Diagnosis of Brugada Syndrome and RV Dysplasia in Children

41 Pages·2015·1.33 MB·English

Pediatric Patients with Inherited
Arrhythmia Syndromes
Akash Patel, MD
Pediatric Electrophysiologist
’
UCSF Benioff Children s Hospital
Assistant Professor of Pediatrics
University of California San Francisco
Slides courtesy of Dr. Ronn Tanel
’
What s so different about
pediatric patients?

Diagnosis

Treatment

Lifestyle

’
What s so different about
pediatric patients?

Diagnosis

Treatment

Lifestyle

Familial Screening

Identification of children or

siblings after an affected family
member is diagnosed
Genotype + Phenotype + or -

Age at Presentation for Inherited
Arrhythmia Syndromes
LQTS
BrS
ARVC
CPVT
0 10 20 30 40
Age
Probability of Cardiac Event in LQTS
Circulation, 1991
For some inherited arrhythmias, an early age at onset of
symptoms is a predictor of future sudden cardiac events
Cardiac Events in LQTS
Goldenberg and Moss, JACC 2008
Sudden Infant Death Syndrome
(SIDS)

SIDS is the sudden death of an infant under
one year of age which remains unexplained
after a thorough case investigation,
including performance of a complete
autopsy, examination of the death scene,
and review of the clinical history.

Willinger et al, 1991
Sudden Infant Death Syndrome
(SIDS)
Does LQTS cause SIDS ?
Genetic testing suggests that

LQTS and other inherited
arrhythmias may cause 5% -
10% of SIDS deaths
ECG screening of parents of

SIDS victims suggests that the
incidence may be higher
Some data regarding SIDS speaks against LQTS

“
playing a significant role, such as the Back to
”
Sleep program