Correspondence

Familial Occurrence of Neurocardiogenic Syncope

N Engl J Med 1994; 331:205July 21, 1994

Article

To the Editor:

We report two cases of neurocardiogenic syncope in a family. A 19-year-old college student was evaluated for abrupt loss of consciousness which had occurred approximately twice each year since the age of 12. Syncopal episodes were associated with abdominal cramping during menstrual periods. An electrocardiogram, a 24-hour Holter-monitor recording, and an echocardiogram were normal. Tilt-table testing was performed. After the patient had been in the head-up position at 60 degrees for nine minutes, she became asystolic and lost consciousness. Asystole lasted 70 seconds. Atropine was administered, and cardiac massage was initiated. The patient recovered. That evening, in the hospital, the patient had a syncopal episode when arising from bed. Cardiac monitoring demonstrated a 30-second period of asystole. Tilt testing was repeated after prophylactic insertion of a temporary pacemaker. Sinus bradycardia and hypotension developed. Oral metoprolol, initiated in a dose of 50 mg four times a day, prevented the bradycardic and hypotensive response to tilt testing.

The patient said that her mother also had episodic syncope. When interviewed, her mother described recurrent syncope, usually occurring during phlebotomy or dental procedures. She agreed to undergo tilt-table testing; within 2 minutes after she was placed in a 60-degree head-up position, asystole developed, lasting 47 seconds.

Neurocardiogenic syncope is a cause of some cases of otherwise unexplained syncope.1 We are not aware of a familial clustering of this disorder. It is possible that the diagnosis in these cases represents a random association. This seems unlikely, given the unusual expression of profound asystole in both mother and daughter.

Christopher J. Cooper, M.D.
Paul Ridker, M.D.
Julie Shea, R.N., M.S.
Mark A. Creager, M.D.
Brigham and Women's Hospital, Boston, MA 02115

1 References

1. 1

   Almquist A, Goldenberg IF, Milstein S, et al. Provocation of bradycardia and hypotension by isoproterenol and upright posture in patients with unexplained syncope. N Engl J Med 1989;320:346-351
   Full Text | Web of Science | Medline

Citing Articles (5)

Citing Articles

1. 1

   Burcu Topcu, Figen Akalın. (2010) The autonomic nervous system dysregulation in response to orthostatic stress in children with neurocardiogenic syncope. Cardiology in the Young 20:02, 165
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   L. R.A. Olde Nordkamp, W. Wieling, A. H. Zwinderman, A. A.M. Wilde, N. van Dijk. (2008) Genetic aspects of vasovagal syncope: a systematic review of current evidence. Europace 11:4, 414-420
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   PETER MITRO, KLAUDIA MUDRÁKOVÁ, HELENA MIČKOVÁ, JÁN DUDÁŠ, PETER KIRSCH, GABRIEL VALOČIK. (2008) Hemodynamic Parameters and Heart Rate Variability during a Tilt Test in Relation to Gene Polymorphism of Renin-Angiotensin and Serotonin System. Pacing and Clinical Electrophysiology 31:12, 1571-1580
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   M. Yousuf Kanjwal, Blair P. Grubb. (2005) Evaluation of syncope. Current Cardiology Reports 7:5, 329-335
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5. 5

   Lewis, Steven L., , O'Toole, Michael, . (1994) Familial Occurrence of Neurocardiogenic Syncope. New England Journal of Medicine 331:22, 1529-1529
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