Skip to main content

Approach to Diagnosis: Echocardiography

  • Chapter
  • First Online:
Book cover Hypertrophic Cardiomyopathy
  • 1383 Accesses

Abstract

The diagnosis of HCM can be challenging. Echocardiography has become the primary method for the initial evaluation of patients with suspected hypertrophic cardiomyopathy. The integrated utilization of two-dimensional echocardiography, Doppler echocardiography, and stress echocardiography allows for the evaluation of the presence and severity of LV wall thickness, diastolic dysfunction, LVOT obstruction, and mitral regurgitation. Thus, echocardiography offers the clinician a comprehensive imaging modality in those with suspected HCM, as well as those already diagnosed and those undergoing surveillance or therapeutic intervention. In this chapter, we detail the benefits of echocardiography for the diagnosis, monitoring, and management of those with suspected or known HCM.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 199.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Adelman AG, McLoughlin MJ, Marquis Y, et al. Left ventricular cineangiographic observations in muscular subaortic stenosis. Am J Cardiol. 1969;24:689–97.

    Article  CAS  PubMed  Google Scholar 

  2. Braunwald E, Lambrew CT, Rockoff SD, et al. Idiopathic hypertrophic subaortic stenosis. I. A description of the disease based upon an analysis of 64 patients. Circulation. 1964;30(Suppl 4):3–119.

    Google Scholar 

  3. Maron BJ, Gottdiener JS, Epstein SE. 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. 1981;48:418–28.

    Article  CAS  PubMed  Google Scholar 

  4. Geske JB, Sorajja P, Nishimura RA, Ommen SR. Evaluation of left ventricular filling pressures by Doppler chocardiography in patients with hypertrophic cardiomyopathy: correlation with direct left atrial pressure measurement at cardiac catheterization. Circulation. 2007;116:2702–8.

    Article  PubMed  Google Scholar 

  5. Yang H, Sun JP, Lever HM, Popovic ZB, Drinko JK, Greenberg NL, et al. Use of strain imaging in detecting segmental dysfunction in patients with hypertrophic cardiomyopathy. J Am Soc Echocardiogr. 2003;16:233–9.

    Article  PubMed  Google Scholar 

  6. Maron BJ, McKenna WJ, Danielson GK, et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation task force on clinical expert consensus documents and the European Society of Cardiology Committee for practice guidelines. J Am Coll Cardiol. 2003;42:1687–713.

    Article  PubMed  Google Scholar 

  7. Feigenbaum H, Armstrong WF, Ryan T, Feigenbaum H. Feigenbaum’s echocardiography. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 139. Print.

    Google Scholar 

  8. Oh JK, Seward JB, Tajik AJ. The Echo manual. Philadelphia: Lippincott Williams & Wilkins; 2006. 22. Print.

    Google Scholar 

  9. Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, Moss AJ, Seidman CE, Young JB. Contemporary definitions and classification of the cardiomyopathies. Circulation. 2006;113:1807–16.

    Article  PubMed  Google Scholar 

  10. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 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;124:2761–96.

    Article  PubMed  Google Scholar 

  11. Feigenbaum H, Armstrong WF, Ryan T, Feigenbaum H. Feigenbaum’s echocardiography. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 181. Print.

    Google Scholar 

  12. Nistri S, Olivotto I, Betocchi S, Losi MA, Valsecchi G, Pinamonti B, Conte MR, Casazza F, Galderisi M, Maron BJ, Cecchi F. Prognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian registry for hypertrophic cardiomyopathy). Am J Cardiol. 2006;98:960–5.

    Article  PubMed  Google Scholar 

  13. Wigle ED, Sasson Z, Henderson MA, et al. Hypertrophic cardiomyopathy. The importance of the site and the extent of hypertrophy. A review. Prog Cardiovasc Dis. 1985;28:1–83.

    Article  CAS  PubMed  Google Scholar 

  14. Maron BJ, Epstein SE. Hypertrophic cardiomyopathy. Recent observations regarding the specificity of three hallmarks of the disease: asymmetric septal hypertrophy, septal disorganization and systolic anterior motion of the anterior mitral leaflet. Am J Cardiol. 1980;45:141–54.

    Article  CAS  PubMed  Google Scholar 

  15. Williams LK, Frenneaux MP, Steeds RP. Echocardiography in hypertrophic cardiomyopathy diagnosis, prognosis, and role in management. Eur J Echocardiogr. 2009;10:iii9–iii14.

    Article  CAS  PubMed  Google Scholar 

  16. Basavarajaiah S, Wilson M, Whyte G, Shah A, McKenna W, Sharma S. Prevalence of hypertrophic cardiomyopathy in highly trained athletes: relevance to pre-participation screening. J Am Coll Cardiol. 2008;51:1033–9.

    Article  PubMed  Google Scholar 

  17. Klues HG, Schiffers A, Maron BJ. 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. 1995;26:1699–708.

    Article  CAS  PubMed  Google Scholar 

  18. Spirito P, Maron BJ. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1990;15:1521–6. 20.

    Article  CAS  PubMed  Google Scholar 

  19. Spirito P, Bellone P, Harris KM, Bernabo P, Bruzzi P, Maron BJ. Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. N Engl J Med. 2000;342:1778–85.

    Article  CAS  PubMed  Google Scholar 

  20. Rickers C, Wilke NM, Jerosch-Herold M, Casey SA, Panse P, Panse N, Weil J, Zenovich AG, Maron BJ. Utility of cardiac magnetic resonance imaging in the diagnosis of hypertrophic cardiomyopathy. Circulation. 2005;112:855–61.

    Article  PubMed  Google Scholar 

  21. Martinez MW. Mid-cavitary obstruction in hypertrophic cardiomyopathy. Heart. 2008;94:10 1287.

    PubMed  Google Scholar 

  22. Monserrat L, Hermida-Prieto M, Fernandez X, Rodríguez I, Dumont C, Cazón L, Cuesta MG, Gonzalez-Juanatey C, Peteiro J, Alvarez N, Penas-Lado M, Castro-Beiras A. Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, and septal defects. Eur Heart J. 2007;28:1953–61.

    Article  CAS  PubMed  Google Scholar 

  23. McKenna WJ, Kleinebenne A, Nihoyannopoulos P, Foale R. Echocardiographic measurement of right ventricular wall thickness in hypertrophic cardiomyopathy: relation to clinical and prognostic features. J Am Coll Cardiol. 1988;1:351–8.

    Article  Google Scholar 

  24. Maron MS, Hauser TH, Dubrow E, Horst TA, Kissinger KV, Udelson JE, Manning WJ. Right ventricular involvement in hypertrophic cardiomyopathy. Am J Cardiol. 2007;100:1293–8.

    Article  PubMed  Google Scholar 

  25. Schiller NB, Shah PM, Crawford M, et al. Recommendations for quantitation of the left ventricle by two-dimensional echocardiography. American Society of Echocardiography Committee on standards, subcommittee on quantitation of two-dimensional echocardiograms. J Am Soc Echocardiogr. 1989;2:358–67.

    Article  CAS  PubMed  Google Scholar 

  26. Maron BJ, Spirito P. Implications of left ventricular remodeling in hypertrophic cardiomyopathy. Am J Cardiol. 1998;81:1339–44.

    Article  CAS  PubMed  Google Scholar 

  27. Olivotto I, Girolami F, Nistri S, Rossi A, Rega L, Garbini F, Grifoni C, Cecchi F, Yacoub MH. The many faces of hypertrophic cardiomyopathy: from developmental biology to clinical practice. J Cardiovasc Transl Res. 2009;2:349–67.

    Article  PubMed  Google Scholar 

  28. Fujiwara H, Onodera T, Tanaka M, Shirane H, Kato H, Yoshikawa J, Osakada G, Sasayama S, Kawai C. Progression from hypertrophic obstructive cardiomyopathy to typical dilated cardiomyopathy-like features in the end stage. Jpn Circ J. 1984;48:1210–4.

    Article  CAS  PubMed  Google Scholar 

  29. Harris KM, Spirito P, Maron MS, et al. Prevalence, clinical profile, and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation. 2006;114:216–25.

    Article  PubMed  Google Scholar 

  30. Douglas PS, Garcia MJ, Haines DE, Lai WW, Manning WJ, Patel AR, Picard MH, Polk DM, Ragosta M, Ward RP, Weiner RB. ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 appropriate use criteria for echocardiography: a report of the American College of Cardiology Foundation appropriate use criteria task force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance Endorsed by the American College of Chest Physicians. J Am Coll Cardiol. 2011;57:1126–66.

    Article  PubMed  Google Scholar 

  31. Feigenbaum H, Armstrong WF, Ryan T, Feigenbaum H. Feigenbaum’s echocardiography. Philadelphia: Lippincott Williams & Wilkins; 2005. 184. Print

    Google Scholar 

  32. Sachdev V, Shizukuda Y, Brenneman CL, Birdsall CW, Waclawiw MA, Arai AE, Mohiddin SA, Tripodi D, Fananapazir L, Plehn JF. Left atrial volumetric remodeling is predictive of functional capacity in nonobstructive hypertrophic cardiomyopathy. Am Heart J. 2005;149(4):730–6.

    Article  PubMed  Google Scholar 

  33. Yang H, et al. Enlarged left atrial volume in hypertrophic cardiomyopathy: a marker for disease severity. J Am Soc Echocardiogr. 2005;18(10):1074–82.

    Article  PubMed  Google Scholar 

  34. Hatle L, Angelsen B. Doppler ultrasound in cardiology. 2nd ed. Philadelphia: Lea & Febiger; 1985.

    Google Scholar 

  35. Izgi C, Akgun T, Men EE, Feray H. Systolic anterior motion of the mitral valve in the absence left ventricular hypertrophy: role of mitral leaflet elongation and papillary muscle displacement. Echocardiography 2010; 27:E36–8. Lefebvre XP, He S, Levine RA, Yoganathan AP. Systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy: an in vitro pulsatile flow study. J Heart Valve Dis. 1995;4:422–38.

    Google Scholar 

  36. He S, Hopmeyer J, Lefebvre XP, Schwammenthal E, Yoganathan AP, Levine RA. Importance of leaflet elongation in causing systolic anterior motion of the mitral valve. J Heart Valve Dis. 1997;6:149–59.

    CAS  PubMed  Google Scholar 

  37. Sherrid MV, Gunsburg DZ, Moldenhauer S, Pearle G. Systolic anterior motion begins at low left ventricular outflow tract velocity in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2000;36:1344–54.

    Article  CAS  PubMed  Google Scholar 

  38. Maron MS, Olivotto I, Zenovich AG, et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outfl ow tract obstruction. Circulation. 2006;114:2232–9.

    Article  PubMed  Google Scholar 

  39. Klues HG, Roberts WC, Maron BJ. Morphological determinants of echocardiographic patterns of mitral valve systolic anterior motion in obstructive hypertrophic cardiomyopathy. Circulation. 1993;87:1570–9.

    Article  CAS  PubMed  Google Scholar 

  40. Hoigne P, Attenhofer Jost CH, Duru F, Oechslin EN, Seifert B, Widmer U, et al. Simple criteria for differentiation of Fabry disease from amyloid heart disease and other causes of left ventricular hypertrophy. Int J Cardiol. 2006;111:413–22.

    Article  PubMed  Google Scholar 

  41. Yu EH, Omran AS, Wigle ED, Williams WG, Siu SC, Rakowski H. Mitral regurgitation in hypertrophic obstructive cardiomyopathy: relationship to obstruction and relief with myectomy. J Am Coll Cardiol. 2000;36:2219–25.

    Article  CAS  PubMed  Google Scholar 

  42. Kaple RK, Murphy RT, DiPaola LM, Houghtaling PL, Lever HM, Lytle BW, et al. Mitral valve abnormalities in hypertrophic cardiomyopathy: echocardiographic features and surgical outcomes. Ann Thorac Surg. 2008;85:1527–35. 1535.

    Article  PubMed  Google Scholar 

  43. Omoto R, Kasai C. Physics and instrumentation of Doppler color flow mapping. Echocardiography. 1987;4:467–83.

    Article  Google Scholar 

  44. Maron MS, Finley JJ, Bos JM, Hauser TH, Manning WJ, Haas TS, et al. Prevalence, clinical significance, and natural history of left ventricular apical aneurysms in hypertrophic cardiomyopathy. Circulation. 2008;118:1541–9.

    Article  PubMed  Google Scholar 

  45. Oh JK, Seward JB, Tajik AJ. The Echo manual. Philadelphia: Lippincott Williams & Wilkins; 2006. 60. Print

    Google Scholar 

  46. Maron BJ, Gottdiener JS, Arce J, et al. Dynamic subaortic obstruction in hypertrophic cardiomyopathy: analysis by pulsed Doppler echocardiography. J Am Coll Cardiol. 1985;6:1–18.

    Article  CAS  PubMed  Google Scholar 

  47. Bom K, de Boo J, Rijsterborgh H. On the aliasing problem in pulsed Doppler cardiac studies. J Clin Ultrasound. 1984;12:559–67.

    Article  CAS  PubMed  Google Scholar 

  48. Panza JA, Petrone RK, Fananapazir L, Maron BJ. Utility of continuous wave Doppler echocardiography in the noninvasive assessment of left ventricular outflow tract pressure gradient in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1992;19(1):91–9.

    Article  CAS  PubMed  Google Scholar 

  49. Shah JS, Esteban MT, Thaman R, Sharma R, Mist B, Pantazis A, Ward D, Kohli SK, Page SP, Demetrescu C, Sevdalis E, Keren A, Pellerin D, McKenna WJ, Elliott PM. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy. Heart. 2008;94:1288–94.

    Article  CAS  PubMed  Google Scholar 

  50. Briguori C, Betocchi S, Losi MA, et al. Noninvasive evaluation of left ventricular diastolic function in hypertrophic cardiomyopathy. Am J Cardiol. 1998;81:180–7.

    Article  CAS  PubMed  Google Scholar 

  51. Maron BJ, Spirito P, Green KJ, Wesley YE, Bonow RO, Arce J. Noninvasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 1987;10:733–42.

    Article  CAS  PubMed  Google Scholar 

  52. Nagueh SF, Lakkis NM, Middleton KJ, Spencer WH III, Zoghbi WA, Quinones MA. Doppler estimation of left ventricular filling pressures in patients with hypertrophic cardiomyopathy. Circulation. 1999;99:254–61.

    Article  CAS  PubMed  Google Scholar 

  53. Nishimura RA, Appleton CP, Redfield MM, Ilstrup DM, Holmes DR Jr, Tajik AJ. Noninvasive Doppler echocardiographic evaluation of left ventricular filling pressures in patients with cardiomyopathies: a simultaneous Doppler echocardiographic and cardiac catheterization study. J Am Coll Cardiol. 1996;28:1226–33.

    Article  CAS  PubMed  Google Scholar 

  54. Geske JB, Sorajja P, Nishimura RA, Ommen SR. Evaluation of left ventricular filling pressures by Doppler echocardiography in patients with hypertrophic cardiomyopathy: correlation with direct left atrial pressure measurement at cardiac catheterization. Circulation. 2007;116:2702–8.

    Article  PubMed  Google Scholar 

  55. Matsumura Y, Elliott PM, Virdee MS, Sorajja P, Doi Y, McKenna WJ. Left ventricular diastolic function assessed using Doppler tissue imaging in patients with hypertrophic cardiomyopathy: relation to symptoms and exercise capacity. Heart. 2002;87:247–51.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  56. McMahon CJ, Nagueh SF, Pignatelli RH, et al. Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy. Circulation. 2004;109:1756–62.

    Article  PubMed  Google Scholar 

  57. Nagueh SF, Smiseth OA, Appleton CP, Byrd BF. el al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29:277–314.

    Article  PubMed  Google Scholar 

  58. Ho CY, Solomon SD. A clinician’s guide to tissue Doppler imaging. Circulation. 2006;113:e396–8.

    PubMed  Google Scholar 

  59. Vinereanu D, Florescu N, Sculthorpe N, Tweddel AC, Stephens MR, Fraser AG. Differentiation between pathologic and physiologic left ventricular hypertrophy by tissue Doppler assessment of long-axis function in patients with hypertrophic cardiomyopathy or systemic hypertension and in athletes. Am J Cardiol. 2001;88:53–8.

    Article  CAS  PubMed  Google Scholar 

  60. Bayrak F, Kahveci G, Mutlu B, Sonmez K, Degertekin M. Tissue Doppler imaging to predict clinical course of patients with hypertrophic cardiomyopathy. Eur J Echocardiogr. 2008;9:278–83.

    Article  PubMed  Google Scholar 

  61. Carasso S, Yang H, Woo A, Vannan MA, Jamorski M, Wigle ED, et al. Systolic myocardial mechanics in hypertrophic cardiomyopathy: novel concepts and implications for clinical status. J Am Soc Echocardiogr. 2008;21:675–83.

    Article  PubMed  Google Scholar 

  62. Van Dalen BM, Kauer F, Soliman OI, Vletter WB, Michels M, ten Cate FJ, et al. Influence of the pattern of hypertrophy on left ventricular twist in hypertrophic cardiomyopathy. Heart. 2009;95:657–61.

    Article  PubMed  Google Scholar 

  63. Popovic ZB, Kwon DH, Mishra M. Association between regional ventricular function and myocardial fibrosis in hypertrophic cardiomyopathy assessed by speckle tracking echocardiography and delayed hyperenhancement magnetic resonance imaging. J Am Soc Echocardiogr. 2008;21:1299–305.

    Article  PubMed  Google Scholar 

  64. Nishimura RA, Ommen SR. Hypertrophic cardiomyopathy: the search for obstruction. Circulation. 2006;114:2200–2.

    Article  PubMed  Google Scholar 

  65. Okeie K, Shimizu M, Yoshio H, Ino H, Yamaguchi M, Matsuyama T, Mabuchi H. Left ventricular systolic dysfunction during exercise and dobutamine stress in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol. 2000;36(3):856–63.

    Article  CAS  PubMed  Google Scholar 

  66. Pellikka PA, Oh JK, Bailey KR, Nichols BA, Monahan KH, Tajik AJ. Dynamic intraventricular obstruction during dobutamine stress echocardiography. A new observation. Circulation. 1992;86(5):1429–32.

    Article  CAS  PubMed  Google Scholar 

  67. Gersh BJ, Maron BJ, Bonow RO, Dearani JA, Fifer MA, Link MS, Naidu SS, Nishimura RA, Ommen SR, Rakowski H, Seidman CE, Towbin JA, Udelson JE, Yancy CW. 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. J Thorac Cardiovasc Surg. 2011;142:1303–8.

    Article  PubMed  Google Scholar 

  68. Kawano S, Iida K, Fujieda K, Yukisada K, Magdi ES, Iwasaki Y, Sugishita Y. Response to isoproterenol as a prognostic indicator of evolution from hypertrophic cardiomyopathy to a phase resembling dilated cardiomyopathy. J Am Coll Cardiol. 1995;25(3):687–92.

    Article  CAS  PubMed  Google Scholar 

  69. Marwick TH, Nakatani S, Haluska B, Thomas JD, Lever HM. Provocation of latent left ventricular outflow tract gradients with amyl nitrite and exercise in hypertrophic cardiomyopathy. Am J Cardiol. 1995;75(12):805–9.

    Article  CAS  PubMed  Google Scholar 

  70. Maron MS, Olivotto I, Zenovich AG, Link MS, Pandian NG, Kuvin JT, Nistri S, Cecchi F, Udelson JE, Maron BJ. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006;114:2232–9.

    Article  PubMed  Google Scholar 

  71. Schwammenthal E, Schwartzkopff B, Block M, Johns J, Lösse B, Engberding R, Breithardt G. Doppler echocardiographic assessment of the pressure gradient during bicycle ergometry in hypertrophic cardiomyopathy. Am J Cardiol. 1992;69(19):1623–8.

    Article  CAS  PubMed  Google Scholar 

  72. Lancellotti P, Pellikka PA, Budts W, Chaudhry FA, et al. Non-ischemic heart disease: recommendations from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. J Am Soc Echocardiogr. 2017;30:101–38.

    Article  PubMed  Google Scholar 

  73. Schnell F, Donal E, Bernard-Brunet A, Reynaud A, Wilson MG, Thebault C, et al. Strain analysis during exercise in patients with left ventricular hypertrophy: impact of etiology. J Am Soc Echocardiogr. 2013;26:1163–9.

    Article  PubMed  Google Scholar 

  74. Butz T, van Buuren F, Mellwig KP, Langer C, Plehn G, Meissner A, et al. Two-dimensional strain analysis of the global and regional myocardial function for the differentiation of pathologic and physiologic left ventricular hypertrophy: a study in athletes and in patients with hypertrophic cardiomyopathy. Int J Cardiovasc Imaging. 2011;27:91–100.

    Article  CAS  PubMed  Google Scholar 

  75. Argulian E, Chaudhry FA. Stress testing in patients with hypertrophic cardiomyopathy. Prog Cardiovasc Dis. 2012;54:477–82.

    Article  PubMed  Google Scholar 

  76. Nagueh SF, Lakkis NM, He ZX, Middleton KJ, Killip D, Zoghbi WA, Quiñones MA, Roberts R, Verani MS, Kleiman NS, Spencer WH III. Role of myocardial contrast echocardiography during nonsurgical septal reduction therapy for hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol. 1998;32:225–9.

    Article  CAS  PubMed  Google Scholar 

  77. Sigwart U. Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy. Lancet. 1995;346:211–4.

    Article  CAS  PubMed  Google Scholar 

  78. Alam M, Dokainish H, Lakkis N. Alcohol septal ablation for hypertrophic obstructive cardiomyopathy: a systematic review of published studies. J Interv Cardiol. 2006;19:319–27.

    Article  PubMed  Google Scholar 

  79. Teis A, Sheppard MN, Alpendurada F. Subaortic membrane: correlation of imaging with pathology. Eur Heart J. 2010;31:2822.

    Article  PubMed  Google Scholar 

  80. Rahman JE, Helou EF, Gelzer-Bell R, Thompson RE, Kuo C, Rodriguez ER, et al. Noninvasive diagnosis of biopsy-proven cardiac amyloidosis. J Am Coll Cardiol. 2004;43(3):410–5.

    Article  PubMed  Google Scholar 

  81. Badano L, Fox K, Sicari R, Zamorano JL. Disease with a main influence on myocardial tissue damage. EAE Textbook of echocardiography. 1st ed: Oxford Medical Press; New York, NY. 2011. p. 335–6.

    Google Scholar 

  82. Phelan D, Collier P, Thavendiranathan P, Popovic ZB, Hanna M, Plana JC, et al. Relative ‘apical sparing’ of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart. 2012;98(19):1442–8.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Matthew W. Martinez .

Editor information

Editors and Affiliations

Questions

Questions

  1. 1.

    What advantage of M-mode echocardiography makes it ideal for measurements in HCM?

    1. A.

      High lateral resolution

    2. B.

      High temporal resolution

    3. C.

      High tissue penetration

    4. D.

      High axial resolution

    Answer: B. The advantage of M-mode echocardiography over other imaging modalities is the very high temporal resolution that it provides. As a result, it allows for the examination of high-frequency motion. For example, subtle abnormalities such as partial mid-systolic closure of the aortic valve due to subvalvular obstruction and systolic anterior motion of the mitral valve are demonstrated best using M-mode echocardiography. The axial resolution of an image is mostly affected by pulse length and frequency. The lateral resolution is affected by beam width, depth, and gain. The depth of penetration is directly related to the wavelength of US.

  2. 2.

    True or False. RV hypertrophy is rare in HCM.

    Answer: False. Right ventricular hypertrophy is often seen in HCM. In one study right ventricular hypertrophy was seen in 44% of known HCM patients. RVH on echo is best seen in the subcostal view. A greater than 5 mm measurement of the RV free wall is associated with RVH.

  3. 3.

    What is a pitfall of using M-mode echocardiography for linear measurements?

    1. A.

      Low temporal resolution

    2. B.

      Poor tissue penetration

    3. C.

      Finding a representative portion of the LV

    Answer: C. Since M-mode echocardiography is essentially an “ice pick” view of the heart, it is often difficult to determine which precise part of the heart is being visualized. Furthermore, M-mode may capture an off-axis cut, thus giving an oblique view of the LV wall. Temporal resolution is highest by M-mode echo.

  4. 4.

    Which of the following is true?

    1. A.

      Classically, LV wall thickness measurements are made at end diastole in the four-chamber view.

    2. B.

      The area of interest for the measurement is only the basal septum.

    3. C.

      Highly trained athlete wall thickness often exceeds 15 mm.

    4. D.

      Extreme wall thickness of greater than 30 mm is associated with sudden cardiac death.

    Answer: D. A greater than 30 mm wall thickness is associated with sudden death and is a Class 2a indication for an implantable defibrillator. The wall thicknesses of highly trained athletes are rarely greater than 15 mm. Typically one measures for LV hypertrophy in the parasternal long or short axis. The four-chamber view is never used. The area of interest is usually the basal septum, but various patterns and distribution of LV hypertrophy (including diffuse and marked) have been reported in HCM.

  5. 5.

    Which of the following statements about echocardiographic left atrial size and HCM is true?

    1. A.

      The left atrium is measured in the parasternal long axis view.

    2. B.

      The preferred measurement for left atrial size is area.

    3. C.

      A left atrial indexed volume of greater than 34ml/m2 has prognostic implications.

    4. D.

      The left atrial volume can only be approximated by 3D echocardiography.

    C. The preferred size measurement for the left atrium is volume indexed by body surface area. 2D echo can be used to approximate left atrial volume by long-/short-axis measurements. These measurements should be done in the four-chamber and two-chamber apical views. A left atrial indexed volume of greater than 34 mL/m2 has been shown to prognosticate more serious cardiovascular events and greater LV hypertrophy, more diastolic dysfunction, and higher filling pressures.

  6. 6.

    In which views are the LVOT gradient checked?

    1. A.

      The apical five-chamber

    2. B.

      The apical three-chamber

    3. C.

      Both a and b

    4. D.

      Neither a nor b

    Answer: C. HCM patients often have LVOT obstruction. It is important to measure gradients properly to ascertain the level and location of the obstruction. The best way to measure LVOT gradients is in the apical four- and five-chamber views. In symptomatic HCM patients who do not have significant pressure gradients at rest, a dynamic obstruction must be investigated.

  7. 7.

    Which of the following is not a recommended parameter to evaluate for diastolic dysfunction grade in HCM patients?

    1. A.

      Pulmonary vein atrial reversal velocity <30 msec.

    2. B.

      E/E’ ratio >14.

    3. C.

      LA volume index of >34 ml/m2.

    4. D.

      Peak velocity of the TR jet >2.8m/sec.

    A. The American Society of Echocardiography established new guidelines for the determination of diastolic dysfunction in 2016. There are now four recommended parameters needed to determine diastolic dysfunction grade. These four parameters are average E/E′ ratio of >14, LA volume index of >34 ml/m2, pulmonary vein atrial reversal velocity time of >30 msec (not <30 msec), and peak velocity of TR jet of >2.8 m/sec.

  8. 8.

    Your patient’s echocardiogram has the following values: E/E′ ratio of 16, LA volume of 37 ml/m2, pulmonary vein reversal velocity time of 25 msec, and TR jet velocity of 2.5 m/sec. What grade diastolic function does the patient have?

    1. A.

      Grade I

    2. B.

      Grade II

    3. C.

      Grade III

    4. D.

      Indeterminate

    Answer: D. The recommended parameters needed to evaluate diastolic dysfunction grade in HCM are average E/E′ ratio of >14, LA volume index of >34 ml/m2, pulmonary vein atrial reversal velocity time of >30 msec, and peak velocity of TR jet of >2.8 m/sec. If less than 50% of the parameters are met, then LA pressure is normal, and grade I diastolic dysfunction is present. If greater than 50% of the parameters are met, then the LA pressure is elevated, and grade II diastolic dysfunction is present. In the situation that exactly 50% of the parameters are met, estimated LA pressures and diastolic grade are indeterminate. Finally, there is grade III diastolic dysfunction in the presence of a restrictive filling pattern (E/A > 2.5, deceleration time of E velocity of <150 msec, and isovolumic relaxation time <50 msec) and reduced E′ velocity. This patient had 50% of the criteria; thus the grade is indeterminate.

  9. 9.

    Contrast echocardiography media plays an important role in hypertrophic cardiomyopathy assessment in all of the following except:

    1. A.

      The evaluation of SAM

    2. B.

      Apical HCM

    3. C.

      Thrombus formation in a “burned-out” reduced LVEF HCM patient

    4. D.

      To identify appropriate coronary vessels for alcohol septal ablation

    Answer: A. Contrast echocardiography plays an important role in HCM evaluation. Often the apical walls are not well visualized on a TTE. The classic spade appearance of the ventricular lumen of an apical HCM patient is well recognized with contrast. In later stages of HCM, one can have severely reduced LVEF. These patients are susceptible to forming LV thrombus which is best identified with contrast. Finally, contrast media is injected into the coronary arteries, to identify and confirm the appropriate septal branch that supplies the myocardium for alcohol septal ablation. It is important to identify the desired area for ablation and to avoid alcohol injection into a papillary muscle or LV free wall. Contrast media does not play a major role in evaluating SAM.

  10. 10.

    TEE is an important tool in HCM evaluation in the following situations except:

    1. A.

      Patient with HCM and concomitant intrinsic degenerative mitral valve disease

    2. B.

      Differentiating HCM from a subaortic membrane

    3. C.

      Intraoperatively during septal myectomy

    4. D.

      Checking LVOT and aortic valve gradients in a patient with HCM and aortic stenosis

    Answer: D. HCM patients often have mitral regurgitation. However, it may be difficult to discern intrinsic mitral valve disease versus MR secondary to SAM in an HCM on a TTE. TEE is the best tool to evaluate for intrinsic mitral valve disease in these patients. Although rare, an important differential of LVOT gradients is subaortic membrane. These are often missed on TTE. Having TEE imaging is a class I indication during intracardiac surgery. HCM evaluation with concomitant AS is often challenging. Checking LVOT and AS gradients in TEE, although possible, is not ideal because it is often difficult to obtain Doppler measurements parallel to flow. TEE can be valuable in these patients when evaluating the aortic valve structure and measuring aortic valve area by planimetry in the setting of high gradients.

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Ray, B., Martinez, M.W. (2019). Approach to Diagnosis: Echocardiography. In: Naidu, S. (eds) Hypertrophic Cardiomyopathy. Springer, Cham. https://doi.org/10.1007/978-3-319-92423-6_4

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-92423-6_4

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-92422-9

  • Online ISBN: 978-3-319-92423-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics