Hypertrophic cardiomyopathy (HCM) is a cardiac muscle disorder caused by autosomal
dominant mutations of 11 or more genes encoding for cardiac sarcomeric proteins [
1
,
2
,
3
]. The structural consequence of these mutations is formation of asymmetrical septal
wall hypertrophy without ventricle dilation [
- Olivotto I.
- Hellawell J.L.
- Farzaneh-Far R.
- Blair C.
- Coppini R.
- Myers J.
- et al.
Novel approach targeting the complex pathophysiology of hypertrophic cardiomyopathy:
the impact of late sodium current inhibition on exercise capacity in subjects with
symptomatic hypertrophic cardiomyopathy (LIBERTY-HCM) trial.
Circ Heart Fail. 2016; 9e002764
[4]
]. According to a study conducted by Maron et al. (1995), 1 in 500 adults in the United
States have these mutations [
[5]
,
[6]
]. A majority of these cases are diagnosed by providers in larger metropolitan healthcare
facilities [
- Maron B.J.
- Gardin J.M.
- Flack J.M.
- Gidding S.S.
- Kurosaki T.T.
- Bild D.E.
Prevalence of hypertrophic cardiomyopathy in a general population of young adults:
echocardiographic analysis of 4111 subjects in the CARDIA Study Coronary Artery Risk
Development in (young) adults.
Circulation. 1995; 92: 785-789
[3]
,
- Olivotto I.
- Hellawell J.L.
- Farzaneh-Far R.
- Blair C.
- Coppini R.
- Myers J.
- et al.
Novel approach targeting the complex pathophysiology of hypertrophic cardiomyopathy:
the impact of late sodium current inhibition on exercise capacity in subjects with
symptomatic hypertrophic cardiomyopathy (LIBERTY-HCM) trial.
Circ Heart Fail. 2016; 9e002764
[7]
,
[8]
]. Less frequently is it seen among older patient populations in community hospital
emergency department (ED) settings [
[7]
]. The National Heart, Lung, and Blood Institute (NHLBI) has deemed HCM as an unmet-need
disease state due to a marked lack of original randomized controlled trials (RCTs)
examining the pharmacological agents used for acute and chronic management of HCM
[
[3]
].- Olivotto I.
- Hellawell J.L.
- Farzaneh-Far R.
- Blair C.
- Coppini R.
- Myers J.
- et al.
Novel approach targeting the complex pathophysiology of hypertrophic cardiomyopathy:
the impact of late sodium current inhibition on exercise capacity in subjects with
symptomatic hypertrophic cardiomyopathy (LIBERTY-HCM) trial.
Circ Heart Fail. 2016; 9e002764
Keywords
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Article Info
Publication History
Published online: September 03, 2018
Accepted:
September 1,
2018
Received in revised form:
August 27,
2018
Received:
April 18,
2018
Identification
Copyright
© 2018 Elsevier Inc. All rights reserved.
