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. 1997 Jun;77(6):549–552. doi: 10.1136/hrt.77.6.549

Idiopathic dilated cardiomyopathy: familial prevalence and HLA distribution.

C J McKenna 1, M B Codd 1, H A McCann 1, D D Sugrue 1
PMCID: PMC484799  PMID: 9227300

Abstract

OBJECTIVES: To compare HLA distribution in familial and non-familial dilated cardiomyopathy, because a serum marker that could identify families at risk of developing dilated cardiomyopathy should be of use in screening for the disease. PATIENTS: 100 patients with dilated cardiomyopathy. METHODS: 200 first degree relatives from 56 of the proband families were screened for dilated cardiomyopathy by echocardiography. The HLA profile of the patients with dilated cardiomyopathy, as well as of the familial and non-familial subgroups, was compared with that of 9000 normal controls. RESULTS: The familial prevalence of dilated cardiomyopathy in this patient group was "definite" in 14 of 56 (25%) and "possible" in 25 of 56 (45%). The HLA-DR4 frequency in the 100 patients with dilated cardiomyopathy was similar to that in the 9000 controls (39% v 32%). However, the DR4 subtype was significantly more common in the 25 probands with a familial tendency to dilated cardiomyopathy than in the 31 probands with non-familial dilated cardiomyopathy (68% v 32%; P < 0.05). CONCLUSIONS: The present finding supports an HLA linked predisposition to familial dilated cardiomyopathy. The HLA type DR4 was significantly more common in familial than in non-familial cases. The DR4 halotype was associated with two thirds of the families at risk for dilated cardiomyopathy.

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Selected References

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  1. Anderson J. L., Carlquist J. F., Lutz J. R., DeWitt C. W., Hammond E. H. HLA A, B and DR typing in idiopathic dilated cardiomyopathy: a search for immune response factors. Am J Cardiol. 1984 May 1;53(9):1326–1330. doi: 10.1016/0002-9149(84)90088-2. [DOI] [PubMed] [Google Scholar]
  2. Arbustini E., Gavazzi A., Pozzi R., Grasso M., Pucci A., Campana C., Graziano G., Martinetti M., Cuccia M., Salvaneschi L. The morphologic spectrum of dilated cardiomyopathy and its relation to immune-response genes. Am J Cardiol. 1989 Nov 1;64(16):991–995. doi: 10.1016/0002-9149(89)90796-0. [DOI] [PubMed] [Google Scholar]
  3. Ashall F., Bramwell M. E., Harris H. A new marker for human cancer cells. 1 The Ca antigen and the Ca1 antibody. Lancet. 1982 Jul 3;2(8288):1–6. doi: 10.1016/s0140-6736(82)91150-3. [DOI] [PubMed] [Google Scholar]
  4. Caforio A. L., Bonifacio E., Stewart J. T., Neglia D., Parodi O., Bottazzo G. F., McKenna W. J. Novel organ-specific circulating cardiac autoantibodies in dilated cardiomyopathy. J Am Coll Cardiol. 1990 Jun;15(7):1527–1534. doi: 10.1016/0735-1097(90)92821-i. [DOI] [PubMed] [Google Scholar]
  5. Caforio A. L., Keeling P. J., Zachara E., Mestroni L., Camerini F., Mann J. M., Bottazzo G. F., McKenna W. J. Evidence from family studies for autoimmunity in dilated cardiomyopathy. Lancet. 1994 Sep 17;344(8925):773–777. doi: 10.1016/s0140-6736(94)92339-6. [DOI] [PubMed] [Google Scholar]
  6. Carlquist J. F., Menlove R. L., Murray M. B., O'Connell J. B., Anderson J. L. HLA class II (DR and DQ) antigen associations in idiopathic dilated cardiomyopathy. Validation study and meta-analysis of published HLA association studies. Circulation. 1991 Feb;83(2):515–522. doi: 10.1161/01.cir.83.2.515. [DOI] [PubMed] [Google Scholar]
  7. Goerss J. B., Michels V. V., Burnett J., Driscoll D. J., Miller F., Rodeheffer R., Tajik A. J., Schaid D. Frequency of familial dilated cardiomyopathy. Eur Heart J. 1995 Dec;16 (Suppl O):2–4. doi: 10.1093/eurheartj/16.suppl_o.2. [DOI] [PubMed] [Google Scholar]
  8. Grant S. C., Sheldon S., Dyer P. A., Levy R. D., Brooks N. H. Do specific HLA antigens predispose to ischaemic heart disease or idiopathic dilated cardiomyopathy? Br Heart J. 1994 Jan;71(1):76–78. doi: 10.1136/hrt.71.1.76. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Henry W. L., Gardin J. M., Ware J. H. Echocardiographic measurements in normal subjects from infancy to old age. Circulation. 1980 Nov;62(5):1054–1061. doi: 10.1161/01.cir.62.5.1054. [DOI] [PubMed] [Google Scholar]
  10. Herskowitz A., Ahmed-Ansari A., Neumann D. A., Beschorner W. E., Rose N. R., Soule L. M., Burek C. L., Sell K. W., Baughman K. L. Induction of major histocompatibility complex antigens within the myocardium of patients with active myocarditis: a nonhistologic marker of myocarditis. J Am Coll Cardiol. 1990 Mar 1;15(3):624–632. doi: 10.1016/0735-1097(90)90637-5. [DOI] [PubMed] [Google Scholar]
  11. Huber K. C., Gersh B. J., Sugrue D. D., Frye R. L., Bailey K. R., Ritts R. E., Jr T-lymphocyte subsets in patients with idiopathic dilated cardiomyopathy. Int J Cardiol. 1989 Jan;22(1):59–66. doi: 10.1016/0167-5273(89)90136-8. [DOI] [PubMed] [Google Scholar]
  12. Keeling P. J., Gang Y., Smith G., Seo H., Bent S. E., Murday V., Caforio A. L., McKenna W. J. Familial dilated cardiomyopathy in the United Kingdom. Br Heart J. 1995 May;73(5):417–421. doi: 10.1136/hrt.73.5.417. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Limas C. J., Limas C. HLA antigens in idiopathic dilated cardiomyopathy. Br Heart J. 1989 Nov;62(5):379–383. doi: 10.1136/hrt.62.5.379. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Limas C., Limas C. J., Boudoulas H., Bair R., Graber H., Sparks L., Wooley C. F. Anti-beta-receptor antibodies in familial cardiomyopathy: correlation with HLA-DR and HLA-DQ gene polymorphisms. Am Heart J. 1994 Feb;127(2):382–386. doi: 10.1016/0002-8703(94)90128-7. [DOI] [PubMed] [Google Scholar]
  15. Limas C., Limas C. J., Boudoulas H., Graber H., Bair R., Sparks L., Wooley C. F. T-cell receptor gene polymorphisms in familial cardiomyopathy: correlation with anti-beta-receptor autoantibodies. Am Heart J. 1992 Nov;124(5):1258–1263. doi: 10.1016/0002-8703(92)90409-o. [DOI] [PubMed] [Google Scholar]
  16. Martinetti M., Dugoujon J. M., Caforio A. L., Schwarz G., Gavazzi A., Graziano G., Arbustini E., Lorini R., McKenna W. J., Bottazzo G. F. HLA and immunoglobulin polymorphisms in idiopathic dilated cardiomyopathy. Hum Immunol. 1992 Nov;35(3):193–199. doi: 10.1016/0198-8859(92)90105-v. [DOI] [PubMed] [Google Scholar]
  17. Michels V. V., Moll P. P., Miller F. A., Tajik A. J., Chu J. S., Driscoll D. J., Burnett J. C., Rodeheffer R. J., Chesebro J. H., Tazelaar H. D. The frequency of familial dilated cardiomyopathy in a series of patients with idiopathic dilated cardiomyopathy. N Engl J Med. 1992 Jan 9;326(2):77–82. doi: 10.1056/NEJM199201093260201. [DOI] [PubMed] [Google Scholar]
  18. Michels V. V., Moll P. P., Rodeheffer R. J., Miller F. A., Jr, Tajik A. J., Burnett J. C., Jr, Driscoll D. J., Thibodeau S. N., Ansari A. A., Herskowitz A. Circulating heart autoantibodies in familial as compared with nonfamilial idiopathic dilated cardiomyopathy. Mayo Clin Proc. 1994 Jan;69(1):24–27. doi: 10.1016/s0025-6196(12)61607-3. [DOI] [PubMed] [Google Scholar]
  19. Smith J. D., Rose M. L., Pomerance A., Burke M., Yacoub M. H. Reduction of cellular rejection and increase in longer-term survival after heart transplantation after HLA-DR matching. Lancet. 1995 Nov 18;346(8986):1318–1322. doi: 10.1016/s0140-6736(95)92341-1. [DOI] [PubMed] [Google Scholar]
  20. Sugrue D. D., Holmes D. R., Jr, Gersh B. J., Edwards W. D., McLaran C. J., Wood D. L., Osborn M. J., Hammill S. C. Cardiac histologic findings in patients with life-threatening ventricular arrhythmias of unknown origin. J Am Coll Cardiol. 1984 Nov;4(5):952–957. doi: 10.1016/s0735-1097(84)80056-x. [DOI] [PubMed] [Google Scholar]

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