Congenital long QT syndrome (LQTS) is an inherited disorder of myocardial repolarization
resulting from gene mutations in potassium and sodium ion channels.
1
The prenatal diagnosis of LQTS is often retrospective for several reasons. First,
fetal echocardiography identifies only the mechanical consequences of electrophysiologic
events,
2
and fetal electrocardiograms do not provide tracings of adequate quality for clinical
application. Second, mothers with the LQTS mutation whose offspring are at increased
risk for LQTS may have normal QTc intervals on the baseline electrocardiogram.
3
Finally, the typical presentation of fetal LQTS is asymptomatic and mild sinus bradycardia
at rates at or barely below normal.
4
,
5
,
6
,
7
,
8
A rare presentation is second-degree atrioventricular block and ventricular tachycardia
leading to congestive heart failure.
4
,
9
,
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,
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We describe the diagnosis and treatment of a hydropic fetus with sustained ventricular
tachycardia and atrioventricular block. LQTS was diagnosed by fetal magnetocardiography
based on QTc prolongation and Torsades de Pointes.To read this article in full you will need to make a payment
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References
- Molecular and cellular mechanisms of cardiac arrhythmias.Cell. 2001; 104: 569-580
- Recognition of fetal arrhythmias by echocardiography.J Clin Ultrasound. 1985; 13: 255-263
- The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome.N Engl J Med. 1992; 327: 846-852
- Prenatal findings with prolonged QT interval in the neonatal period.Heart. 1997; 77: 198-204
- Fetal sinus bradycardia and the long QT syndrome.Am J Obstet Gynecol. 2001; 185: 743-747
- Romano-Ward syndrome diagnosed as moderate fetal bradycardia. A case report.J Repro Med. 1995; 40: 725-728
- Prenatal diagnosis of long QT syndrome using fetal magnetocardiography.Prenat Diag. 1999; 19: 677-680
- Fetal presentation of congenital long QT syndrome.Pediatr Cardiol. 1999; 20: 441-444
- Fetal ventricular tachycardia in the long QT syndrome.Cardiol Young. 1998; 8: 119-122
- Fetus with long QT syndrome manifested by tachyarrhythmia.Prenat Diagn. 1999; 19: 990-992
- A variant of long QT syndrome manifested as fetal tachycardia associated with ventricular septal defect.Heart. 1999; 82: 386-388
- Early prenatal management of a fetal ventricular tachycardia treated in utero by amiodarone with long term follow-up.Prenat Diagn. 2000; 20: 449-452
- Quinidine-induced action potential prolongation, early afterdepolarizations, and triggered activity in canine Purkinje fibers. Effects of stimulation rate, potassium, and magnesium.Circulation. 1989; 79: 674-686
- Placental transfer of lidocaine hydrochloride after prolonged continuous maternal intravenous administration.Can J Anaesth. 1995; 42: 338-340
Article info
Publication history
Accepted:
February 12,
2003
Received in revised form:
February 12,
2003
Received:
November 20,
2002
Identification
Copyright
© 2003 Excerpta Medica Inc. Published by Elsevier Inc. All rights reserved.
