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

  • Myocardial Disease (M Patel, Section Editor)
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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.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 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.

    Article  PubMed  Google Scholar 

  2. 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.

    Article  CAS  PubMed  Google Scholar 

  3. Maron BJ, Maron MS, Semsarian C. Genetics of hypertrophic cardiomyopathy after 20 years: clinical perspectives. J Am Coll Cardiol. 2012;60(8):705–15.

    Article  PubMed  Google Scholar 

  4. 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.

    Article  PubMed  Google Scholar 

  5. 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.

    Google Scholar 

  6. 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.

    Article  PubMed  Google Scholar 

  7. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  8. 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.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Bogaert K, Olivotto I. MR Imaging in hypertrophic cardiomyopathy: from magnet to bedside. Radiology. 2014;273(2):329–48.

    Article  PubMed  Google Scholar 

  10. 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.

    Article  Google Scholar 

  11. 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.

    Article  PubMed  Google Scholar 

  12. 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.

    Article  Google Scholar 

  13. 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.

    Article  CAS  Google Scholar 

  14. 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.

    Article  PubMed  Google Scholar 

  15. 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.

    Article  PubMed  Google Scholar 

  16. 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.

    Article  PubMed  Google Scholar 

  17. 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.

    Article  Google Scholar 

  18. 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.

    Article  PubMed  Google Scholar 

  19. 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.

    Article  Google Scholar 

  20. 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.

    Article  CAS  PubMed  Google Scholar 

  21. 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.

    Article  PubMed  Google Scholar 

  22. 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.

    Article  PubMed  Google Scholar 

  23. 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.

    Article  PubMed  Google Scholar 

  24. 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.

    Article  PubMed  Google Scholar 

  25. 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.

    Article  Google Scholar 

  26. Sorajja P, Ommen SR, Holmes DR, Dearani JA, et al. Survival after alcohol septal ablation for obstructive hypertrophic cardiomyopathy. Circulation. 2012;126:2374–80.

    Article  PubMed  Google Scholar 

  27. 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.

    Article  Google Scholar 

  28. 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.

    Article  CAS  PubMed  Google Scholar 

  29. 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.

    Article  PubMed  Google Scholar 

  30. 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.

    Article  Google Scholar 

  31. 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.

    CAS  PubMed  Google Scholar 

  32. 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.

    Article  CAS  PubMed  Google Scholar 

  33. 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.

    Article  PubMed  Google Scholar 

  34. 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.

    Article  PubMed  Google Scholar 

  35. 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.

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Correspondence to Andrew Wang.

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Ariane Fraiche declares that he has no conflict of interest.

Andrew Wang reports grants from Gilead Science, grants and personal fees from Myokardia Inc, and personal fees from Heart Metabolics.

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This article does not contain any studies with human participants or animals performed by any of the authors.

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This article is part of the Topical Collection on Myocardial Disease

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Fraiche, A., Wang, A. Hypertrophic Cardiomyopathy: New Evidence Since the 2011 American Cardiology of Cardiology Foundation and American Heart Association Guideline. Curr Cardiol Rep 18, 70 (2016). https://doi.org/10.1007/s11886-016-0751-8

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  • DOI: https://doi.org/10.1007/s11886-016-0751-8

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