Diagnosis of Brugada Syndrome and RV Dysplasia in Children PDF
Preview Diagnosis of Brugada Syndrome and RV Dysplasia in Children
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
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