Case Report
Brugada Syndrome and Pregnancy: Highlights on
Antenatal and Prenatal Management
Laura Giambanco, Domenico Incandela, Antonio Maiorana, Walter Alio, and Luigi Alio
Obstetric and Gynecology Department, Civico Hospital, Piazza N. Leotta 4, 90100 Palermo, Italy
Correspondence should be addressed to Domenico Incandela; domenicoincandela@hotmail.it
Received 28 November 2013; Accepted 12 February 2014; Published 22 May 2014
Academic Editors: R. P. Kaufman, M. G. Porpora, and S. Salhan
Copyright © 2014 Laura Giambanco et al. Tis is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Introduction. Brugada syndrome is characterized by a disruption of heart’s normal rhythm. It is an autosomal dominant disease
due to a mutation of SNC5A gene. Its prevalence is low all over the world, but it is a lethal disease. Sudden cardiac death is the
result of phenotypic manifestation of Brugada syndrome. Among asymptomatic Brugada patients, arrhythmia could be provoked
by physical activity, fever, or pregnancy. About obstetrical management, very few data or reports have been published since this
syndrome has been diagnosed in late 1992. Case Presentation. A 20-year-old pregnant woman at 13 weeks of gestation was referred
to our department because of her familial history of sudden cardiac deaths. Brothers and sisters of her mother died of Brugada
syndrome in childhood or older and live components of this family were carrier of mutation in Brugada gene. Te pregnancy was
uneventful. Te patient gave birth vaginally without any arrhythmia. Strictly cardiological monitoring was performed during labour,
delivery, and 12 hours of the postpartum. Conclusion. Even though patient at low risk may never have arrhythmia, some conditions
could represent a Brugada trigger. Te management could be very easy and uneventful. Otherwise it could be very difcult with
need of ECMO or antiarrhythmics drugs or intracardiac device. Obstetrical management of Brugada pregnant women should be
very strict and multidisciplinary in cooperation with cardiologist and anaesthesiologist and should provide an informed consent
to the couple.
1. Background
Brugada syndrome is characterized by dysfunction of heart’s
normal rhythm. Tis disorder could lead to uncoordinated
ventricular electrical activity (ventricular arrhythmia, syn-
cope, sudden cardiac death). Symptomatic patients have
typical ECG signs: ST segment elevation in right precordial
without demonstrable structural heart disease [1, 2]. Brugada
syndrome is an autosomal dominant disease.
Te frst genic mutation was recognized in SNC5A, gene
involved in functioning sodium channels [3]. Indeed only
15%–30% of Brugada families have this mutation. Further
sodium/calcium channel mutations have been identifed in
some genes: SNC1B, CACNA1C, CACNB2, and GDP1L [3].
Patients with Brugada syndrome have normal heart function
but are at risk of cardiac arrhythmia; thus, the syndrome is
one of the leading causes of death for young men in Southeast
Asia [4]. Te only efective prevention of sudden death is
implantable cardioverter defbrillators (ICD) [3, 4], but it is
not still clear which patients should be treated.
Te sex-related diference in the phenotypic expression of
the Brugada syndrome is more pronounced than in any other
autosomally transmitted arrhythmic syndrome. Te basis for
this intriguing sex-related distinction is not fully understood.
Potential explanations are gender-related intrinsic difer-
ences in ionic currents and hormonal infuences. Due to this
hormonal infuence, pregnancy represents a particular situa-
tion in the life of women with BS. To date, data elucidating
the role of hormonal changes secondary to pregnancy in the
clinical outcome of this population have been missing [5].
It is possible to subdivide patients as symptomatic,
asymptomatic, positive, or negative to drugs test and member
of symptomatic or asymptomatic family. Te management,
the risk stratifcation, and the quality of life are variable for
each category. Nowadays Brugada syndrome is endemic in
Southeast Asia and is increasing in Europe and in USA, but
Hindawi Publishing Corporation
Case Reports in Obstetrics and Gynecology
Volume 2014, Article ID 531648, 3 pages
http://dx.doi.org/10.1155/2014/531648