(Circulation. 2000;102:e46.)
© 2000 American Heart Association, Inc.
Images in Cardiovascular Medicine |
From the University of Florida, Jacksonville (D.S.G.), and the University of Rochester, Rochester, NY.
Correspondence to Daniel S. Goldman, MD, University of Florida Health Science Center, 655 W 8th St, Jacksonville, FL 32209.
A36-year-old woman was
referred after her son had syncopal episodes associated with a
markedly prolonged QT interval, which was treated with ß-blockers and
a subsequent pacemaker. The mother had a history of
"seizures" as a child that had been treated with various
medications, which did not appear to affect her events. She continued
to have episodes 2 to 3 times a year, and witnesses said she would
"stiffen up and gasp for air." She had had no syncope for many
years but had episodes of dizziness and near syncope when anxious or
under emotional stress. These episodes were not associated with
palpitations. Her ECG revealed a corrected QT interval of 496
(Bazzetts formula), and an echocardiogram was remarkable for moderate
mitral regurgitation without prolapse. Her QT interval
did not shorten during treadmill testing. During recovery, she
demonstrated biphasic inferolateral T-wave changes that were more
pronounced with hyperventilation. On the basis of her long QT interval,
she was placed on a ß-blocker, and in view of her near syncopal
symptoms, an event recorder was placed. Most of her strips were
unremarkable except for an occasional premature
ventricular contraction. On one occasion when she became
anxious and hyperventilated in a parking lot, she recorded the
strip seen in Figure 1
, showing marked
T-wave alternans. On the basis of data from the International Long QT
Registry,1 2 implantation of an implantable
cardioverter-defibrillator was recommended. This was done, and 8 months
later, while in a stressful situation (and on ß-blockers), the
patient had the episode of polymorphic ventricular
tachycardia shown in Figure 2
, which was associated with syncope and
required multiple shocks from her device.
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Footnotes
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Lukes Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Lukes Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1267, Houston, TX 77030.
References
1. Zareba W, Moss AJ, Le Cessie S, et al. T wave alternans in idiopathic long QT syndrome. J Am Coll Cardiol. 1994;23:15411546.[Abstract]
2. Zareba W, Moss AJ, Andrews ML, Robinson JL, and the International Long QT Registry Research Group. Visible T-wave alternans in carriers of LQT1, LQT2, LQT3 gene mutations. J Am Coll Cardiol. 2000;35(suppl A):160A. Abstract.
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