Authors
EShruthi Radhakrishnan1,BS, Vani Gupta1,BS, Harrison Dean2, BS, Bernadette Riley1,DO, Todd J Cohen1, MD
1New York Institute of Technology College of Osteopathic Medicine, Old Westbury, NY
2New York Institute of Technology College of Osteopathic Medicine at Arkansas State University, Jonesboro, AR
Introduction
Neurocardiogenic syncope is an exaggerated triggered response of the autonomic nervous system with resultant hypotension and bradycardia. Asystole, however, is rare and might limit one's physical activity if severe enough. We present a case report of a competitive skater experiencing dramatic recurrent syncope with asystole.
Case Presentation
A 27-year-old female competitive ice skater with hypermobile Ehlers-Danlos Syndrome (hEDS) Type III, anxiety, and a long QTC interval of 470ms was referred for evaluation of palpitations and recurrent and dramatic syncope. Medications included sertraline for her anxiety. Work-up included genetic testing for EDS and Long-QT syndrome, which ruled out vascular EDS and congenital long-QT genes. Extended telemetry demonstrated sinus arrest following venipuncture (20 seconds with a rare atrial escape rhythm of 9 bpm) with associated syncope. An echocardiogram revealed mild mitral valve prolapse and mild left ventricular hypertrophy, with normal ejection fraction. A passive Tilt table test (without drug therapy) at 70 degrees resulted in an asystolic arrest (30 seconds with one junctional escape beat). She was initially treated with metoprolol (ineffective), then switched to pindolol (resulted in insomnia), and eventually tolerated acebutolol. She forgot to take her medication and felt severe presyncope and went to the ER, where they performed another venipuncture (while sitting) with resultant sinus arrest lasting 30 seconds with a rare junctional escape beat and complete loss of consciousness. Because of the dramatic nature and extent of asystole during syncope, a repeat scheduled venipuncture was performed in our clinic in the Trendelenburg position while wearing support stockings and monitored on beta-blocker therapy. The patient reported feelings of presyncope, but no loss of consciousness, and ECG showed no change in heart rate. She received an implantable loop recorder (ILR) to correlate symptoms to bradycardia and determine the effectiveness of therapy.
Discussion
Patients with hEDS are typically predisposed to postural orthostatic syncope. Neurocardiogenic syncope is less common, and the severe asystolic variant has been rarely reported in this population. This case demonstrates the importance of telemetry monitoring (external and implantable) in order to help define the mechanism of syncope and demonstrate the effectiveness of therapy. Although the documented pauses and asystole were dramatic, a pacemaker may be unnecessary, especially with patient education, salting, hydration, support stockings, and beta-blocker therapy. She was advised to minimize venipunctures unless absolutely necessary, pre-hydrate with electrolyte solutions, wear support stockings, and be in Trendelenburg during future venipunctures. Our investigation demonstrated the effectiveness of this approach and the benefit of ILR monitoring to ensure the long-term effectiveness of therapy in this hEDS patient with asystolic neurocardiogenic syncope.
References
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