Title of article :
Mortality Risk of Untreated Myosin-Binding Protein C–Related Hypertrophic Cardiomyopathy: Insight Into the Natural History
Author/Authors :
Nannenberg، نويسنده , , Eline A. and Michels، نويسنده , , Michelle and Christiaans، نويسنده , , Imke and Majoor-Krakauer، نويسنده , , Danielle and Hoedemaekers، نويسنده , , Yvonne M. and van Tintelen، نويسنده , , J. Peter and Lombardi، نويسنده , , M. Paola and ten Cate، نويسنده , , Folkert J. and Schinkel، نويسنده , , Arend F.L. and Tijssen، نويسنده , , Jan G.P. and van La، نويسنده ,
Issue Information :
روزنامه با شماره پیاپی سال 2011
Pages :
9
From page :
2406
To page :
2414
Abstract :
Objectives al of this study was to assess the mortality of hypertrophic cardiomyopathy (HCM), partly in times when the disease was not elucidated and patients were untreated. ound feared for the risk of sudden cardiac death (SCD). Insight in the natural history of the disorder is needed to design proper screening strategies for families with HCM. s arge, 200-year multigenerational pedigrees (identified by using genealogical searches) and in 140 small (contemporary) pedigrees (first-degree relatives of the proband) with HCM caused by a truncating mutation in the myosin-binding protein C gene (n = 1,118), we determined all-cause mortality using the family tree mortality ratio method. The studyʹs main outcome measure was the standardized mortality ratio (SMR). s large pedigrees, overall mortality was not increased (SMR 0.86 [95% confidence interval (CI): 0.72 to 1.03]), but significant excess mortality occurred between 10 and 19 years (SMR 2.7 [95% CI: 1.2 to 5.2]). In the small families, the SMR was increased (SMR 3.2 [95% CI: 2.3 to 4.3]) and excess mortality was observed between 10 and 39 years (SMR 3.2 [95% CI: 2.3 to 4.3]) and 50 and 59 years (SMR 1.9 [95% CI: 1.4 to 2.5]). sions ntified specific age categories with increased mortality risks in HCM families. The small, referred pedigrees had higher mortality risks than the large 200-year multigenerational pedigrees. Our findings support the strategy of starting cardiological and genetic screening in the first-degree relatives of a proband from 10 years onward and including persons in the screening at least until the age of 60 years. Screening of more distant relatives is probably most efficient between 10 and 19 years.
Keywords :
mortality , Natural history , family tree mortality ratio method , hypertrophic cardiomyopathy , myosin-binding protein C gene
Journal title :
JACC (Journal of the American College of Cardiology)
Serial Year :
2011
Journal title :
JACC (Journal of the American College of Cardiology)
Record number :
1753296
Link To Document :
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