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Value of Genetic Testing for the Prediction of Long-Term Outcome in Patients with Hypertrophic Cardiomyopathy

Abstract Pathogenic gene mutations are found in about 50 % of hypertrophic cardiomyopathy (HC) patients. Previous studies have shown an association between sarcomere mutations and medium-term outcome. The association with long-term outcome has not been described. The aim of this cohort study was to...

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Published in:The American journal of cardiology 2016-09, Vol.118 (6), p.881-887
Main Authors: van Velzen, Hannah G., MD, Vriesendorp, Pieter A., MD, Oldenburg, Rogier A., MD, PhD, van Slegtenhorst, Marjon A., PhD, van der Velden, Jolanda, MD, PhD, Schinkel, Arend F.L., MD, PhD, Michels, Michelle, MD, PhD
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Language:English
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Summary:Abstract Pathogenic gene mutations are found in about 50 % of hypertrophic cardiomyopathy (HC) patients. Previous studies have shown an association between sarcomere mutations and medium-term outcome. The association with long-term outcome has not been described. The aim of this cohort study was to assess the long-term outcomes of genotype positive (G+) and genotype negative (G-) HC patients. The study population consisted of 626 HC patients (512 probands, and 114 relatives) who underwent phenotyping and genetic testing between 1985 and 2014. End points were: all-cause mortality, cardiovascular (CV) mortality, heart failure (HF) related mortality and sudden cardiac death/aborted sudden cardiac death (SCD/aborted SCD). Kaplan Meier and multivariate cox regression analyses were performed. A pathogenic mutation was detected in 327 (52%) patients. G+ probands were younger than G- probands (46±15 vs 55±15 years, p
ISSN:0002-9149
1879-1913
DOI:10.1016/j.amjcard.2016.06.038