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From the Cardiology Unit, Department of Medicine (E.J.R., W.Z., A.J.M.),
the Department of Biostatistics (W.J.H.), and the Department of Community and
Preventive Medicine (J.L.R., M.A.), University of Rochester (NY) School of
Medicine and Dentistry; the Institute of Clinical Medicine, University of
Milan, Italy (E.H.L.); and the Department of Cardiology, University of Pavia
and Policlinico S. Matteo IRCCS, Pavia, Italy (P.J.S.).
Correspondence to Wojciech Zareba, MD, PhD, Heart Research Follow-up Program, Box 653, University of Rochester Medical Center, Rochester, NY 14642. E-mail Heartwz{at}heart.rochester.edu
Background—The effects of pregnancy
on women with the hereditary long QT syndrome are currently unknown.
The appropriate medical management of pregnant patients with the long
QT syndrome has not been established.
Methods and Results—The study was a retrospective
analysis of the 422 women (111 probands affected with the long
QT syndrome and 311 first-degree relatives) enrolled in the long QT
syndrome registry who had one or more pregnancies. The first-degree
relatives were classified as affected (QTc >0.47), borderline
(QTc=0.45 to 0.47), and unaffected (QTc <0.45). Cardiac events were
defined as the combined incidence of long QT syndrome–related death,
aborted cardiac arrest, and syncope. The incidence of cardiac events
was compared during equal prepregnancy, pregnancy, and postpartum
intervals (40 weeks each). Multivariate logistic
regression analysis was performed by use of a mixed-effects
model to identify independent predictors of cardiac events among
probands. The pregnancy and postpartum intervals were not associated
with cardiac events among first-degree relatives. The postpartum
interval was independently associated with cardiac events among
probands (odds ratio [OR], 40.8; 95% confidence interval [CI], 3.1
to 540; P=.01); the pregnancy interval was not
associated with cardiac events. Treatment with ß-adrenergic blockers
was independently associated with a decrease in the risk for cardiac
events among probands (OR, 0.023; 95% CI, 0.001 to 0.44;
P=.01).
Conclusions—The postpartum interval is associated with a
significant increase in risk for cardiac events among probands with the
long QT syndrome but not among first-degree relatives.
Prophylactic treatment with ß-adrenergic blockers should
be continued during the pregnancy and postpartum intervals in probands
with the long QT syndrome.
© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Influence of Pregnancy on the Risk for Cardiac Events in Patients With Hereditary Long QT Syndrome
Key Words: pregnancy • long QT syndrome • cardiovascular diseases
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