurance. It is common for POTS patients to get bad headaches. fatigue, and pain from infusions. Infusions can also be a hassle.
• Subcutaneous IG tends to be tolerated better. It is difficult to find an equivalent dose with IVIg if patients do not have enough subcutaneous tissues. Dr. Chung usually starts patients with IVIG, and the switches to subcutaneous IG.
• Dr. Chung doesn’t use agents like plaquenil and the others listed above very often.
• There isn’t clear evidence to support any of these immunotherapy agents at this point. More studies need to be done.
• Randomized controlled trial studies need to occur for this to become more widespread and more acceptable to insurance.

Venous Disease and Dysautonomia, Underdiagnosed and Misunderstood. By Alexis Cutchins MD.

• Dr. Cutchins stated that this is largely anecdotal based on her experience.
• Venous disease causes blood pooling in the lower extremities or pelvis, contributing to POTS.
• There is a lot of symptom overlap between POTS and pelvic venous disease.
• Symptoms of lower extremity venous insufficiency include aching legs, swollen feet and ankles, burning or tingling in legs, cramps in legs especially at night, itchy and thin skin over affected vein, and pain that worsens upon standing.
• Iliac vein compression- left iliac vein becomes compressed by the right iliac artery. Symptoms include left leg symptoms such as swelling, heaviness, and varicosity.
• Nutcracker syndrome- entrapment of left renal vein between the abdominal aorta and superior mesenteric artery. Symptoms include hematuria, orthostatic proteinuria, flank pain, abdominal pain, pelvic venous congestion, fatigue, orthostatic intolerance, dysmenorrhea.
• Pelvic venous disease overall typically features pelvic pain that worsens in an upright position, dyspareunia, vulvodynia, dysuria, low back or hip pain, and varicosities.
• It is quite possible that mixed connective tissue disease seen in the POTS population makes them more likely to develop venous disease.
• In a study, 70% of POTS patients had iliac vein compression while only 40% of the normal group had iliac vein compression.
• Treatment for lower extremity venous insufficiency includes compression garments, radio frequency ablation, or supplementation with diosmin.
• Treatment for nutcracker includes aspirin, renal vein stent, or autologous renal transplant.
• Treatment of iliac vein compression includes an endovascular stent or sclerotherapy/coiling.
• Treatment in terms of Dysautonomia is not clear. Concerns include MCAS and reactivity, stent embolization, stent thrombosis, infection risk, and worsening of symptoms.
• One patient of Dr. Cutchins had MCAS and Dysautonomia along with lower extremity venous insufficiency. Venous ablation had a profound positive effect on her Dysautonomia symptoms.
• Another patient had POTS and mild iliac vein compression. An iliac vein stent improved pelvic and dysautonomia symptoms.
• It’s not a cure to treat these venous diseases, but they may be helpful in the improvement of dysautonomia symptoms of these patients.
• Dr. Cutchins screened all her patients- 88% had lower extremity venous insufficiency and 80% had pelvic venous disease.
• The data from 6 of her dysautonomia patients that were treated for their venous diseases ended up with significant improvement across broad areas of symptoms.
• Dr. Cutchins does all the usual treatments for her patients but also evaluates them for venous disease using both an extensive review of systems and imaging.
• She orders doppler imaging for suspected lower extremity venous insufficiency and venograms for suspected pelvic venous disease.

Disclaimer: I am not a medical professional. Statements on this site are not meant to be taken as medical advice. These statements reflect my personal experiences having mild-ish post-viral POTS and ME. Due to the wide spectrum of these diseases, comorbidities, and everyone being different, your experiences may be very different than mine.

Note: If you post a comment, this site does NOT have a feature to notify you of responses to your comment. I have not found a good solution for that yet. However, I usually respond to every comment in a timely manner, so be sure to check back.