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Published in: Current Cardiology Reports 8/2016

01-08-2016 | Myocardial Disease (M Patel, Section Editor)

Hypertrophic Cardiomyopathy: New Evidence Since the 2011 American Cardiology of Cardiology Foundation and American Heart Association Guideline

Authors: Ariane Fraiche, Andrew Wang

Published in: Current Cardiology Reports | Issue 8/2016

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Abstract

Since publication of the 2011 American College of Cardiology Foundation (ACCF) and American Heart Association (AHA) Guideline for the diagnosis and treatment of hypertrophic cardiomyopathy (HCM), more recent studies offer greater insights about this condition. With increased recognition of the role of sarcomere protein mutations and myocardial structural abnormalities in the pathophysiology of this disease, new evidence offers potential improvements for the management of patients with HCM. In this review of studies published since 2011, we highlight several studies that may impact diagnostic considerations, risk stratification, and treatment of symptoms in HCM.
Literature
1.
go back to reference Gersh BJ, Maron BJ, Bonow RO, Dearani JA, et al. American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American Society of Echocardiography; American Society of Nuclear Cardiology; Heart Failure Society of America; Heart Rhythm Society; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011;124(24):2761–96.CrossRefPubMed Gersh BJ, Maron BJ, Bonow RO, Dearani JA, et al. American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines; American Association for Thoracic Surgery; American Society of Echocardiography; American Society of Nuclear Cardiology; Heart Failure Society of America; Heart Rhythm Society; Society for Cardiovascular Angiography and Interventions; Society of Thoracic Surgeons. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation. 2011;124(24):2761–96.CrossRefPubMed
2.
go back to reference Maron BJ, Gardin JM, Flack JM, et al. 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;92:785–9.CrossRefPubMed Maron BJ, Gardin JM, Flack JM, et al. 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;92:785–9.CrossRefPubMed
3.
go back to reference Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol. 2012;60(8):705–15.CrossRefPubMed Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol. 2012;60(8):705–15.CrossRefPubMed
4.
go back to reference Semsarian C, Ingles J, Maron M, Maron B. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015;65(12):1249–54.CrossRefPubMed Semsarian C, Ingles J, Maron M, Maron B. New perspectives on the prevalence of hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015;65(12):1249–54.CrossRefPubMed
5.
go back to reference Ho CY, Lakdawala NK, Cirino AL, Lipshultz SE, et al. Diltiazem treatment for pre-clinical hypertrophic cardiomyopathy sarcomere mutation carriers: a pilot randomized trial to modify disease expression. J Am Coll Cardiol Heart Fail. 2015;3(2):180–8. Ho CY, Lakdawala NK, Cirino AL, Lipshultz SE, et al. Diltiazem treatment for pre-clinical hypertrophic cardiomyopathy sarcomere mutation carriers: a pilot randomized trial to modify disease expression. J Am Coll Cardiol Heart Fail. 2015;3(2):180–8.
6.
go back to reference Maron BJ, Ommen SR, Semsarian C, Spirito P, et al. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. 2014;64(1):83–99.CrossRefPubMed Maron BJ, Ommen SR, Semsarian C, Spirito P, et al. Hypertrophic cardiomyopathy: present and future, with translation into contemporary cardiovascular medicine. J Am Coll Cardiol. 2014;64(1):83–99.CrossRefPubMed
7.
go back to reference Captur G, Lopes LR, Mohun TJ, Patel V, et al. Prediction of sarcomere mutations in subclinical hypertrophic cardiomyopathy. Circ Cardiovasc Imaging. 2014;7:863–71.CrossRefPubMedPubMedCentral Captur G, Lopes LR, Mohun TJ, Patel V, et al. Prediction of sarcomere mutations in subclinical hypertrophic cardiomyopathy. Circ Cardiovasc Imaging. 2014;7:863–71.CrossRefPubMedPubMedCentral
8.
go back to reference Ho CY, Abbasi SA, Neilan TG, et al. T1 measurements identify extracellular volume expansion in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. Circ Cardiovasc Imaging. 2013;6(3):415–22.CrossRefPubMedPubMedCentral Ho CY, Abbasi SA, Neilan TG, et al. T1 measurements identify extracellular volume expansion in hypertrophic cardiomyopathy sarcomere mutation carriers with and without left ventricular hypertrophy. Circ Cardiovasc Imaging. 2013;6(3):415–22.CrossRefPubMedPubMedCentral
9.
go back to reference Bogaert K, Olivotto I. MR Imaging in hypertrophic cardiomyopathy: from magnet to bedside. Radiology. 2014;273(2):329–48.CrossRefPubMed Bogaert K, Olivotto I. MR Imaging in hypertrophic cardiomyopathy: from magnet to bedside. Radiology. 2014;273(2):329–48.CrossRefPubMed
10.
go back to reference Fontana M, Banypersad SM, Treibel TA, Maestrini V, et al. Native T1 mapping in transthyretin amyloidosis. J Am Coll Cardiol Cardiovasc Imaging. 2014;7(2):157–65.CrossRef Fontana M, Banypersad SM, Treibel TA, Maestrini V, et al. Native T1 mapping in transthyretin amyloidosis. J Am Coll Cardiol Cardiovasc Imaging. 2014;7(2):157–65.CrossRef
11.
go back to reference Sado DM, White SK, Piechnik SK, Banypersad SM, et al. Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping. Circ Cardiovasc Imaging. 2013;6(3):392–8.CrossRefPubMed Sado DM, White SK, Piechnik SK, Banypersad SM, et al. Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping. Circ Cardiovasc Imaging. 2013;6(3):392–8.CrossRefPubMed
12.
go back to reference Authors/Task Force members, Elliott PM, Anastasakis A, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733.CrossRef Authors/Task Force members, Elliott PM, Anastasakis A, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014;35:2733.CrossRef
13.
go back to reference Maron BJ, Casey SA, Poliac LC, Gohman TE, et al. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. J Am Med Assoc. 1999;281(7):650–5.CrossRef Maron BJ, Casey SA, Poliac LC, Gohman TE, et al. Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. J Am Med Assoc. 1999;281(7):650–5.CrossRef
14.
go back to reference Maron BJ, Rowin EJ, Casey SA, Link MS, et al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol. 2015;65(18):1915–28.CrossRefPubMed Maron BJ, Rowin EJ, Casey SA, Link MS, et al. Hypertrophic cardiomyopathy in adulthood associated with low cardiovascular mortality with contemporary management strategies. J Am Coll Cardiol. 2015;65(18):1915–28.CrossRefPubMed
15.
go back to reference Maron BJ, Casey SA, Chan RH, Garberich RF, et al. Independent assessment of the European Society of Cardiology sudden death risk model for hypertrophic cardiomyopathy. Am J Cardiol. 2015;116:757–64.CrossRefPubMed Maron BJ, Casey SA, Chan RH, Garberich RF, et al. Independent assessment of the European Society of Cardiology sudden death risk model for hypertrophic cardiomyopathy. Am J Cardiol. 2015;116:757–64.CrossRefPubMed
16.
go back to reference Masri A, Pierson LM, Smedira NG, Agarwal S, et al. Predictors of long term outcomes in patient with hypertrophic cardiomyopathy undergoing cardiopulmonary stress testing and echocardiography. Am Heart J. 2015;169(5):684–92.CrossRefPubMed Masri A, Pierson LM, Smedira NG, Agarwal S, et al. Predictors of long term outcomes in patient with hypertrophic cardiomyopathy undergoing cardiopulmonary stress testing and echocardiography. Am Heart J. 2015;169(5):684–92.CrossRefPubMed
17.
go back to reference Desai MY, Bhonsale A, Patel P, Naji P, et al. Exercise echocardiography in asymptomatic HCM: exercise capacity, and not LV outflow tract predicts long-term outcomes. J Am Coll Cardiol Cardiovasc Imaging. 2014;7(1):26–36.CrossRef Desai MY, Bhonsale A, Patel P, Naji P, et al. Exercise echocardiography in asymptomatic HCM: exercise capacity, and not LV outflow tract predicts long-term outcomes. J Am Coll Cardiol Cardiovasc Imaging. 2014;7(1):26–36.CrossRef
18.
go back to reference Spirito P, Autore C, Formisano F, Assenza GE, et al. Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors. Am J Cardiol. 2014;113:1550–5.CrossRefPubMed Spirito P, Autore C, Formisano F, Assenza GE, et al. Risk of sudden death and outcome in patients with hypertrophic cardiomyopathy with benign presentation and without risk factors. Am J Cardiol. 2014;113:1550–5.CrossRefPubMed
19.
go back to reference Green JJ, Berger JS, Kramer CM, Salerno M. Prognostic value of late gadolinium enhancement in clinical outcomes for hypertrophic cardiomyopathy. J Am Coll Cardiol Cardiovasc Imaging. 2012;5(4):370–7.CrossRef Green JJ, Berger JS, Kramer CM, Salerno M. Prognostic value of late gadolinium enhancement in clinical outcomes for hypertrophic cardiomyopathy. J Am Coll Cardiol Cardiovasc Imaging. 2012;5(4):370–7.CrossRef
20.
go back to reference Briasoulis A, Mallikethi-Reddy S, Palla M, Alash I, et al. Myocardial fibrosis on cardiac magnetic resonance and cardiac outcomes in hypertrophic cardiomyopathy: a meta-analysis. Heart. 2015;101:1406–11.CrossRefPubMed Briasoulis A, Mallikethi-Reddy S, Palla M, Alash I, et al. Myocardial fibrosis on cardiac magnetic resonance and cardiac outcomes in hypertrophic cardiomyopathy: a meta-analysis. Heart. 2015;101:1406–11.CrossRefPubMed
21.•
go back to reference Chan RH, Maron BJ, Olivotto I, Pencina MJ. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation. 2014;130:484–95. This multicenter, observational HCM cohort study evaluated the prognostic significance of late gadolinium enhancement (LGE) by cardiac MRI and found that extent of LGE was associated with an increased risk of SCD events (LGE of ≥15% of LV mass demonstrated a 2-fold increase) in long term follow-up.CrossRefPubMed Chan RH, Maron BJ, Olivotto I, Pencina MJ. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation. 2014;130:484–95. This multicenter, observational HCM cohort study evaluated the prognostic significance of late gadolinium enhancement (LGE) by cardiac MRI and found that extent of LGE was associated with an increased risk of SCD events (LGE of ≥15% of LV mass demonstrated a 2-fold increase) in long term follow-up.CrossRefPubMed
22.
go back to reference Kramer CM, Appelbaum E, Desai MY, Desvigne-Nickens P, et al. Hypertrophic cardiomyopathy registry: the rationale and design of an international, observational study of hypertrophic cardiomyopathy. Am Heart J. 2015;170(2):223–30.CrossRefPubMed Kramer CM, Appelbaum E, Desai MY, Desvigne-Nickens P, et al. Hypertrophic cardiomyopathy registry: the rationale and design of an international, observational study of hypertrophic cardiomyopathy. Am Heart J. 2015;170(2):223–30.CrossRefPubMed
23.
go back to reference Maron MS, Rowin EJ, Olivotto I, Casey SA, et al. Contemporary natural history and management of nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2016;67(12):1399–409.CrossRefPubMed Maron MS, Rowin EJ, Olivotto I, Casey SA, et al. Contemporary natural history and management of nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2016;67(12):1399–409.CrossRefPubMed
24.
go back to reference Tower-Rader A, Furiasse N, Puthumana JJ, Kruse J, et al. Effects of septal myomectomy on left ventricular diastolic function and left atrial volume in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2014;114:1568–72.CrossRefPubMed Tower-Rader A, Furiasse N, Puthumana JJ, Kruse J, et al. Effects of septal myomectomy on left ventricular diastolic function and left atrial volume in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2014;114:1568–72.CrossRefPubMed
25.
go back to reference Viresendorp PA, Schinkel AFL, Soliman OII, Kofflard MJM, et al. Long-term benefit of myomectomy and anterior mitral leaflet extension in obstructive hypertrophic cardiomyopathy. Am J Cardiol. 2015;115:670–67.CrossRef Viresendorp PA, Schinkel AFL, Soliman OII, Kofflard MJM, et al. Long-term benefit of myomectomy and anterior mitral leaflet extension in obstructive hypertrophic cardiomyopathy. Am J Cardiol. 2015;115:670–67.CrossRef
26.
go back to reference Sorajja P, Ommen SR, Holmes DR, Dearani JA, et al. Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation. 2012;126:2374–80.CrossRefPubMed Sorajja P, Ommen SR, Holmes DR, Dearani JA, et al. Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation. 2012;126:2374–80.CrossRefPubMed
27.
go back to reference Liebregts M, Steggerda RC, Vriesendorp PA, van Velzen H, Schinkel AF, Willems R, et al. Long-term outcome of alcohol septal ablation for obstructive hypertrophic cardiomyopathy in the young and the elderly. J Am Coll Cardiol Cardiovasc Interv. 2016;9(5):463–9.CrossRef Liebregts M, Steggerda RC, Vriesendorp PA, van Velzen H, Schinkel AF, Willems R, et al. Long-term outcome of alcohol septal ablation for obstructive hypertrophic cardiomyopathy in the young and the elderly. J Am Coll Cardiol Cardiovasc Interv. 2016;9(5):463–9.CrossRef
28.•
go back to reference Coppini R, Ferrantini C, Yao L, Fan P, et al. Late sodium current inhibition reverses electromechanical dysfunction in human hypertrophic cardiomyopathy. Circulation. 2013;127:575–84. This study showed that ranolazine, a late sodium channel antagonist, partially reversed cellular abnormalities in HCM myocytes in vitro. These results are the basis for the ongoing multicenter, randomized, controlled trial of a novel, late sodium channel antagonist, eleclazine, in symptomatic HCM patients.CrossRefPubMed Coppini R, Ferrantini C, Yao L, Fan P, et al. Late sodium current inhibition reverses electromechanical dysfunction in human hypertrophic cardiomyopathy. Circulation. 2013;127:575–84. This study showed that ranolazine, a late sodium channel antagonist, partially reversed cellular abnormalities in HCM myocytes in vitro. These results are the basis for the ongoing multicenter, randomized, controlled trial of a novel, late sodium channel antagonist, eleclazine, in symptomatic HCM patients.CrossRefPubMed
29.
go back to reference Olivotto I, Hellawell JL, Farzaneh-Far R, Blair C. Approach targeting the complex pathophysiology of hypertrophic cardiomyopathy: impact of late sodium current inhibition on exercise capacity in subjects with symptomatic hypertrophic cardiomyopathy (LIBERTY-HCM) trial. Circ Heart Fail. 2016;9:e002764.CrossRefPubMed Olivotto I, Hellawell JL, Farzaneh-Far R, Blair C. Approach targeting the complex pathophysiology of hypertrophic cardiomyopathy: impact of late sodium current inhibition on exercise capacity in subjects with symptomatic hypertrophic cardiomyopathy (LIBERTY-HCM) trial. Circ Heart Fail. 2016;9:e002764.CrossRefPubMed
30.•
go back to reference Green EM, Wakimoto H, Anderson RL, Evanchik MJ, et al. A small-molecule inhibitor of sarcomere contractility suppresses hypertrophic cardiomyopathy in mice. Science. 2016;352:617–21. This study found that a novel agent that reduces contractility by decreasing the adenosine triphosphatase activity of the cardiac myosin heavy chain prevented the pathologic changes in the myocardium of mice with human HCM sarcomere mutation.CrossRef Green EM, Wakimoto H, Anderson RL, Evanchik MJ, et al. A small-molecule inhibitor of sarcomere contractility suppresses hypertrophic cardiomyopathy in mice. Science. 2016;352:617–21. This study found that a novel agent that reduces contractility by decreasing the adenosine triphosphatase activity of the cardiac myosin heavy chain prevented the pathologic changes in the myocardium of mice with human HCM sarcomere mutation.CrossRef
31.
go back to reference Coats CJ, Rantell K, Bartnik A, Patel A, et al. Cardiopulmonary exercise testing and prognosis in hypertrophic cardiomyopathy. Circ Heart Fail. 2015;8(6):1022–31.PubMed Coats CJ, Rantell K, Bartnik A, Patel A, et al. Cardiopulmonary exercise testing and prognosis in hypertrophic cardiomyopathy. Circ Heart Fail. 2015;8(6):1022–31.PubMed
32.
go back to reference Axelsson A, Iversen K, Veijlstrup N, Norsk J. Efficacy and safety of the angiotensin II receptor blocker losartan for hypertrophic cardiomyopathy: the INHERIT randomised, double-blind, placebo-controlled trial. Lancet Diabetes Endocrinol. 2015;3:123–31.CrossRefPubMed Axelsson A, Iversen K, Veijlstrup N, Norsk J. Efficacy and safety of the angiotensin II receptor blocker losartan for hypertrophic cardiomyopathy: the INHERIT randomised, double-blind, placebo-controlled trial. Lancet Diabetes Endocrinol. 2015;3:123–31.CrossRefPubMed
33.
go back to reference Santangeli P, Biase LD, Themistoclakis S, Raviele A, et al. Catheter ablation of atrial fibrillation in hypertrophic cardiomyopathy: long-term outcomes and mechanisms of arrhythmia recurrence. Circ Arrhythmia and Electrophysiology. 2013;6:1089–94.CrossRefPubMed Santangeli P, Biase LD, Themistoclakis S, Raviele A, et al. Catheter ablation of atrial fibrillation in hypertrophic cardiomyopathy: long-term outcomes and mechanisms of arrhythmia recurrence. Circ Arrhythmia and Electrophysiology. 2013;6:1089–94.CrossRefPubMed
34.
go back to reference Contreras-Valdes FM, Buxton AE, Josephson ME, Anter E. Atrial fibrillation ablation in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015;65(14):1485–7.CrossRefPubMed Contreras-Valdes FM, Buxton AE, Josephson ME, Anter E. Atrial fibrillation ablation in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2015;65(14):1485–7.CrossRefPubMed
35.
go back to reference Zhao DS, Shen Y, Zhang Q, Lin G, et al. Outcomes of catheter ablation of atrial fibrillation in patients with hypertrophic cardiomyopathy: a systematic review and meta-analysis. Europace. 2015. Zhao DS, Shen Y, Zhang Q, Lin G, et al. Outcomes of catheter ablation of atrial fibrillation in patients with hypertrophic cardiomyopathy: a systematic review and meta-analysis. Europace. 2015.
Metadata
Title
Hypertrophic Cardiomyopathy: New Evidence Since the 2011 American Cardiology of Cardiology Foundation and American Heart Association Guideline
Authors
Ariane Fraiche
Andrew Wang
Publication date
01-08-2016
Publisher
Springer US
Published in
Current Cardiology Reports / Issue 8/2016
Print ISSN: 1523-3782
Electronic ISSN: 1534-3170
DOI
https://doi.org/10.1007/s11886-016-0751-8

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