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The evaluation and management of decompensated hypertrophic cardiomyopathy in the emergency department

Published:September 03, 2018DOI:https://doi.org/10.1016/j.ajem.2018.09.003
      Hypertrophic cardiomyopathy (HCM) is a cardiac muscle disorder caused by autosomal dominant mutations of 11 or more genes encoding for cardiac sarcomeric proteins [
      • Nishimura R.A.
      • Holmes Jr., D.R.
      Hypertrophic obstructive cardiomyopathy.
      ,
      • Maron B.J.
      • Maron M.S.
      • Semsarian C.
      Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives.
      ,
      • 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.
      ]. The structural consequence of these mutations is formation of asymmetrical septal wall hypertrophy without ventricle dilation [
      • Popjes E.D.
      • St John Sutton M.
      Hypertrophic cardiomyopathy. Pathophysiology, diagnosis and treatment.
      ]. According to a study conducted by Maron et al. (1995), 1 in 500 adults in the United States have these mutations [
      • Maron B.J.
      Hypertrophic cardiomyopathy: an important global disease.
      ,
      • 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.
      ]. A majority of these cases are diagnosed by providers in larger metropolitan healthcare facilities [
      • 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.
      ,
      • Maron B.J.
      • Spirito P.
      Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history.
      ,
      • Spirito P.
      • Chiarella F.
      • Carratino L.
      • Berisso M.Z.
      • Bellotti P.
      • Vecchio C.
      Clinical course and prognosis of hypertrophic cardiomyopathy in an outpatient population.
      ]. Less frequently is it seen among older patient populations in community hospital emergency department (ED) settings [
      • Maron B.J.
      • Spirito P.
      Impact of patient selection biases on the perception of hypertrophic cardiomyopathy and its natural history.
      ]. 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 [
      • 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.
      ].

      Keywords

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