Current Heart Failure Reports

, 5:238

Management of peripartum cardiomyopathy

  • Karen Sliwa
  • Kemi Tibazarwa
  • Denise Hilfiker-Kleiner


Peripartum cardiomyopathy (PPCM) is a form of heart failure that occurs in women within 1 month of predelivery and 5 months postdelivery. Echocardiography demonstrates features of cardiomyopathy with impaired ejection fraction; global dilatation and thinned-out walls are sometimes present. The symptoms and signs of PPCM are similar to those in patients with idiopathic dilated cardiomyopathy. The acute form of PPCM is a clinical syndrome, with reduced cardiac output, tissue hypoperfusion, and increase in the pulmonary capillary wedge pressure. Monitoring of the patient with the acute form of PPCM should be initiated as soon as possible. The types and levels of monitoring required for an individual patient vary widely depending on the severity of the cardiac decompensation and response to initial therapy. The syndrome carries a high morbidity and mortality, and diagnosis is often delayed. This review summarizes recent data charting the incidence, recent advances in the understanding of the pathophysiology of PPCM, and outlines the current treatment options available.

References and Recommended Reading

  1. 1.
    Pearson GD, Veille JC, Rahimtoola S, et al.: Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. JAMA 2000, 283:1183–1188.PubMedCrossRefGoogle Scholar
  2. 2.
    Sliwa K, Fett J, Elkayam U: Peripartum cardiomyopathy. Lancet 2006, 368:687–693.PubMedCrossRefGoogle Scholar
  3. 3.
    Elkayam U, Akhter MW, Singh H, et al.: Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005, 111:2050–2055.PubMedCrossRefGoogle Scholar
  4. 4.
    Chapa JB, Heiberger HB, Weinert L, et al.: Prognostic value of echocardiography in peripartum cardiomyopathy. Obstet Gynecol 2005, 105:1303–1308.PubMedGoogle Scholar
  5. 5.
    Kist WJ, Janssen NG, Kalk JJ, et al.: Thrombophilias and adverse pregnancy outcome—a confounded problem! Thromb Haemost 2008, 99:77–85.PubMedGoogle Scholar
  6. 6.
    Maron BJ, Towbin JA, Thiene G, et al.: Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups, and Council on Epidemiology and Prevention. Circulation 2006, 113:1807–1816.PubMedCrossRefGoogle Scholar
  7. 7.
    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.PubMedCrossRefGoogle Scholar
  8. 8.
    Deneux-Tharaux C, Berg C, Bouvier-Colle MH, et al.: Underreporting of pregnancy-related mortality in the United States or Europe. Obstet Gynecol 2005, 106:684–692. [Published erratum appears in Obstet Gynecol 2006, 107:209.]PubMedGoogle Scholar
  9. 9.
    Brar SS, Khan SS, Sandhu GK, et al.: Incidence, mortality, and racial differences in peripartum cardiomyopathy. Am J Cardiol 2007, 100:302–304.PubMedCrossRefGoogle Scholar
  10. 10.
    Hayakawa Y, Chandra M, Miao W, et al.: Inhibition of cardiac myocyte apoptosis improves cardiac function and abolishes mortality in the peripartum cardiomyopathy of Galpha(q) transgenic mice. Circulation 2003, 108:3036–3041.PubMedCrossRefGoogle Scholar
  11. 11.
    Hilfiker-Kleiner D, Kaminski K, Podewski E, et al.: A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum cardiomyopathy. Cell 2007, 128:589–600.PubMedCrossRefGoogle Scholar
  12. 12.
    Forster O, Hilfiker-Kleiner D, Ansari A, et al.: Reversal of IFN-gamma, oxLDL and prolactin serum levels correlate with clinical improvement in patients with peripartum cardiomyopathy. Eur J Heart Fail 2008, 10:861–868.PubMedCrossRefGoogle Scholar
  13. 13.
    Diwan A, Wansapura J, Syed FM, et al.: Nix-mediated apoptosis links myocardial fibrosis, cardiac remodeling, and hypertrophy decompensation. Circulation 2008, 117:396–404.PubMedCrossRefGoogle Scholar
  14. 14.
    Fett JD, Ansari AA, Sundstrom JB, Combs GF: Peripartum cardiomyopathy: a selenium disconnection and an autoimmune connection. Int J Cardiol 2002, 86:311–316.PubMedCrossRefGoogle Scholar
  15. 15.
    Lampert MB, Lang RM: Peripartum cardiomyopathy. Am Heart J 1995, 130:860–870.PubMedCrossRefGoogle Scholar
  16. 16.
    Sliwa K, Skudicky D, Bergemann A, et al.: Peripartum cardiomyopathy: analysis of clinical outcome, left ventricular function, plasma levels of cytokines and Fas/APO-1. J Am Coll Cardiol 2000, 35:701–705.PubMedCrossRefGoogle Scholar
  17. 17.
    Sliwa K, Skudicky D, Candy G, et al.: The addition of pentoxifylline to conventional therapy improves outcome in patients with peripartum cardiomyopathy. Eur J Heart Fail 2002, 4:305–309.PubMedCrossRefGoogle Scholar
  18. 18.
    Forster O, Ansari AA, Sliwa K: Current issues in diagnosis and management of peripartum cardiomyopathy. Womens Health 2006, 2:587–596.Google Scholar
  19. 19.
    Nieminen MS, Bohm M, Cowie MR, et al.: Executive summary of the guidelines on the diagnosis and treatment of acute heart failure: the Task Force on Acute Heart Failure of the European Society of Cardiology. Eur Heart J 2005, 26:384–416.PubMedCrossRefGoogle Scholar
  20. 20.
    Sliwa K, Forster O, Libhaber E, et al.: Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients. Eur Heart J 2006, 27:441–446.PubMedCrossRefGoogle Scholar
  21. 21.
    Bultmann BD, Klingel K, Nabauer M, et al.: High prevalence of viral genomes and inflammation in peripartum cardiomyopathy. Am J Obstet Gynecol 2005, 193:363–365.PubMedCrossRefGoogle Scholar
  22. 22.
    Cenac A, Djibo A, Djangnikpo L: Peripartum dilated cardiomyopathy. A model of multifactor disease? [in French]. Rev Med Interne 1993, 14:1033.PubMedGoogle Scholar
  23. 23.
    Fett JD: Viral infection as a possible trigger for the development of peripartum cardiomyopathy. Int J Gynaecol Obstet 2007, 97:149–150.PubMedCrossRefGoogle Scholar
  24. 24.
    Rizeq MN, Rickenbacher PR, Fowler MB, Billingham ME: Incidence of myocarditis in peripartum cardiomyopathy. Am J Cardiol 1994, 74:474–477.PubMedCrossRefGoogle Scholar
  25. 25.
    Harrison RG: Suppression of lactation. Semin Perinatol 1979, 3:287–297.PubMedGoogle Scholar
  26. 26.
    Nagafuchi H, Suzuki N, Kaneko A, et al.: Prolactin locally produced by synovium infiltrating T lymphocytes induces excessive synovial cell functions in patients with rheumatoid arthritis. J Rheumatol 1999, 26:1890–1900.PubMedGoogle Scholar
  27. 27.
    Kothari SS: Aetiopathogenesis of peripartum cardiomyopathy: prolactin-selenium interaction? Int J Cardiol 1997, 60:111–114.PubMedCrossRefGoogle Scholar
  28. 28.
    Peter JV, Moran JL, Phillips-Hughes J, et al.: Effect of noninvasive positive pressure ventilation (NIPPV) on mortality in patients with acute cardiogenic pulmonary oedema: a meta-analysis. Lancet 2006, 367:1155–1163.PubMedCrossRefGoogle Scholar
  29. 29.
    Nieminen MS, Böm M, Cowie MR, et al.: Executive summary of the guidelines on the diagnosis and treatment of acute heart failure: the Task Force on Acute Heart Failure of the European Society of Cardiology. Eur Heart J 2005, 26:384–416.PubMedCrossRefGoogle Scholar
  30. 30.
    Johnson MR, Naftel DC, Hobbs RE, et al.: The incremental risk of female sex in heart transplantation: a multiinstitutional study of peripartum cardiomyopathy and pregnancy. Cardiac Transplant Research Database Group. J Heart Lung Transplant 1997, 16:801–812.PubMedGoogle Scholar
  31. 31.
    Hovsepian PG, Ganzel B, Sohi GS, et al.: Peripartum cardiomyopathy treated with a left ventricular assist device as a bridge to cardiac transplantation. South Med J 1989, 82:527–528.PubMedGoogle Scholar
  32. 32.
    Hilfiker-Kleiner D, Meyer GP, Schieffer E, et al.: Recovery from postpartum cardiomyopathy in 2 patients by blocking prolactin release with bromocriptine. J Am Coll Cardiol 2007, 50:2354–2355.PubMedCrossRefGoogle Scholar
  33. 33.
    Rathore SS, Wang Y, Krumholz HM: Sex-based differences in the effect of digoxin for the treatment of heart failure. N Engl J Med 2002, 347:1403–1411.PubMedCrossRefGoogle Scholar
  34. 34.
    Adams KF Jr, Patterson JH, Gattis WA, et al.: Relationship of serum digoxin concentration to mortality and morbidity in women in the digitalis investigation group trial: a retrospective analysis. J Am Coll Cardiol 2005, 46:497–504.PubMedCrossRefGoogle Scholar
  35. 35.
    Fett JD, Christie LG, Carraway RD, Murphy JG: Five-year prospective study of the incidence and prognosis of peripartum cardiomyopathy at a single institution. Mayo Clin Proc 2005, 80:1602–1606.PubMedGoogle Scholar
  36. 36.
    Felker GM, Thompson RE, Hare JM, et al.: Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. N Engl J Med 2000, 342:1077–1084.PubMedCrossRefGoogle Scholar
  37. 37.
    Ellis JE, Ansari AA, Fett JD, et al.: Inhibition of progenitor dendritic cell maturation by plasma from patients with peripartum cardiomyopathy: role in pregnancy-associated heart disease. Clin Dev Immunol 2005, 12:265–273.PubMedCrossRefGoogle Scholar
  38. 38.
    Sliwa K, Forster O, Zhanje F, et al.: Outcome of subsequent pregnancy in patients with documented peripartum cardiomyopathy. Am J Cardiol 2004, 93:1441–1443.PubMedCrossRefGoogle Scholar
  39. 39.
    Elkayam U, Tummala PP, Rao K, et al.: Maternal and fetal outcomes of subsequent pregnancies in women with peripartum cardiomyopathy. N Engl J Med 2001, 344:1567–1571. [Published erratum appears in N Engl J Med 2001, 345:552.]PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Karen Sliwa
    • 1
  • Kemi Tibazarwa
  • Denise Hilfiker-Kleiner
  1. 1.Soweto Cardiovascular Research UnitChris Hani Baragwanath HospitalJohannesburgSouth Africa

Personalised recommendations