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Clinical and imaging description of the Maron subtypes of hypertrophic cardiomyopathy

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Abstract

Longitudinal strain analysis enables early detection of left ventricular (LV) contraction abnormalities in patients with preserved ejection fraction. Hypertrophic cardiomyopathy (HCM) is associated with low values of regional and global longitudinal myocardial deformations. In addition to contraction abnormalities, LV regional strain abnormalities are partially related to the degree of hypertrophy. This study sought to describe global longitudinal strain (GLS) in HCM patients as categorized using the Maron’s classification. Complete echocardiography examinations, including GLS analysis, were performed in consecutive HCM patients followed up in two French HCM-clinics. A total of 271 patients (mean age 49 ± 16yrs; 71 % male) were evaluated. In this population, the most frequently classified hypertrophy pattern was Type II (47 %), following the Maron’s classification. Type III was characterized by a higher degree of LV hypertrophy in terms of mass and maximal wall thickness, and was more frequently obstructive at rest, with lower GLS values (−15.3 ± 3.9 %, p = 0.016), higher E/E’ ratio (13.4 ± 6.7, p < 0.001), and a more frequently inadequate blood pressure response to exercise (30 %, p = 0.04) compared to other patterns. The variable that correlated best with GLS was LV mass index (r = 0.49, p < 0.01), while GLS did not significantly correlate with left ventricular outflow tract obstruction. This study demonstrated that the Type III HCM pattern presented with lower GLS, which was partially related to higher LV mass index, more elevated LV filling pressures, and a more frequently inadequate blood pressure response to exercise, in comparison with other patterns categorized using the Maron’s classification.

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Abbreviations

GLS:

Global longitudinal strain

HCM:

Hypertrophic cardiomyopathy

LV:

Left ventricle

LVEF:

Left ventricular ejection fraction

LVOTO:

Left ventricular outflow tract obstruction

References

  1. Maron MS, Olivotto I, Betocchi S, Casey SA, Lesser JR, Losi MA, Cecchi F, Maron BJ (2003) Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N Engl J Med 348:295–303

    Article  PubMed  Google Scholar 

  2. Ball W, Ivanov J, Rakowski H, Wigle ED, Linghorne M, Ralph-Edwards A, Williams WG, Schwartz L, Guttman A, Woo A (2011) Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment. J Am Coll Cardiol 58:2313–2321

    Article  PubMed  Google Scholar 

  3. Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ (2006) Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation 114:2232–2239

    Article  PubMed  Google Scholar 

  4. Vaglio JC Jr, Ommen SR, Nishimura RA, Tajik AJ, Gersh BJ (2008) Clinical characteristics and outcomes of patients with hypertrophic cardiomyopathy with latent obstruction. Am Heart J 156:342–347

    Article  PubMed  Google Scholar 

  5. Serri K, Reant P, Lafitte M, Berhouet M, Le Bouffos V, Roudaut R, Lafitte S (2006) Global and regional myocardial function quantification by two-dimensional strain: application in hypertrophic cardiomyopathy. J Am Coll Cardiol 47:1175–1181

    Article  PubMed  Google Scholar 

  6. Lafitte S, Perlant M, Reant P, Serri K, Douard H, DeMaria A, Roudaut R (2009) Impact of impaired myocardial deformations on exercise tolerance and prognosis in patients with asymptomatic aortic stenosis. Eur J Echocardiogr 10:414–419

    Article  PubMed  Google Scholar 

  7. Yingchoncharoen T, Gibby C, Rodriguez LL, Grimm RA, Marwick TH (2012) Association of myocardial deformation with outcome in asymptomatic aortic stenosis with normal ejection fraction. Circ Cardiovasc Imaging 5:719–725

    Article  PubMed  Google Scholar 

  8. Mignot A, Donal E, Zaroui A, Reant P, Salem A, Hamon C, Monzy S, Roudaut R, Habib G, Lafitte S (2010) Global longitudinal strain as a major predictor of cardiac events in patients with depressed left ventricular function: a multicenter study. J Am Soc Echocardiogr 23:1019–1024

    Article  PubMed  Google Scholar 

  9. Buss SJ, Emami M, Mereles D, Korosoglou G, Kristen AV, Voss A, Schellberg D, Zugck C, Galuschky C, Giannitsis E, Hegenbart U, Ho AD, Katus HA, Schonland SO, Hardt SE (2012) Longitudinal left ventricular function for prediction of survival in systemic light-chain amyloidosis: incremental value compared with clinical and biochemical markers. J Am Coll Cardiol 60:1067–1076

    Article  PubMed  Google Scholar 

  10. Liu YW, Su CT, Sung JM, Wang SP, Su YR, Yang CS, Tsai LM, Chen JH, Tsai WC (2013) Association of left ventricular longitudinal strain with mortality among stable hemodialysis patients with preserved left ventricular ejection fraction. Clin J Am Soc Nephrol 8:1564–1574

    Article  PubMed Central  PubMed  Google Scholar 

  11. Saito M, Okayama H, Yoshii T, Higashi H, Morioka H, Hiasa G, Sumimoto T, Inaba S, Nishimura K, Inoue K, Ogimoto A, Shigematsu Y, Hamada M, Higaki J (2012) Clinical significance of global two-dimensional strain as a surrogate parameter of myocardial fibrosis and cardiac events in patients with hypertrophic cardiomyopathy. Eur Heart J Cardiovasc Imaging 13:617–623

    Article  PubMed  Google Scholar 

  12. Yang H, Sun JP, Lever HM, Popovic ZB, Drinko JK, Greenberg NL, Shiota T, Thomas JD, Garcia MJ (2003) Use of strain imaging in detecting segmental dysfunction in patients with hypertrophic cardiomyopathy. J Am Soc Echocardiogr 16:233–239

    Article  PubMed  Google Scholar 

  13. Carasso S, Yang H, Woo A, Vannan MA, Jamorski M, Wigle ED, Rakowski H (2008) Systolic myocardial mechanics in hypertrophic cardiomyopathy: novel concepts and implications for clinical status. J Am Soc Echocardiogr 21:675–683

    Article  PubMed  Google Scholar 

  14. Maron BJ, Gottdiener JS, Epstein SE (1981) Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. Am J Cardiol 48:418–428

    Article  CAS  PubMed  Google Scholar 

  15. Klues HG, Schiffers A, Maron BJ (1995) Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol 26:1699–1708

    Article  CAS  PubMed  Google Scholar 

  16. Sicari R, Nihoyannopoulos P, Evangelista A, Kasprzak J, Lancellotti P, Poldermans D, Voigt JU, Zamorano JL, European Association of Echocardiography (2008) Stress echocardiography expert consensus statement: European association of echocardiography (EAE) (a registered branch of the ESC). Eur J Echocardiogr 9:415–437

    Article  PubMed  Google Scholar 

  17. Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJ, Chamber Quantification Writing Group, American Society of Echocardiography’s Guidelines and Standards Committee, European Association of Echocardiography (2005) Recommendations for chamber quantification: a report from the American Society of echocardiography’s guidelines and standards committee and the chamber quantification writing group, developed in conjunction with the European association of echocardiography, a branch of the European society of cardiology. J Am Soc Echocardiogr 18:1440–1463

    Article  PubMed  Google Scholar 

  18. Gottdiener JS, Bednarz J, Devereux R, Gardin J, Klein A, Manning WJ, Morehead A, Kitzman D, Oh J, Quinones M, Schiller NB, Stein JH, Weissman NJ, American Society of Echocardiography (2004) American society of echocardiography recommendations for use of echocardiography in clinical trials. J Am Soc Echocardiogr 17:1086–119. Review

  19. Quiñones MA, Douglas PS, Foster E, Gorcsan J 3rd, Lewis JF, Pearlman AS, Rychik J, Salcedo EE, Seward JB, Stevenson JG, Thys DM, Weitz HH, Zoghbi WA, American College of Cardiology, American Heart Association, American College of Physicians-American Society of Internal Medicine, American Society of Echocardiography, Society of Cardiovascular Anesthesiologists, Society of Pediatric Echocardiography (2003) ACC/AHA clinical competence statement on echocardiography: a report of the American college of cardiology/American heart association/American college of Physicians-American society of internal medicine task force on clinical competence. J Am Coll Cardiol 41:687–708

    Article  PubMed  Google Scholar 

  20. Maron BJ, Bonow RO, Cannon RO III, Leon MB, Epstein SE. Hypertrophic cardiomyopathy (1987) Interrelations of clinical manifestations, pathophysiology, and therapy. N Engl J Med 316:780–789. Review

  21. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American society of echocardiography endorsed by the European association of echocardiography, a registered branch of the European society of cardiology, and the Canadian society of echocardiography. J Am Soc Echocardiogr 23:685–713

    Article  PubMed  Google Scholar 

  22. Yoganathan AP, Cape EG, Sung HW, Williams FP, Jimoh A (1988) Review of hydrodynamic principles for the cardiologist: applications to the study of blood flow and jets by imaging techniques. J Am Coll Cardiol 12:1344–1353

    Article  CAS  PubMed  Google Scholar 

  23. Inoue K, Okayama H, Nishimura K, Nagai T, Suzuki J, Ogimoto A, Saito M, Yoshii T, Hiasa G, Sumimoto T, Funada J, Shigematsu Y, Hamada M, Higaki J (2013) Impact of septal curvature on regional strain in patients with hypertrophic cardiomyopathy. Circ J 77:1040–1045

    Article  PubMed  Google Scholar 

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Correspondence to Patricia Reant.

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Reant, P., Donal, E., Schnell, F. et al. Clinical and imaging description of the Maron subtypes of hypertrophic cardiomyopathy. Int J Cardiovasc Imaging 31, 47–55 (2015). https://doi.org/10.1007/s10554-014-0529-2

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  • DOI: https://doi.org/10.1007/s10554-014-0529-2

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