An Evaluation of Age at Symptom-Onset, Proband Status and Sex as Predictors of Disease Severity in Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F21%3A10429947" target="_blank" >RIV/00064203:_____/21:10429947 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11130/21:10429947
Result on the web
<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=usXf1Gpj-j" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=usXf1Gpj-j</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1016/j.hrthm.2021.07.061" target="_blank" >10.1016/j.hrthm.2021.07.061</a>
Alternative languages
Result language
angličtina
Original language name
An Evaluation of Age at Symptom-Onset, Proband Status and Sex as Predictors of Disease Severity in Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia
Original language description
BACKGROUND: Children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for sudden death and a risk stratification tool does not exist. OBJECTIVE: To determine whether proband status, age of symptom-onset and/or sex are independent predictors of cardiac events. METHODS: A multicenter, ambispective, cohort of pediatric CPVT patients was categorized by sex, proband status and age of symptom-onset (D1-first decade of life [symptom-onset <10 years] or D2-second decade of life [symptom-onset 10-18 years inclusive]). Demographics, therapy, genetics and outcomes were compared between groups. RESULTS: A total of 133 patients were included and stratified into 58 D1- and 75 D2-patients, 68 females and 65 males, and 106 probands and 27 relatives. Localization of RYR2 variants to hotspots differed based on proband status and age of symptom-onset. The cardiac event rate was 33% (n=44/133), inclusive of a 3% (n=4/133) mortality rate, over a median of 6-years (IQR:3-11) after time of symptom-onset. Proband status, rather than age of symptom-onset or sex, was an independent predictor of time to first cardiac event (p=0.008, HR=4.4). The 5-, 10- and 15- year event-free survival rate for probands were 77%, 56%, 46% and for relatives were 96%, 91%, 86%, respectively. Event-risk after diagnosis was 48% (n=32/67) in patients on β-blocker or flecainide alone versus 10% (n=5/48) on β-blocker plus flecainide and/or left cardiac sympathetic denervation (p<0.001). CONCLUSION: Proband status, but not age of symptom-onset or male sex, independently predicted an earlier-onset of cardiac events. A larger sample size would enable a comprehensive investigation of other risk factors.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30201 - Cardiac and Cardiovascular systems
Result continuities
Project
—
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2021
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Heart Rhythm
ISSN
1547-5271
e-ISSN
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Volume of the periodical
18
Issue of the periodical within the volume
11
Country of publishing house
US - UNITED STATES
Number of pages
8
Pages from-to
1825-1832
UT code for WoS article
000729403800006
EID of the result in the Scopus database
2-s2.0-85113307351