Abstract
Abstract 9842: Characterization of Young Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Based on Age at Presentation
Circulation (New York, N.Y.), Vol.140(Suppl_1 Suppl 1), pp.A9842-A9842
11/19/2019
DOI: 10.1161/circ.140.suppl_1.9842
Abstract
BackgroundCatecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare inherited arrhythmia disorder characterized by adrenergically induced ventricular tachycardia and sudden death. It is unknown whether age at presentation predicts disease course in children. We sought to characterize clinical course based on age of symptom onset.MethodsThis is a prospective cohort study of CPVT patients diagnosed <19 years old. Patients were divided into two groupsearly-onset patients (EOP), symptom onset <10 years, and adolescent-onset patients (AOP), symptom onset from 10-18 years inclusive. Cardiac events were arrhythmic syncope, appropriate ICD shock, cardiac arrest (CA) or death. Clinical status, therapy, genetics and outcomes were analyzed. Significance (p <0.05) was determined using Fisher’s exact and logrank tests.ResultsWe compared 64 EOP and 76 AOP. The median age at symptom onset for EOP and AOP was 6 (IQR4-8) years and 13 (IQR11-16) years, respectively. In both groups, syncope (n=85) was the most common manifestation of CPVT followed by CA (n=25). Convulsive syncope was more frequent among EOP (22%) than AOP (8%; p=0.03). ICDs were implanted in 23 EOP and 22 AOP14 (61%) were implanted for primary prevention in EOP versus 5 (23%) in AOP (p=0.02). Over a median follow-up of 7 years after symptom onset, 25 (53%) EOP and 21 (30%) AOP experienced cardiac events (p=0.01; Figure 1). Accordingly, 5 arrhythmic deaths occurred in EOP and 0 in AOP. In 70 trios, de novo variants were higher in EOP (69%) than AOP (29%; p=0.002). Of 54 RYR2 variants found in AOP, 23 (43%) clustered in the N-terminus compared to 5 (14%) in EOP (p=0.005).ConclusionAge at symptom onset in pediatric CPVT patients is a predictor of disease course and de novo RYR2 genotype. Early onset CPVT correlates with greater disease burden, highlighting the importance of early therapeutic optimization. Further studies are needed to understand contributors to age at presentation.
Details
- Title: Subtitle
- Abstract 9842: Characterization of Young Patients With Catecholaminergic Polymorphic Ventricular Tachycardia Based on Age at Presentation
- Creators
- Dania Kallas - Pediatrics, BC Children’s Hosp, Vancouver, CanadaSonia Franciosi - Pediatrics, BC Children’s Hosp, Vancouver, CanadaThomas Roston - Pediatrics, BC Children’s Hosp, Vancouver, CanadaKrystien Lieve - Amsterdam University Medical CentersSit Kwok - Paediatric Cardiology, Queen Mary Hosp, Hong Kong, Hong KongLinda Knight - Cardiology, Sibley Heart Cntr, Atlanda, GAPrince Kannankeril - Vanderbilt UniversityAllison Hill - Cardiology, Children’s Hosp Los Angeles, Los Angeles, CAAndrew Krahn - University of British ColumbiaMartin LaPage - University of Michigan–Ann ArborSusan Etheridge - University of UtahIan Law - University of IowaSeshadri Balaji - Oregon Health & Science UniversityChristopher Johnsrude - University of LouisvilleDavid Backhoff - University of GöttingenSvjetlana Tisma-Dupanovic - Sisters of Mercy Health SystemJoseph Atallah - Pediatrics, Stollery Children’s Hosp, Edmonton, CanadaJames Perry - Pediatrics, Rady Children’s Hosp, San Diego, CAAdam Kean - Indiana UniversityGeorgia Sarquella-Brugada - Cardiology, Hosp Sant Joan de Deu, Barcelona, SpainJoshua Kovach - Pediatrics, Children’s Hosp of Wisconsin, Milwaukee, WIAnna Kamp - Pediatrics, Nationwide Children’s Hosp, Columbus, OHPeter Aziz - Cleveland ClinicYung Lau - University of AlabamaJordana Kron - Virginia Commonwealth UniversityArthur Wilde - Amsterdam University Medical CentersShubhayan Sanatani - Pediatrics, BC Children’s Hosp, Vancouver, Canada
- Resource Type
- Abstract
- Publication Details
- Circulation (New York, N.Y.), Vol.140(Suppl_1 Suppl 1), pp.A9842-A9842
- Publisher
- by the American College of Cardiology Foundation and the American Heart Association, Inc
- DOI
- 10.1161/circ.140.suppl_1.9842
- ISSN
- 0009-7322
- eISSN
- 1524-4539
- Language
- English
- Date published
- 11/19/2019
- Academic Unit
- Stead Family Department of Pediatrics; Cardiology
- Record Identifier
- 9984354384002771
Metrics
9 Record Views