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Peripartum cardiomyopathy: Etiology, clinical manifestations, and diagnosis

Wendy Tsang, MD
Amy C Bales, MD
Roberto M Lang, MD
Section Editor
Candice Silversides, MD, MS, FRCPC
Deputy Editor
Susan B Yeon, MD, JD, FACC


Peripartum cardiomyopathy (PPCM, also called pregnancy-associated cardiomyopathy) is a rare cause of heart failure (HF) that affects women late in pregnancy or in the early puerperium [1]. Although initially described in 1849 [2], it was not recognized as a distinct clinical entity until the 1930s [3]. Earlier terms for this condition include toxic postpartum HF, Meadows’ syndrome, Zaria syndrome, and postpartum myocardiosis.

This topic will discuss the etiology, clinical manifestations, and diagnosis of PPCM. Treatment and prognosis of PPCM, critical illness during pregnancy and the peripartum period, HF during pregnancy, and issues related to pregnancy in women with acquired or congenital heart disease are discussed separately. (See "Peripartum cardiomyopathy: Treatment and prognosis" and "Critical illness during pregnancy and the peripartum period" and "Management of heart failure during pregnancy" and "Acquired heart disease and pregnancy" and "Pregnancy in women with congenital heart disease: General principles".)


A variety of definitions have been used to identify peripartum cardiomyopathy (PPCM) [1,4-7]. We favor the definition developed by the 2010 European Society of Cardiology (ESC) Working Group on Peripartum Cardiology [1]. The Working Group sought to avoid under-diagnosis of PPCM by adopting a broad definition (which was also included in the 2011 ESC guidelines on management of cardiovascular diseases during pregnancy) [1,7].

The 2010 ESC Working Group defined PPCM as an idiopathic cardiomyopathy with the following characteristics:

Development of heart failure (HF) toward the end of pregnancy or in the months following delivery

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Literature review current through: Nov 2017. | This topic last updated: Feb 26, 2016.
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  1. Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail 2010; 12:767.
  2. Richie C. Clinical contribution to the pathology, diagnosis and treatment of certain chronic diseases of the heart. Edinb Med Surg J 1849; 2:333.
  3. Hafkesbring E, Hull E. "Toxic" postpartal heart disease. New Orleans Med Surg J 1937; 89:550.
  4. Demakis JG, Rahimtoola SH, Sutton GC, et al. Natural course of peripartum cardiomyopathy. Circulation 1971; 44:1053.
  5. 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.
  6. Hibbard JU, Lindheimer M, Lang RM. A modified definition for peripartum cardiomyopathy and prognosis based on echocardiography. Obstet Gynecol 1999; 94:311.
  7. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J 2011; 32:3147.
  8. 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.
  9. SEFTEL H, SUSSER M. Maternity and myocardial failure in African women. Br Heart J 1961; 23:43.
  10. Davidson NM, Parry EH. The etiology of peripartum cardiac failure. Am Heart J 1979; 97:535.
  11. WOOLFORD RM. Postpartum myocardosis. Ohio State Med J 1952; 48:924.
  12. PIERCE JA, PRICE BO, JOYCE JW. Familial occurrence of postpartal heart failure. Arch Intern Med 1963; 111:651.
  13. Sliwa K, Damasceno A, Mayosi BM. Epidemiology and etiology of cardiomyopathy in Africa. Circulation 2005; 112:3577.
  14. Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995; 130:860.
  15. 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.
  16. Sliwa K, Böhm M. Incidence and prevalence of pregnancy-related heart disease. Cardiovasc Res 2014; 101:554.
  17. Sanderson JE, Adesanya CO, Anjorin FI, Parry EH. Postpartum cardiac failure--heart failure due to volume overload? Am Heart J 1979; 97:613.
  18. Fillmore SJ, Parry EH. The evolution of peripartal heart failure in Zaria, Nigeria. Some etiologic factors. Circulation 1977; 56:1058.
  19. Patten IS, Rana S, Shahul S, et al. Cardiac angiogenic imbalance leads to peripartum cardiomyopathy. Nature 2012; 485:333.
  20. Halkein J, Tabruyn SP, Ricke-Hoch M, et al. MicroRNA-146a is a therapeutic target and biomarker for peripartum cardiomyopathy. J Clin Invest 2013; 123:2143.
  21. Bello N, Rendon IS, Arany Z. The relationship between pre-eclampsia and peripartum cardiomyopathy: a systematic review and meta-analysis. J Am Coll Cardiol 2013; 62:1715.
  22. Steegers EA, von Dadelszen P, Duvekot JJ, Pijnenborg R. Pre-eclampsia. Lancet 2010; 376:631.
  23. Sliwa K, Mebazaa A. Possible joint pathways of early pre-eclampsia and congenital heart defects via angiogenic imbalance and potential evidence for cardio-placental syndrome. Eur Heart J 2014; 35:680.
  24. Haghikia A, Podewski E, Libhaber E, et al. Phenotyping and outcome on contemporary management in a German cohort of patients with peripartum cardiomyopathy. Basic Res Cardiol 2013; 108:366.
  25. Kamiya CA, Kitakaze M, Ishibashi-Ueda H, et al. Different characteristics of peripartum cardiomyopathy between patients complicated with and without hypertensive disorders. -Results from the Japanese Nationwide survey of peripartum cardiomyopathy-. Circ J 2011; 75:1975.
  26. 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.
  27. Podewski EK, Hilfiker-Kleiner D, Hilfiker A, et al. Alterations in Janus kinase (JAK)-signal transducers and activators of transcription (STAT) signaling in patients with end-stage dilated cardiomyopathy. Circulation 2003; 107:798.
  28. Ricke-Hoch M, Bultmann I, Stapel B, et al. Opposing roles of Akt and STAT3 in the protection of the maternal heart from peripartum stress. Cardiovasc Res 2014; 101:587.
  29. 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.
  30. Sliwa K, Förster O, Libhaber E, et al. Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients. Eur Heart J 2006; 27:441.
  31. Melvin KR, Richardson PJ, Olsen EG, et al. Peripartum cardiomyopathy due to myocarditis. N Engl J Med 1982; 307:731.
  32. Sanderson JE, Olsen EG, Gatei D. Peripartum heart disease: an endomyocardial biopsy study. Br Heart J 1986; 56:285.
  33. O'Connell JB, Costanzo-Nordin MR, Subramanian R, et al. Peripartum cardiomyopathy: clinical, hemodynamic, histologic and prognostic characteristics. J Am Coll Cardiol 1986; 8:52.
  34. Midei MG, DeMent SH, Feldman AM, et al. Peripartum myocarditis and cardiomyopathy. Circulation 1990; 81:922.
  35. Bültmann BD, Klingel K, Näbauer M, et al. High prevalence of viral genomes and inflammation in peripartum cardiomyopathy. Am J Obstet Gynecol 2005; 193:363.
  36. Rizeq MN, Rickenbacher PR, Fowler MB, Billingham ME. Incidence of myocarditis in peripartum cardiomyopathy. Am J Cardiol 1994; 74:474.
  37. Nelson JL. Pregnancy, persistent microchimerism, and autoimmune disease. J Am Med Womens Assoc (1972) 1998; 53:31.
  38. Ansari AA, Fett JD, Carraway RE, et al. Autoimmune mechanisms as the basis for human peripartum cardiomyopathy. Clin Rev Allergy Immunol 2002; 23:301.
  39. Cénac A, Beaufils H, Soumana I, et al. Absence of humoral autoimmunity in peripartum cardiomyopathy. A comparative study in Niger. Int J Cardiol 1990; 26:49.
  40. van Spaendonck-Zwarts KY, van Tintelen JP, van Veldhuisen DJ, et al. Peripartum cardiomyopathy as a part of familial dilated cardiomyopathy. Circulation 2010; 121:2169.
  41. Morales A, Painter T, Li R, et al. Rare variant mutations in pregnancy-associated or peripartum cardiomyopathy. Circulation 2010; 121:2176.
  42. van Spaendonck-Zwarts KY, Posafalvi A, van den Berg MP, et al. Titin gene mutations are common in families with both peripartum cardiomyopathy and dilated cardiomyopathy. Eur Heart J 2014; 35:2165.
  43. Ware JS, Li J, Mazaika E, et al. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies. N Engl J Med 2016; 374:233.
  44. Veille JC. Peripartum cardiomyopathies: a review. Am J Obstet Gynecol 1984; 148:805.
  45. Homans DC. Peripartum cardiomyopathy. N Engl J Med 1985; 312:1432.
  46. Mendelson MA, Chandler J. Postpartum cardiomyopathy associated with maternal cocaine abuse. Am J Cardiol 1992; 70:1092.
  47. Lampert MB, Hibbard J, Weinert L, et al. Peripartum heart failure associated with prolonged tocolytic therapy. Am J Obstet Gynecol 1993; 168:493.
  48. Kothari SS. Aetiopathogenesis of peripartum cardiomyopathy: prolactin-selenium interaction? Int J Cardiol 1997; 60:111.
  49. Cénac A, Simonoff M, Moretto P, Djibo A. A low plasma selenium is a risk factor for peripartum cardiomyopathy. A comparative study in Sahelian Africa. Int J Cardiol 1992; 36:57.
  50. Fett JD, Ansari AA, Sundstrom JB, Combs GF. Peripartum cardiomyopathy: a selenium disconnection and an autoimmune connection. Int J Cardiol 2002; 86:311.
  51. Amos AM, Jaber WA, Russell SD. Improved outcomes in peripartum cardiomyopathy with contemporary. Am Heart J 2006; 152:509.
  52. Chapa JB, Heiberger HB, Weinert L, et al. Prognostic value of echocardiography in peripartum cardiomyopathy. Obstet Gynecol 2005; 105:1303.
  53. Goland S, Modi K, Hatamizadeh P, Elkayam U. Differences in clinical profile of African-American women with peripartum cardiomyopathy in the United States. J Card Fail 2013; 19:214.
  54. Lee W. Clinical management of gravid women with peripartum cardiomyopathy. Obstet Gynecol Clin North Am 1991; 18:257.
  55. Grewal J, Siu SC, Ross HJ, et al. Pregnancy outcomes in women with dilated cardiomyopathy. J Am Coll Cardiol 2009; 55:45.
  56. Desai D, Moodley J, Naidoo D. Peripartum cardiomyopathy: experiences at King Edward VIII Hospital, Durban, South Africa and a review of the literature. Trop Doct 1995; 25:118.
  57. Kane A, Mbaye M, Ndiaye MB, et al. [Evolution and thromboembolic complications of the idiopathic peripartal cardiomyopathy at Dakar University Hospital: forward-looking study about 33 cases]. J Gynecol Obstet Biol Reprod (Paris) 2010; 39:484.
  58. Simeon IA. Echocardiographic profile of peripartum cardiomyopathy in a tertiary care hospital in sokoto, Nigeria. Indian Heart J 2006; 58:234.
  59. Mandal D, Mandal S, Mukherjee D, et al. Pregnancy and subsequent pregnancy outcomes in peripartum cardiomyopathy. J Obstet Gynaecol Res 2011; 37:222.
  60. Forster O, Hilfiker-Kleiner D, Ansari AA, 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.
  61. Blauwet LA, Cooper LT. Diagnosis and management of peripartum cardiomyopathy. Heart 2011; 97:1970.
  62. Fett JD. The role of MRI in peripartum cardiomyopathy. Int J Cardiol 2009; 137:185.
  63. Baruteau AE, Leurent G, Martins RP, et al. Peripartum cardiomyopathy in the era of cardiac magnetic resonance imaging: first results and perspectives. Int J Cardiol 2010; 144:143.
  64. Leurent G, Baruteau AE, Larralde A, et al. Contribution of cardiac MRI in the comprehension of peripartum cardiomyopathy pathogenesis. Int J Cardiol 2009; 132:e91.
  65. Kawano H, Tsuneto A, Koide Y, et al. Magnetic resonance imaging in a patient with peripartum cardiomyopathy. Intern Med 2008; 47:97.
  66. Caballero-Borrego J, García-Pinilla JM, Rueda-Calle E, de Teresa-Galván E. [Evidence of gadolinium late-enhancement on cardiac magnetic resonance imaging in a patient with peripartum cardiomyopathy]. Rev Esp Cardiol 2008; 61:219.
  67. Mouquet F, Lions C, de Groote P, et al. Characterisation of peripartum cardiomyopathy by cardiac magnetic resonance imaging. Eur Radiol 2008; 18:2765.
  68. Marmursztejn J, Vignaux O, Goffinet F, et al. Delayed-enhanced cardiac magnetic resonance imaging features in peripartum cardiomyopathy. Int J Cardiol 2009; 137:e63.
  69. Barone-Rochette G, Rodière M, Lantuejoul S. Value of cardiac MRI in peripartum cardiomyopathy. Arch Cardiovasc Dis 2011; 104:263.