Features of the Clinical Courses and Life Prognosis of Patients with the Familial Form of Dilated Cardiomyopathy

Nuraly A. Kurbanov, PhD*, Temur A. Abdullaev, PhD, ScD, Ravshanbek D. Kurbanov PhD, ScD

Republican Specialized Center of Cardiology, Tashkent, Uzbekistan

*Corresponding author: Nuraly A. Kurbanov, PhD, Republican Specialized Center of Cardiology,

4 Osiyo str., 100052, Tashkent, Uzbekistan. Tel: 998-97-3332812


Purpose: To study the prevalence, clinico-hemodynamic characteristics and long-term prognosis of patients with the familial form of DCM. Material and methods: Over the period between 2000 and 2009, 221 patients with DCM were examined. Of these, 27 (12.2% of 221) cases of familial cardiomyopathy were diagnosed, based on anamnesis and clinico-functional examination. Subsequently, for comparative assessment, two groups were identified: Group I had 27 patients with familial DCM and Group II included 77 patients with the sporadic form. All the patients underwent ECG, ECG by Holter, a 6-minute walking test, roentgencardiometry, coronary angiography and their life prognosis were estimated. Results: In this study, it was established that the prevalence of familial DCM totaled to 12.2% versus the non-familial form, was associated with a much younger age group, in 1/3 cases was transmitted through the maternal line, and some patients were characterized by atrioventricular heart block. Conclusion: An analysis of near-term prognosis of the patients has revealed that the familial form and age below 30 years are associated with a rapid progression of the clinical course, coupled with increasing mortality in the first 12 months of follow-up.

familial form of DCM; clinico-hemodynamic characteristics; prognosis.
  1. Csonady M, Hogya M, Kallai A. Familial dilated cardiomyopathy:  a worse prognosis compared with sporadic forms.  British Heart J 1995; 74 (2):171-173.
  2. Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008;29:270-276.  
  3. Arbustini E, PilottoA, Repetto A, et al. Autosomal dominant dilated cardiomyopathy with atrioventricular block: a lamin  A/C  defect –related disease. J Am Coll Cardiol 2002; 39: 981-990
  4. Keeling PJ, Gang Y, Smith G, et  al. Familial dilated cardiomyopathy in the United Kingdom. Br. Heart J 1995; 73:417-421.
  5. Grunig E, Tasman JA, Kucherer H, et al. Frequency and phenotypes of familial dilated cardiomyopathy.  J Am Coll Cardiol 1998; 31:186-19.
  6. Osterziel KJ, Scheffold T, Perrot A,  et   al. Genetics of dilated cardiomyopathy. Z Kardiol 2001; 90 (7): 461-469.
  7.  Мestroni  L, Rocco C.  Advances in molecular genetics of dilated cardiomyopathy. Cardiology Clinics 1998; 16: 603-609.
  8.  Richardson P, McKenna W, Bristow M, et al.  Report of the 1995 World Organization. International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation 1996; 93:841-842.
  9.  Bahler RC. Assessment of prognosis in idiopathic dilated cardiomyopathy. Chest 2002; 121:1016-1019.
  10. Moretti M. Merlo M, Barbati G, et al.  Prognostic Value of a Systematic Familial Screening in Idiopathic Dilated Cardiomyopathy: The Experience of Trieste Heart Muscle Disease. Circulation 2009; 120: S904
  11.  Maron B, Towbin J, Thiene G, et al. Contemporal definitions and classifications of the cardiomyopathies. Circulation 2006; 113: 1807-1816.
  12.  Li D, Tapscoft T, Gonzalez O, et al. Desmin mutation responsible for idiopathic dilated cardiomyopathy. Circulation 1999; 100:46-4.
  13.  Valantine HA, Hunt SA, Fowler MB, et al. Frequency of familial nature of dilated cardiomyopathy and usefulness of cardiac transplantation in this subset. Am J Cardiol 1989; 63: 959-63.
  14.  Zachara E, Caforio AL, Carboni GP, et al. Familial aggregation of idiopathic dilated cardiomyopathy: clinical features and pedigree analysis in 14 families. Br Heart J 1993; 69: 129-35.
  15.  Gavazzi A, De Maria R, Renosto G, et al. The spectrum of left ventricular size in dilated cardiomyopathy: clinical correlates and prognostic implications. SPIC (Italian Multicenter Cardiomyopathy Study) Group. Am Heart J 1993; 125:410 – 22.
  16.  Graber HL, Unverferth DV, Baker PB, et al. Evolution of a hereditary cardiac conduction and muscle disorder: a study involving a family with six generations affected. Circulation 1986; 74: 21-35.  
  17. Michels VV, Driscoll DJ, Miller FA, et al. Progression of familial and non-familial dilated cardiomyopathy: long-term follow-up. Heart 2003; 89:757-761.

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 Int J Biomed. 2011; 1(3):139-142. © 2011 International Medical Research and Development Corporation. All rights reserved.