Family screening for hypertrophic cardiomyopathy: Is it time to change practice guidelines?

Abstract Aims Current guidelines recommend initiating family screening for hypertrophic cardiomyopathy (HCM) after age 10 or 12 years unless early screening criteria are met. The aim was to evaluate if current screening guidelines miss early onset disease. Methods and results Children who underwent...

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Bibliographic Details
Published in:European heart journal 2019-12, Vol.40 (45), p.3672-3681
Main Authors: Lafreniere-Roula, Myriam, Bolkier, Yoav, Zahavich, Laura, Mathew, Jacob, George, Kristen, Wilson, Judith, Stephenson, Elizabeth A, Benson, Leland N, Manlhiot, Cedric, Mital, Seema
Format: Article
Language:English
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Fast Track Clinical Research
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Summary:Abstract Aims Current guidelines recommend initiating family screening for hypertrophic cardiomyopathy (HCM) after age 10 or 12 years unless early screening criteria are met. The aim was to evaluate if current screening guidelines miss early onset disease. Methods and results Children who underwent family screening for HCM before age 18 years were analysed. Major cardiac events (MaCEs) were defined as death, sudden cardiac death (SCD), or need for major cardiac interventions (myectomy, implantable cardioverter-defibrillator insertion, transplantation). Of 524 children screened, 331 were under 10 years of age, 9.9% had echocardiographic evidence of HCM, and 1.1% were symptomatic at first screening. The median (interquartile range) age at HCM onset was 8.9 (4.7–13.4) years, and at MaCE was 10.9 (8.5–14.3) years with a median time to MaCE from HCM onset of 1.5 (0.5–4.1) years. About 52.5% phenotype-positive children and 41% with MaCEs were
ISSN:0195-668X
1522-9645
DOI:10.1093/eurheartj/ehz396