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Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia associated with variety of symptoms like Headache, Abdominal discomfort, Dizziness/presyncope, Nausea, Fatigue, Lightheadedness, Sweating Sleep disorder, Tremor, Anxiety, Palpitations, Exercise intolerance which is believed to be caused by an underlying infectious and/or autoimmune trigger. HYQVIA is an immune globulin with a recombinant human hyaluronidase indicated for the treatment of Primary Immunodeficiency (PI) in adults. This includes, but is not limited to, common variable immunodeficiency (CVID), X-linked agammaglobulinemia, congenital agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies. HYQVIA is a gammaglobulin and is a slow metabolizer given subcutaneously once a month.There is data that Intravenous immunoglobulin(IVIg) helps POTS patients. We present a case of 57 year old female diagnosed with autonomic failure,orthostatic intolerance and primary immune deficiency. She was given IVIg for primary immune deficiency. She was previously reported for severe autonomic failure. From then, she was doing extremely well, had more energy and she thinks more clearly. She also had much better attitude. She was getting albumin every 2 weeks or so for volume expansion. She felt IVIg had helped her. Her immunoglobulin was switched from Gammagard to HYQVIA 35 grams. After she did, her blood pressure had gone up. She took it with albumin and she felt palpitations and chest pain.She was stopped on albumin, as albumin and HYQVIA combined could cause more volume expansion and push her into fluid overload or even congestive heart failure. After she stopped albumin without HYQVIA, she did well. Similarly, she cut back on midodrine which is an α1 adrenergic agonist, and works by stimulating receptors that noradrenaline normally works on. After swallowing, midodrine is rapidly converted into another, more active drug that binds to noradrenaline receptors causing blood vessels to narrow, thereby increasing blood pressure andreduction in heart rate in POTS patients. She was only HYQVIA and had been feeling extremely well.

Background: Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia associated with variety of symptoms like Headache, Abdominal discomfort, Dizziness/presyncope, Nausea, Fatigue, Lightheadedness, Sweating Sleep disorder, Tremor, Anxiety, Palpitations, Exercise intolerance. Median arcuate ligament syndrome (MALS, also known as celiac artery compression syndrome) is a condition characterized by abdominal pain, delayed gastric emptying, nausea, weight loss and other symptoms of autonomic dysfunction attributed to compression of the celiac artery and possibly the celiac ganglia by the median arcuate ligament. The researchers suggest that MALS should be considered in POTS patients who have persistent gastrointestinal symptoms. The purpose of this research is to study the celiac artery velocity in POTS patients. That led us to think about an operative procedure that can reduce POTS symptoms through MALS surgery. Patients and Method: We present the case of a 17 year old male with POTS symptoms not improving with medical management and celiac artery ultrasound positive for MALS.His celiac artery velocities was higher in Inspiration as well as Expiration and MALS is confirmed with CT angiogram.He underwent laparoscopic MALS surgery where the Median arcuate ligament is flipped thereby decreasing the extrinsic compression on the celiac artery. Results: There appears to be tremendous improvement in the patient’s symptoms after the surgery for MALS.His nausea had almost disappeared.Before surgery he had vomiting twice a week, after surgery he had vomiting four times in three months. His lightheadedness had disappeared. Bluish discoloration of arms and feet when he used to stand had disappeared in the hospital after the procedure. Sensations of hands had improved. Heat intolerance had improved. His Sleep and constipation improved. There was no much improvement in fatigue. Autonomic tilt table test is repeated after surgery to see if there is definite objective change, Heart rate in the first 10 minutes of tilt test before the surgery was >120bpm,Heart rate after the surgery was <100 bpm. Conclusions: Laparoscopic MALS surgery was found to be extremely helpful in relieving POTS symptoms with immediate results.

A 77 year old man was referred to us with fainting spells especially after standing/walking few steps. Initially he went to a neurologist and ENT specialist where the cause could not be determined and symptoms continued. During annual physical, he noticed that his blood pressue dropped when he stood up suddenly. He had severe fainting spells and was placed pacemaker in him.However,pacemaker did not make a huge difference.Blood Pressure monitoring was done and noticed 50 mmHg drop upon standing. Pacemaker was changed to constant pacing where his dizziness continued but he had no falls. He had periodic vision problems, numbness in fingers and legs, digestive problems, stomach issues and balance problems. His tilt table test showed orthostatic hypotension. Medical management (florinef, mestinone, midodrine) did not relieve his symptoms .Pacemaker sensor was turned on and made more sensitive that after he taps on the pacemaker, heart rate increases for 10 minutes. We told him to tap on pacemaker whenever he is dizzy and see if there is any improvement in orthostatic symptoms. The later treatment had made a huge difference; he is practically asymptomatic and was very much satisfied with his current health. He is using the tapping maneuver 4-5 times a day and his fainting disappeared and no syncope. First time our case report on Pacemaker tapping demonstrated significant improvement in treating symptoms of orthostatic hypotension.

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