Postural Orthostatic Tachycardia Syndrome:

Transcription

1 Postural Orthostatic Tachycardia Syndrome: A Case Presentation Interesting Cases from the Annals of Women s Heart Care

2 I have no financial relationships or commercial interests to disclose that are relevant to this activity.

3 Learning Objectives: Recognize Participants will be able to recognize the diagnostic characteristics of Postural Orthostatic Tachycardia Syndrome (POTS) as experienced by a patient described in this case presentation. Identify Participants will be able to identify medical and non medical treatment interventions recommended in this specific POTS case. Understand After listening to this case presentation, participants will have a better understanding regarding how POTS may specifically affect the lives of our women patients.

4 JD is a 33 year old woman with a past medical history of migraine headaches with aura, chronic abdominal pain possibly related to sphincter of Oddi dysfunction, who was in otherwise good health. One day while at a bagel shop, she developed flushing, lightheadedness and a presyncopal episode. Initially she thought she was simply dehydrated. In the days and weeks after, she subsequently developed multiple episodes of near and complete syncope. Episodes were often associated with prodrome of nausea and vomiting. Over about eight weeks, patient estimated she had around 30 syncopal episodes.

5 Medications: PRN Maxalt, Zofran, Xanax (prior to flying), Percocet and Tramadol Social History: Engaged Works a financial advisor from home Smokes ¼ PPD x 10 years One glass of wine two times a week Denied Caffeine Family Medical History: Mother with DM and ischemic CM, heart transplant No other family history of CAD

6 Pertinent Positives Associated with Syncope Prodromal nausea and emesis Apple watch shows heart rates of beats per minute when upright with every day activity and just prior to syncopal episodes Unable to tolerate exercise and every day activities secondary to exacerbation of symptoms of dizziness, palpitations, fatigue, nausea Feels normal only when sitting with feet elevated Intermittent diarrhea and constipation Neurologic symptoms: ongoing migraine headaches and forgetfulness Acrocyanosis variation? JD reports discololeration in her lower extremities Symptoms ongoing longer than six months

7 Pertinent Negatives with Syncope No Seizure Activity Noted No Incontinence With Early Episodes Initially, No Knowledge Of Hypotension No Prolonged Confusion Or Altered Mental Status Post Syncope

8 Diagnostic Features of POTS Heart rate increase of greater than or equal to 30 beats per minute within 10 minutes (7) Symptoms worsen with standing and improved with recumbence (7) Symptoms last greater than or equal to six months (7) Absence of other overt cause of orthostatic symptoms or tachycardia (eg, active bleeding acute dehydration, medications) (7)

9 POTS and Women Prevalence is estimated at a rate of 170 cases per 100,000 individuals, but the actual number of cases is likely underestimated (4) Female to male ratio is 5:1 with an average age of onset age 30 (3) 80 to 85% of POTS patient are women of child bearing ages years (2) Symptoms often develop after pregnancy, major surgery, or viral illness (7) 80% of female patients report an exacerbation of symptoms around menstruation (5)

10 Diagnostics Echocardiogram: Normal chamber sizes, normal biventricular function, EF 55-60%, no significant valve disease, pulmonary artery pressures could not be estimated Heart Monitor: Sinus Rhythm and sinus tachycardia, sinus bradycardia, APC and PVC. With symptoms, patient had sinus rhythm and sinus tachycardia ECG: Normal sinus rhythm, normal QTC Tilt Table Test: Positive for tachycardia and syncope, peak heart rate 140 beats per minute with systolic blood pressure drop from 91 to 86 Orthostatic Blood Pressure: Supine BP 122/78, HR 79, sitting 125/86 and 89, standing 114/85 and 108 CT Pulmonary Angiogram: No evidence of acute or chronic thromboembolic disease, no other abnormalities

11 Diagnostics Labs: CBC normal, sodium 144, potassium 3.7, TSH 1.32, ANA negative, Sedimentation rate was 6, RF less than 9, CRP less than 5, Vitamin B12 206, dysautonomia panel negative, 24 hour urine for 5- HIAA normal, Cortisol level 4.7, Hepatitis A, B, and C negative, Cortrosyn stim test normal Head CT Without Contrast: No evidence of acute intracranial process MRI Of The Brain: Normal Examination MRI Of The Abdomen: Diffuse hepatic steatosis, previous cholecystectomy, no mass or biliary dilation Lower Extremity Venous Duplex: No evidence of acute or superficial venous thrombosis EEG: Non specific findings in the right temporal lobe, slowing and rare phase reversal sharp waves

12 Nonpharmacological Intervention Hydration: Recommend 8-10 cups of water Salt: Recommend a dietary intake of 8-10 grams per day Support Stocking: full leg, girdles with mm Hg of pressure Intravenous Fluids: 1-2 liters of normal saline over an hour, two times a week administered by the infusion clinic Exercise: Recumbent bike

13 Pharmacological Interventions Midodrine: peripheral a-1 agonist serving as a vasoconstrictor. Initiated 5 mg three times a day with gradual uptitration to 10 mg three times a day Pyridostigmine: peripheral acetylcholinesterase inhibitor that can increase levels of synaptic acetylcholine at the autoganglia and peripheral muscarinic parasympathetic receptors. Initiated 60 mg three times a day Neurology recently added lamotrigine 25 mg, used to treat seizure disorder, twice a day with plans to up titrate to 100 mg twice a day. Vitamin B12 injections also ordered in hopes to improve brain fog and fatigue.

14 Patient Impact Approximately 25% of POTS patients are disabled and unable to work (1) Researchers found that the quality of life in POTS patients is comparable to patients on dialysis for kidney failure (6) Frequency of Episode: Multiple episodes daily and after treatment, episodes reduced to one a day Driving: Recommended patient avoid driving Pregnancy: Medical therapy has not been studied well in pregnancy Career: JD has been able to continue to work from home Referrals: Neurology follows locally and JD has been referred to Vanderbilt University for further evaluation and treatment

15 Conclusions Patients suffering from POTS may experience a myriad of symptoms including dizziness, near and full syncope, tachycardia, fatigue, sleep disturbance, brain fog, and irritable bowel symptoms. In adult women, the diagnostic feature that is specifically seen is a heart rate increase of greater than or equal to 30 beats per minute within 10 minutes of standing. With a combination of non pharmacological and pharmacological interventions, a majority of patients diagnosed and treated are able to return to a satisfactory lifestyle.

16 References 1. Blair P Grubb. Postural Tachycardia Syndrome. Circulation. 2008; 117: Garland EM, Raj SR, Black BK, Harris PA, Robertson D. The hemodynamic and neurohumoral phenotype of postural tachycardia syncdrome. Neurology. 2007;69: Low PA, Sandroni P, Joyner M, Shen WK (March 2009). "Postural tachycardia syndrome (POTS)". Journal of Cardiovascular Electrophysiology. 20 (3): Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R (December 2011). "Postural tachycardia syndrome--current experience and concepts". Nature Reviews. Neurology. 8 (1): Peggs KJ, Nguyen H, Enayat D, Keller NR, Al-Handy A, Raj SR. Gynecologic disorders and menstrual cycle lightheadedness in postural orthostatic tachycardia syndrome. Int J Gynaecol Obstet. 2012;118: POTS-A World Tour, lecture presented by Dr. Satish Raj during the 2013 Dysautonomia International Conference. 7. Raj, SR. Postural tachycardia Syndrome (POTS). Circulation. 2013;127: