Evaluation of age at symptom onset, proband status, and sex as predictors of disease severity in pediatric catecholaminergic polymorphic ventricular tachycardia.
Heart Rhythm
; 18(11): 1825-1832, 2021 11.
Article
en En
| MEDLINE
| ID: mdl-34333088
ABSTRACT
BACKGROUND:
Children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for sudden death, and a risk stratification tool does not exist.OBJECTIVE:
The purpose of this study was to determine whether proband status, age at 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 at 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 female and 65 male; 106 probands and 27 relatives). Localization of RYR2 variants to hotspots differed based on proband status and age at 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 (interquartile range 3-11) after time of symptom onset. Proband status, rather than age at of symptom onset or sex, was an independent predictor of time to first cardiac event (P = .008; hazard ratio = 4.4). The 5-, 10- and 15-year event-free survival rates for probands were 77%, 56%, and 46%, respectively, and for relatives were 96%, 91%, and 86%, respectively. Event risk after diagnosis was 48% (32/67) in patients on ß-blocker or flecainide alone vs 10% (5/48) in patients on ß-blocker plus flecainide and/or left cardiac sympathetic denervation (P <.001).CONCLUSION:
Proband status, but not age at 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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Taquicardia Ventricular
Tipo de estudio:
Clinical_trials
/
Diagnostic_studies
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Etiology_studies
/
Prognostic_studies
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Risk_factors_studies
Límite:
Adolescent
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Child
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Female
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Humans
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Male
País/Región como asunto:
America do norte
Idioma:
En
Revista:
Heart Rhythm
Año:
2021
Tipo del documento:
Article
País de afiliación:
Canadá