Recent Advances in the Natural History of Dilated Cardiomyopathy: A Review of the Heart Muscle Disease Registry of Trieste

  • M. Moretti
  • A. Di Lenarda
  • G. Sinagra
Part of the Topics in Anaesthesia and Critical Care book series (TIACC)


Dilated cardiomyopathy (DCM) is heart muscle disease characterized by left ventricular or biventricular dilatation and impaired myocardial contractility [1]. It is an important cause of morbidity and mortality, and is one of the two most frequent indications for cardiac transplantation. The prevalence of DCM in the United States has been estimated at around 0.04% [2], with an annual incidence of 0.005–0.006% [2, 3].


Dilate Cardiomyopathy Left Bundle Branch Block Left Ventricular Filling Idiopathic Dilate Cardiomyopathy Restrictive Pattern 
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.
    Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies (1996) Circulation 93:841–2Google Scholar
  2. 2.
    Codd MB, Sugrue DD, Gersh BJ et al (1989) Epidemiology of idiopathic dilated and hypertrophic cardiomyopathy. A population-based study in Olmsted County, Minnesota, 1975–1984. Circulation 80:564–72Google Scholar
  3. 3.
    Mestroni L, Rocco C, Gregori D et al (1999) Familial dilated cardiomyopathy: evidence for genetic and phenotypic heterogeneity. Heart Muscle Disease Study Group. J Am Coll Cardiol 34:181–90PubMedCrossRefGoogle Scholar
  4. 4.
    Gavazzi A, Lanzarini L, Cornalba C et al (1984) Dilated (congestive) cardiomyopathy. G Ital Cardiol 14:492–8PubMedGoogle Scholar
  5. 5.
    Franciosa JA, Wilen M, Ziesche S et al (1983) Survival in men with severe chronic left ventricular failure due to either coronary heart disease or idiopathic dilated cardiomyopathy. Am J Cardiol 51:831–6PubMedCrossRefGoogle Scholar
  6. 6.
    Di Lenarda A, Secoli G, Perkan A et al (1994) Changing mortality in dilated cardiomyopathy. Br Heart J 72:46–51Google Scholar
  7. 7.
    Di Lenarda A, Sabbadini G, Gortan R et al (2001) Has the prognosis of idiopathic dilated cardiomyopathy improved in the community? The Heart Muscle Disease Registry of Trieste (abstract). J Am Coll Cardiol 37:169AGoogle Scholar
  8. 8.
    Massa L, Vitali-Serdoz L, Di Lenarda A et al (2002) Prognostic stratification and long-term follow-up of optimally treated patients with ischemic cardiomyopathy. The Trieste HF Registry (abstract). Eur J Heart Fail 1(Suppl 1):18Google Scholar
  9. 9.
    Di Lenarda A, Sinagra G, Sabbadini G et al (2001) Long-term follow-up in asymptomatic dilated cardiomyopathy treated with beta-blockers. The Heart Muscle Disease Registry of Trieste (abstract) Eur Heart J 22(Suppl): 637Google Scholar
  10. 10.
    Michels VV, Moll PP, Miller FA et al (1992) The frequency of familial dilated cardiomyopathy in a series of patients with idiopathic dilated cardiomyopathy. N Engl J Med 326:77–82PubMedCrossRefGoogle Scholar
  11. 11.
    Grunig E, Tasman JA, Kucherer H et al (1998) Frequency and phenotypes of familial dilated cardiomyopathy. J Am Coll Cardiol 31:186–94PubMedCrossRefGoogle Scholar
  12. 12.
    Gregori D, Rocco C, Miocic S, Mestroni L (2001) Estimating the frequency of familial dilated cardiomyopathy in the presence of misclassification errors. J Appl Stat 28:53–62CrossRefGoogle Scholar
  13. 13.
    Sinagra G, Di Lenarda A, Brodsky GL et al for the Heart Muscle Disease Study Group (2001) New insights into the molecular basis of familial dilated cardiomyopathy. Ital Heart J 2:280–6PubMedGoogle Scholar
  14. 14.
    Kamisago M, Sharma SD, De Palma SR et al (2000) Mutations in sarcomere protein genes as a cause of dilated cardiomyopathy. N Engl J Med 343:1688–96PubMedCrossRefGoogle Scholar
  15. 15.
    Brodsky GL, Muntoni F, Miocic S et al (2000) A lamin A/C gene mutation associated with dilated cardiomyopathy with variable skeletal muscle involvement. Circulation 101:473–6PubMedGoogle Scholar
  16. 16.
    Jacobs PM, Hanson EL, Crispell KA et al (2001) Novel lamin A/C gene mutations in two families with dilated cardiomyopathy and conduction system disease. J Card Fail 7:249–56CrossRefGoogle Scholar
  17. 17.
    Arbustini E, Pilotto A, Repetto A et al (2002) Autosomal dominant dilated cardiomyopathy with atrioventricular block: a lamin A/C defect-related disease. J Am Coll Cardiol 39:981–90PubMedCrossRefGoogle Scholar
  18. 18.
    Taylor MR, Fain PR, Sinagra G et al (2003) Natural history of dilated cardiomyopathy due to lamin A/C gene mutations. J Am Coll Cardiol 41:771–80PubMedCrossRefGoogle Scholar
  19. 19.
    Appleton CP, Hatle LK, Popp RL (1988) Relation of transmitral flow velocity patterns to left ventricular diastolic function: new insights from a combined hemodynamic and Doppler echocardiographic study. J Am Coll Cardiol 12:426–40PubMedGoogle Scholar
  20. 20.
    Giannuzzi P, Imparato A, Temporelli PL et al (1994) Doppler-derived mitral deceleration time of early filling as a strong predictor of pulmonary capillary wedge pressure in postinfarction patients with left ventricular systolic dysfunction. J Am Coll Cardiol 23:1630–7PubMedCrossRefGoogle Scholar
  21. 21.
    Pozzoli M, Capomolla S, Pinna G et al (1996) Doppler echocardiography reliably predicts pulmonary artery wedge pressure in patients with chronic HF with and without mitral regurgitation. J Am Coll Cardiol 27:883–93PubMedCrossRefGoogle Scholar
  22. 22.
    Vanoverschelde JL, Raphael DA, Robert AR et al (1990) Left ventricular filling in dilated cardiomyopathy: relation to functional class and hemodynamics. J Am Coll Cardiol 15:1288–95PubMedGoogle Scholar
  23. 23.
    Tabet JY, Logeart D, Geyer C et al (2000) Comparison of the prognostic value of left ventricular filling and peak oxygen uptake in patients with systolic HF. Eur Heart J 21:1864–71PubMedCrossRefGoogle Scholar
  24. 24.
    Pinamonti B, Di Lenarda A, Sinagra G et al (1993) Restrictive left ventricular filling pattern in dilated cardiomyopathy assessed by Doppler echocardiography: clinical, echocardiographic and hemodynamic correlations and prognostic implications. J Am Coll Cardiol 22:808–15PubMedGoogle Scholar
  25. 25.
    Pinamonti B, Zecchin M, Di Lenarda A et al (1997) Persistence of restrictive left ventricular filling pattern in dilated cardiomyopathy: an ominous prognostic sign. J Am Coll Cardiol 29:604–12PubMedCrossRefGoogle Scholar
  26. 26.
    Traversi E, Pozzoli M, Cioffi G et al (1996) Mitral flow velocity changes after 6 months of optimized therapy provide important hemodynamic and prognostic information in patients with chronic HF. Am Heart J 132:809–19PubMedCrossRefGoogle Scholar
  27. 27.
    Temporelli PL, Corra U, Imparato A et al (1998) Reversible restrictive left ventricular diastolic filling with optimized oral therapy predicts a more favorable prognosis in patients with chronic HF. J Am Coll Cardiol 31:1591–7PubMedCrossRefGoogle Scholar
  28. 28.
    Hofmann T, Meinertz T, Kasper W et al (1988) Mode of death in idiopathic dilated cardiomyopathy: multivariate analysis of prognostic determinants. Am Heart J 116:1455–63PubMedCrossRefGoogle Scholar
  29. 29.
    Meinertz T, Hofmann T, Kasper W et al (1984) Significance of ventricular arrhythmias in idiopathic dilated cardiomyopathy. Am J Cardiol 53:902–7PubMedCrossRefGoogle Scholar
  30. 30.
    Diaz RA, Obasohan A, Oakley CM (1987) Prediction of outcome in dilated cardiomyopathy. Br Heart J 58:393–9PubMedGoogle Scholar
  31. 31.
    Keogh AM, Baron DW, Hickie JB. (1990) Prognostic guides in patients with idiopathic or ischemic dilated cardiomyopathy assessed for cardiac transplantation. Am J Cardiol 65:903–8PubMedCrossRefGoogle Scholar
  32. 32.
    Morgera T, Di Lenarda A, Rakar S et al (2002) Substrate and prognostic impact of ventricular tachycardia in dilated cardiomyopathy (abstract). Eur J Heart Fail 1(Suppl 1):63Google Scholar
  33. 33.
    Caruso AC, Marcus FI, Hahn EA et al (1997) Predictors of arrhythmic death and cardiac arrest in the ESVEM trial. Circulation 96:1888–92PubMedGoogle Scholar
  34. 34.
    Cohn JN, Johnson G, Ziesche S et al (1991) A comparison of enalapril with hydralazine-isosorbide dinitrate in the treatment of chronic congestive HF. N Engl J Med 325:303–10PubMedCrossRefGoogle Scholar
  35. 35.
    Grimm W, Hoffmann JJ, Muller HH et al (2002) Implantable defibrillator event rates in patients with idiopathic dilated cardiomyopathy, nonsustained ventricular tachycardia on Holter and a left ventricular ejection fraction below 30%. J Am Coll Cardiol 39:780–7PubMedCrossRefGoogle Scholar
  36. 36.
    Zecchin M, Di Lenarda A, Bonin M et al (2001) Incidence and predictors of sudden cardiac death during long term follow-up in patients with dilated cardiomyopathy on optimal medical therapy. Ital Heart J 2:213–21PubMedGoogle Scholar
  37. 37.
    The Digitalis Investigation Group (1997) The effect of digoxin on mortality and morbidity in patients with HF. N Engl J Med 336:525–33CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2007

Authors and Affiliations

  • M. Moretti
    • 1
  • A. Di Lenarda
    • 1
  • G. Sinagra
    • 1
  1. 1.Cardiovascular Department“Ospedali Riuniti” and University of TriesteTriesteItaly

Personalised recommendations