Format

Send to

Choose Destination
Heart Rhythm. 2016 Jun;13(6):1274-82. doi: 10.1016/j.hrthm.2016.02.013. Epub 2016 Feb 24.

Impact of clinical and genetic findings on the management of young patients with Brugada syndrome.

Author information

1
CHU Nantes, Institut du Thorax, Nantes, France. Electronic address: antoine.andorin@gmail.com.
2
Saint George's University of London, London, United Kingdom.
3
AP-HP, Hôpital Bichat, Paris, France.
4
Center for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico Italiano, Milan, Italy; Department of Molecular Medicine, University of Pavia Pavia, Italy.
5
Center for Cardiac Arrhythmias of Genetic Origin, IRCCS Istituto Auxologico Italiano, Milan, Italy.
6
CHU Strasbourg, Strasbourg, France.
7
CHU Bordeaux, Hôpital Cardiologique du Haut Lévêque, Bordeaux, France.
8
CHR-GHSR La Réunion, Saint Pierre, France.
9
CHU Rennes, Hôpital Pontchaillou, Rennes, France.
10
AP-HP, Hôpital Necker-Enfants Malades, Paris, France.
11
CHU Poitiers, Poitiers, France.
12
CHU Dijon, Dijon, France.
13
Hôpital des Enfants, Toulouse, France.
14
CHU Nantes, Institut du Thorax, Nantes, France.
15
CHU Tours, Hôpital Trousseau, Tours, France.
16
Department of Pediatric Cardiology, Leiden University Medical Centre, Leiden, The Netherlands; Department of Pediatric Cardiology and.
17
Department of Clinical and Experimental Cardiology, Academic Medical Centre, Heart Centre, University of Amsterdam, Amsterdam, The Netherlands,; Princess Al-Jawhara Al-Brahim Centre of Excellence in Research of Hereditary Disorders, Jeddah, Kingdom of Saudi Arabia.

Abstract

BACKGROUND:

Brugada syndrome (BrS) is an arrhythmogenic disease associated with sudden cardiac death (SCD) that seldom manifests or is recognized in childhood.

OBJECTIVES:

The objectives of this study were to describe the clinical presentation of pediatric BrS to identify prognostic factors for risk stratification and to propose a data-based approach management.

METHODS:

We studied 106 patients younger than 19 years at diagnosis of BrS enrolled from 16 European hospitals.

RESULTS:

At diagnosis, BrS was spontaneous (n = 36, 34%) or drug-induced (n = 70, 66%). The mean age was 11.1 ± 5.7 years, and most patients were asymptomatic (family screening, (n = 67, 63%; incidental, n = 13, 12%), while 15 (14%) experienced syncope, 6(6%) aborted SCD or symptomatic ventricular tachycardia, and 5 (5%) other symptoms. During follow-up (median 54 months), 10 (9%) patients had life-threatening arrhythmias (LTA), including 3 (3%) deaths. Six (6%) experienced syncope and 4 (4%) supraventricular tachycardia. Fever triggered 27% of LTA events. An implantable cardioverter-defibrillator was implanted in 22 (21%), with major adverse events in 41%. Of the 11 (10%) patients treated with hydroquinidine, 8 remained asymptomatic. Genetic testing was performed in 75 (71%) patients, and SCN5A rare variants were identified in 58 (55%); 15 of 32 tested probands (47%) were genotype positive. Nine of 10 patients with LTA underwent genetic testing, and all were genotype positive, whereas the 17 SCN5A-negative patients remained asymptomatic. Spontaneous Brugada type 1 electrocardiographic (ECG) pattern (P = .005) and symptoms at diagnosis (P = .001) were predictors of LTA. Time to the first LTA event was shorter in patients with both symptoms at diagnosis and spontaneous Brugada type 1 ECG pattern (P = .006).

CONCLUSION:

Spontaneous Brugada type 1 ECG pattern and symptoms at diagnosis are predictors of LTA events in the young affected by BrS. The management of BrS should become age-specific, and prevention of SCD may involve genetic testing and aggressive use of antipyretics and quinidine, with risk-specific consideration for the implantable cardioverter-defibrillator.

KEYWORDS:

Arrhythmia; Brugada syndrome; Genetics; Pediatrics; Quinidine

PMID:
26921764
DOI:
10.1016/j.hrthm.2016.02.013
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center