Hypertrophic Cardiomyopathy and Sudden Death in the Young: a Pathologist’s View

  • C. Basso
  • D. Corrado
  • A. Angelini
  • G. Thiene
Conference paper


Hypertrophic cardiomyopathy (HCM) is a highly arrhythmogenic primary heart muscle disease. Clinical manifestations are variable ranging from a benign asymptomatic course to severe heart failure and cardiac arrest. Ventricular arrhythmias, palpitations and syncope are frequent signs and symptoms. Sudden, unexpected cardiac arrest is a pending catastrophe in the natural history of HCM.


Sudden Death Hypertrophic Cardiomyopathy Restrictive Cardiomyopathy Systolic Anterior Motion Trophic Cardiomyopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Maron BJ, Bonow RO, Cannon RO, Leon MB, Epstein SE (1987) Hypertrophic cardiomyopathy. Interrelations of clinical manifestations, pathophysiology and therapy (1). N Engl J Med 316:780–789 and 844-852PubMedGoogle Scholar
  2. 2.
    Teare RD (1958) Asymmetrical septal hypertrophy of the heart in young adults. Br Heart J 20:1–8PubMedCrossRefGoogle Scholar
  3. 3.
    Braunwald E, Morrow AG, Cornell WP, Aygen MM, Hilbish TF (1960) Idiopathic hypertrophic subaortic stenosis. Am J Med 29:924–945CrossRefGoogle Scholar
  4. 4.
    Wigle ED, Rakowski H, Kimball BP, Williams WG (1995) Hypertrophic cardiomyopathy. Clinical spectrum and treatment. Circulation 92:1680–1692Google Scholar
  5. 5.
    Klues HG, Schiffers A, Maron BJ (1995) Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophie cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol 26:1699–1708PubMedCrossRefGoogle Scholar
  6. 6.
    Wigle ED, Sasson Z, Henderson MA, Ruddy TD, Fulop J, Rakowski H, Williams WG (1985) Hypertrophic cardiomyopathy: the importance of the site and the extent of hypertrophy: a review. Prog Cadiovasc Dis 28:1–83CrossRefGoogle Scholar
  7. 7.
    Brugada P, Bar FWHM, De Zwann C, Roy D, Green M, Wellens HJJ (1982) “Sawfish ” systolic narrowing of the left anterior descending coronary artery: an angiographie sign of hypertrophie cardiomyopathy. Circulation 66:800–803PubMedCrossRefGoogle Scholar
  8. 8.
    Maron BJ, Roberts WC (1979) Quantitative analysis of cardiac muscle cell disorganization in the ventricular septum of patients with hypertrophie cardiomyopathy. Circulation 59:689–706PubMedCrossRefGoogle Scholar
  9. 9.
    Maron BJ, Anan TJ, Roberts WC (1981) Quantitative analysis of the distribution of cardiac muscle cell disorganization in the left ventricular wall of patients with hypertrophic cardiomyopathy. Circulation 63:882–894PubMedCrossRefGoogle Scholar
  10. 10.
    Ferrans VJ, Rodriguez ER (1983) Specificity of light and electron microscopic features of hypertrophie obstructive and nonobstructive cardiomyopathy. Qualitative, quantitative and etiologic aspects. Eur Heart J 4 [Suppl F]:9–22Google Scholar
  11. 11.
    StJohn Sutton MG, Lie JT, Anderson KR, O’Brien PC, Frye RL (1980) Histologie specificity of hypertrophie obstructive cardiomyopathy: myocardial fiber disarray and myocardial fibrosis. Br Heart J 44:433–443CrossRefGoogle Scholar
  12. 12.
    Factor SM, Butany J, Sole MJ, Wigle ED, Williams WC, Pojkind M (1991) Pathologic fibrosis and matrix connective tissue in the subaortic myocardium of patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 17:1343–1351PubMedCrossRefGoogle Scholar
  13. 13.
    Davies MJ, McKenna WJ (1995) Hypertrophic cardiomyopathy-pathology and pathogenesis. Histopathology 26:493–500PubMedCrossRefGoogle Scholar
  14. 14.
    Maron BJ, Epstein SE, Roberts WC (1979) Hypertrophic cardiomyopathy and transmural myocardial infarction without significant atherosclerosis of the extramural coronary arteries. Am J Cardiol 43:1086–1102PubMedCrossRefGoogle Scholar
  15. 15.
    Maron BJ, Wolfson JK, Epstein SE, Roberts WC (1986) Intramural (“small vessel ”) coronary artery disease in hypertrophie cardiomyopathy. J Am Coll Cardiol 8:545–557PubMedCrossRefGoogle Scholar
  16. 16.
    Slade AKB, Saumarez RC, McKenna WJ (1993) The arrhythmogenic substrate-diagnostic and therapeutic implications: hypertrophie cardiomyopathy. Eur Heart J 14 [Suppl E]:84–90PubMedCrossRefGoogle Scholar
  17. 17.
    Morrow AG, Brockenbrough EC (1961) Surgical treatment of idiopathic hypertrophie subaortic stenosis: technique and hemodynamic results of subaortic ventriculomyotomy. Ann Surg 154:181–189PubMedCrossRefGoogle Scholar
  18. 18.
    Spirito P, Seidman CE, McKenna WJ, Maron BJ (1997) The management of hypertrophic cardiomyopathy. N Engl J Med 336:775–785PubMedCrossRefGoogle Scholar
  19. 19.
    Wigle ED, Wilansky S (1987) Diastolic dysfunction in hypertrophie cardiomyopathy. Heart Failure 3:82–93Google Scholar
  20. 20.
    Maron BJ, Spirito P, Greene KJ, Wesley YE, Bonow RO, Arce J (1987) Non invasive assessment of left ventricular diastolic function by pulsed Doppler echocardiography in patients with hypertrophie cardiomyopathy. J Am Coll Cardiol 10:733–742PubMedCrossRefGoogle Scholar
  21. 21.
    Inoue T, Morooka S, Hayashi T, Takayanagi K, Sakai Y, Fujito T, Fujinuma S, Takabatake Y (1991) Global and regional abnormalities of left ventricular diastolic filling in hypertrophie cardiomyopathy. Clin Cardiol 14:573–577PubMedCrossRefGoogle Scholar
  22. 22.
    Shirani J, Maron BJ, Cannon III RO, Shahin S, Roberts WC (1993) Clinico-pathologic features of hypertrophie cardiomyopathy managed by cardiac transplantation. Am J Cardiol 72:434–440PubMedCrossRefGoogle Scholar
  23. 23.
    Boffa GM, Thiene G, Nava A, Dalla Volta S (1991) Cardiomyopathy: a necessary revision of the WHO classification. Int J Cardiol 30:1–7PubMedCrossRefGoogle Scholar
  24. 24.
    Boffa GM, Chioin R, Pellegrino PA, Scognamiglio R, Fasoli G, Razzolini R, Milanesi O, Thiene G (1983) Cardiomiopatia restrittiva primaria. Rev Lat Cardiol 4:263–275Google Scholar
  25. 25.
    Angelini A, Calzolari V, Boffa GM, Calabrese F, Valente M, Thiene G (1995) Primary restrictive cardiomyoapthy. Eur Heart J 16 [Suppl]:395 (abstr)Google Scholar
  26. 26.
    Maron BJ, Lang L, Moller JH, Edwards JE (1980) Cardiomyopathies characterized by evidence of resistence to left ventricular inflow. Cathet Cardiovasc Diag 6:29–39CrossRefGoogle Scholar
  27. 27.
    Arbustini E, Buonanno C, Trevi P, Pennelli N, Ferrans VJ, Thiene G (1983) Cardiac ultrastructure in primary restrictive cardiomyopathy. Chest 84:236–238PubMedCrossRefGoogle Scholar
  28. 28.
    Edwards WD (1987) Cardiomyopathies. Hum Pathol 18:625–635PubMedCrossRefGoogle Scholar
  29. 29.
    Katritsis D, Wilmshurst PT, Wendon JA, Davies MJ, Webb-People MM (1991) Primary restrictive cardiomyopathy:clinical and pathologic characteristics. J Am Coll Cardiol 18:1230–1235PubMedCrossRefGoogle Scholar
  30. 30.
    Aroney C, Bett N, Redford D (1988) Familial restrictive cardiomyopathy. Aust Nz J Med 18:877–878CrossRefGoogle Scholar
  31. 31.
    McKenna WJ, Stewart JT, Nihoyannopoulos P, McGinfy F, Davies MJ (1990) Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass. Br Heart J 63:287–290CrossRefGoogle Scholar
  32. 32.
    McKenna WJ, Camm AJ (1989) Sudden death in hypertrophie cardiomyopathy: assessment of patients at high risk. Circulation 80:1489–1492PubMedCrossRefGoogle Scholar
  33. 33.
    Maron BJ, Fananapazir L (1992) Sudden death in hypertrophie cardiomyopathy. Circulation 85 [Suppl I]:57–63Google Scholar
  34. 34.
    Maron BJ, Roberts WJ, Epstein SE (1982) Sudden death in hypertrophie cardiomyopathy: a profile of 78 patients. Circulation 67:1388–1394CrossRefGoogle Scholar
  35. 35.
    McKenna WJ, Deanfield J, Faruqui A, England D, Oakley C, Goodwin J (1981) Prognosis in hypertrophie cardiomyopathy: role of age and clinical, electrocardiographic and hemodinamic features. Am J Cardiol 47:532–538PubMedCrossRefGoogle Scholar
  36. 36.
    Spirito P, Chiarella F, Carratino L, Berisso MZ, Bellotti P, Vecchio C (1989) Clinical course and prognosis of hypertrophie cardiomyopathy in an outpatient population. N Engl J Med 320:749–755PubMedCrossRefGoogle Scholar
  37. 37.
    Cecchi F, Olivotto I, Montereggi A, Santoro G, Dolara A, Maron BJ (1995) Hypertrophic cardiomyopathy in Tuscany: clinical course and outcome in an unselected regional population. J Am Coll Cardiol 26:1529–1536PubMedCrossRefGoogle Scholar
  38. 38.
    McKenna WJ, Deanfield JE (1984) Hypertrophic cardiomyopathy. An important cause of sudden death. Arch Dis Chil 59:971–975CrossRefGoogle Scholar
  39. 39.
    McKenna WJ, Franklin RCG, Nihoyannopoulos P, Robinson KR, Deanfield JE (1988) Arrhythmia and prognosis in infants, children and adolescents with hypertrophic cardiomyopathy. J Am Coll Cardiol 11:147–153PubMedCrossRefGoogle Scholar
  40. 40.
    Spirito P, Maron BJ, Bonow RO, Epstein SE (1987) Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol 60:123–129PubMedCrossRefGoogle Scholar
  41. 41.
    Topaz O, Edwards JE (1985) Pathologic features of sudden death in children, adolescents and young adults. Chest 87:476–482PubMedCrossRefGoogle Scholar
  42. 42.
    Burke AP, Farb A, Virmani R, Goodin J, Smialek JE (1991) Sports-related and non sports-related sudden cardiac death in young adults. Am Heart J 121:568–575PubMedCrossRefGoogle Scholar
  43. 43.
    Basso C, Corrado D, Nava A, Dalla Volta S (1996) Hypertrophic cardiomyopathy: pathologic evidence of myocardial ischemie injury in young sudden death victims. Circulation 94 [Suppl]:I–427 (abstr)CrossRefGoogle Scholar
  44. 44.
    Cannon RO, Rosing DR, Maron BJ, Leon MB, Bonow RO, Watson RM, Epstein SE (1985) Myocardial ischemia in patients with hypertrophie cardiomyopathy: contribution of inadequate vasodilator reserve and elevated left ventricular filling pressures. Circulation 71:234–243PubMedCrossRefGoogle Scholar
  45. 45.
    Dilsizian V, Bonow RO, Epstein SE, Fananapazir L (1993) Myocardial ischemia detected by thallium scintigraphy is frequently related to cardiac arrest and syncope in young patients with hypertrophie cardiomyopathy. J Am Coll Cardiol 22:796–804PubMedCrossRefGoogle Scholar
  46. 46.
    Maron BJ, Roberts WC, McAllister HA, Rosing DR, Epstein SE (1980) Sudden death in young athletes. Circulation 62:218–229PubMedCrossRefGoogle Scholar
  47. 47.
    Corrado D, Basso C, Thiene G (1996) Pathologic findings in victims of sport-related sudden cardiac death. Sports Exercise and Injury 2:78–86Google Scholar
  48. 48.
    Corrado D, Thiene G, Nava A, Rossi L, Pennelli N (1990) Sudden death in young competitive athletes: clinico-pathologic correlations in 22 cases. Am J Med 89:588–596PubMedCrossRefGoogle Scholar
  49. 49.
    Thiene G, Nava A, Corrado D, Rossi L, Pennelli N (1988) Right ventricular cardiomyopathy and sudden death in young people. N Engl J Med 318:129–133PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 1998

Authors and Affiliations

  • C. Basso
  • D. Corrado
  • A. Angelini
  • G. Thiene

There are no affiliations available

Personalised recommendations