Peripartum Cardiomyopathy

  • Lee Anne Stratton
  • Soidjon Khodjaev
  • Srinivas MuraliEmail author


Peripartum cardiomyopathy (PPCM) is a rare form of non-ischemic dilated cardiomyopathy of unknown origin that afflicts previously healthy women of childbearing age in the last month of gestation or during the first 5 months postpartum. The incidence of PPCM is low, and there are identifiable risk factors. The clinical outcomes are variable, though generally better than other forms of dilated cardiomyopathy. Mortality ranges from 5–32% and is significantly higher in women who have more advanced disease at diagnosis. Over half of patients have improvement of ventricular function on medical treatment, though some develop progressive, severe heart failure and even sudden death. Survivors are at risk for recurrence during subsequent pregnancies and should be counseled against it unless there is complete recovery of cardiac function. Treatment of PPCM is the same as heart failure with reduced ejection fraction, and all patients should be managed using ACC/AHA guideline-based drug therapy on goal doses. Certain drugs are not recommended for patients in the antepartum and breastfeeding periods. Patients with severe heart failure may need intravenous inotropic, vasopressor or vasodilator therapy, including temporary and durable mechanical circulatory support and cardiac transplantation.


Cardiomyopathy Pregnancy Heart failure Left ventricular function Mechanical circulatory support Cardiac transplantation Left ventricular thrombus 


  1. 1.
    Lozano R, Naghavi M, Foreman K, et al. Global and regional mortality from 235 causes of death for 20 age groups in 1990 and 2010: a systematic analysis for the Global Burden of Disease Study 2010. Lancet. 2012;380(9859):2095–128. Scholar
  2. 2.
    Say L, Chou D, Gemmill A, Tunçalp Ö, Moller A, Daniels J, Gülmezoglu AM, Temmerman M, Alkema L. Global causes of maternal death: a WHO systematic analysis. Lancet Glob Health. 2014;2:e232–3. Scholar
  3. 3.
    Ntusi NBA, Badri M, Gumedze F, Sliwa K, Mayosi BM. Presentation and outcome between hypertensive heart failure of pregnancy and idiopathic peripartum cardiomyopathy. PLoS One. 2015;10(8):e0133466. Scholar
  4. 4.
    Ware JS, Li J, Mazaika E, Yasso CM, DeSouza T, Cappola TP, Tsai EJ, Hilfiker-Kleiner D, Kamiya CA, Mazzarotto F, Cook SA, Halder I, Prasad SK, Pisarcik J, Hanley-Yanez K, Alharethi R, Damp J, Hsich E, Elkayam U, Sheppard R, Kealey A, Alexis J, Ramani G, Safirstein J, Boehmer J, Pauly DF, Wittstein IS, Thohan V, Zucker MJ, Liu P, Gorscan J, McNamara DM, Seidman CE, Seidman JG, Arany Z. Shared genetic predisposition in peripartum and dilated cardiomyopathies. N Engl J Med. 2016;374(3):233–41. Scholar
  5. 5.
    Sliwa K, Bohm M. Incidence and prevalence of pregnancy-related heart disease. Cardiovasc Res. 2014;101:554–60. Scholar
  6. 6.
    Arany Z, Elkayam U. Peripartum cardiomyopathy. Circulation. 2016;133:1397–409. Scholar
  7. 7.
    Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, Ansari A, Baughman KL. Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. JAMA. 2000;283:1183–8. Scholar
  8. 8.
    Goland S, Modi K, Bitar F, Janmohamed M, Mirocha JM, Czer LSC, Illum S, Hatamizadeh P, Elkayam U. Clinical profile and predictors of complications in peripartum cardiomyopathy. J Card Fail. 2009;15:645–50. Scholar
  9. 9.
    Ntusi NB, Mayosi BM. Aetiology and risk factors of peripartum cardiomyopathy: a systematic review. Int J Cardiol. 2009;131:168–79. Scholar
  10. 10.
    McNamara DM, Elkayam U, Alharethi R, Damp J, Hsich E, Ewald G, Modi K, Alexis JD, Ramani GV, Semigran MJ, Haythe J, Markham DW, Marek J, Gorscan IIIJ, Wu WC, Lin Y, Halder I, Pisarcik J, Cooper LT, Fett JD. Clinical outcomes for peripartum cardiomyopathy in North America: results of the IPAC study (investigations of pregnancy-associated cardiomyopathy). J Am Coll Cardiol. 2015;66(8):905–14. Scholar
  11. 11.
    Hilfiker-Kleiner D, Sliwa K. Pathophysiology and epidemiology of peripartum cardiomyopathy. Nat Rev Cardiol. 2014;11:364–70. Scholar
  12. 12.
    Yancy CW, Jessup M, Task Force on Clinical Practice Guidelines and the Heart Failure Society of America, et al. 2017 ACC/AHA/HFSA focused update of the 2013 ACCF/AHA guideline for the management of heart failure. J Am Coll Cardiol. 2017;70(6):776–803. Scholar
  13. 13.
    Rasmusson K, Brunisholz K, Budge D, et al. Peripartum cardiomyopathy: post-transplant outcomes from the united network for organ sharing database. J Heart Lung Transplant. 2012;31(2):180–6. Scholar
  14. 14.
    Bozkurt B, Colvin M, Cook J, et al. American Heart Association Committee on heart failure and transplantation. Current diagnostic and treatment strategies for specific dilated cardiomyopathies. Circulation. 2016;134:e579–646. CrossRefPubMedGoogle Scholar
  15. 15.
    Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J. 1995;130(4):860–70. Scholar
  16. 16.
    Nishi I, Ishimitsu T, Ishizu T, et al. Peripartum cardiomyopathy and biventricular thrombi. Circ J. 2002;66(9):863–5. Scholar
  17. 17.
    Robson SC, Hunter S, Boys RJ, Dunlop W. Serial study of factors influencing changes in cardiac output during human pregnancy. Am J Phys. 1989;256(4 Pt 2):H1060–5. Scholar
  18. 18.
    Carlin AJ, Alfirevic Z, Gyte GM. Interventions for treating peripartum cardiomyopathy to improve outcomes for women and babies. Cochrane Database Syst Rev. 2010;9:CD008589.Google Scholar
  19. 19.
    Fujitani S, Baldisseri MR. Hemodynamic assessment in a pregnant and peripartum patient. Crit Care Med. 2005;33(10):S354–61. Scholar
  20. 20.
    Tariq S, Aronow WS. Use of inotropic agents in treatment of systolic heart failure. Int J Mol Sci. 2015;16(12):29060–8. Scholar
  21. 21.
    Diao M, Diop IB, Kane A, Camara S, Kane A, Sarr M, Ba SA, Diouf SM. Electrocardiographic recording of long duration (Holter) of 24 hours during idiopathic cardiomyopathy of the peripartum. Arch Mal Coeur Vaiss. 2004;97:25–30.PubMedGoogle Scholar
  22. 22.
    Terata M, Nakai K, Fukushima A, Itoh M, Kikuchi A, Sugiyama T. Detection of peripartum myocardial burden by vector-projected 187 channel electrocardiography and serum NT-proBNP. Int Heart J. 2013;54:140–5. Scholar
  23. 23.
    Sliwa K, Hilfiker-Kleiner D, Petri MC, Mebazaa A, Pieske B, Buchmann E, Regitz-Zagrosek V, Schaufelberger M, Tavazzi L, van Veldhuisen DJ, Watkins H, Shah AJ, Seferovic PM, Elkayam U, Pankuweit S, Papp Z, Mouquet F, McMurray JJV. 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;12:767–78. Scholar
  24. 24.
    Stevenson LW. Tailored therapy to hemodynamic goals for advanced heart failure. Eur J Heart Fail. 1999;1:251–7. Scholar
  25. 25.
    Gevaert S, Van Belleghem Y, Bouchez S, Herck I, De Somer F, De Block Y, Tromp F, Vandecasteele E, Martens F, De Pauw M. Acute and critically ill peripartum cardiomyopathy and ‘bridge to’ therapeutic options; a single center experience with intra-aortic balloon pump, extra corporeal membrane oxygenation and continuous-flow left ventricular assist devices. Crit Care. 2011;15:R93. Scholar
  26. 26.
    Loyaga-Rendon RY, Pamboukian SV, Tallaj TA, Acharya D, Cantor R, Starling RC, Naftel D, Kirklin J. Outcomes of patients with peripartum cardiomyopathy who received mechanical circulatory support. Data from the interagency registry for mechanically assisted circulatory support. Circ Heart Fail. 2014;7:300–9. CrossRefPubMedGoogle Scholar
  27. 27.
    McMurray JJV, Packer M, Desai AS, et al. Angiotensin-neprilysin inhibition versus enalapril in heart failure. N Engl J Med. 2014;371(11):993–1004. Scholar
  28. 28.
    Sliwa K, Petrie MC, Hilfiker-Kleiner D, Mebazaa A, Jackson A, Johnson MR, van der Meer P, Mbakwem A, Bauersachs J. Long-term prognosis, subsequent pregnancy, contraception and overall management of peripartum cardiomyopathy: a practical guidance paper from the heart failure Association of the European Society of cardiology group on Peripartum cardiomyopathy. Eur J Heart Fail. 2018;20:951–62. Scholar
  29. 29.
    Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, Shotan A. Pregnancy-associated cardiomyopathy: clinical characteristics and comparison between early and late presentation. Circulation. 2005;111:2050–5. Scholar
  30. 30.
    Johnson-Coyle L, Jenson L, Sobey A. Peripartum cardiomyopathy. Review and practice guidelines. Am J Crit Care. 2012;21:89–98. Scholar
  31. 31.
    Forster O, Hilfiker-Kleiner D, Ansari A, Sundstrom J, Libhaber E, Tshani W, Becker A, Yip A, Klein G, Sliwa K. Reversal of IFN-γ, oxLDL and prolactin serum levels correlate with clinical improvement in patients with peripartum cardiomyopathy. Eur J Heart Fail. 2008;10:861–8. Scholar
  32. 32.
    Goland S, Bitar F, Modi K, et al. Evaluation of the clinical relevance of baseline left ventricular ejection fraction as a predictor of recovery or persistence of severe dysfunction in women in the United States with peripartum cardiomyopathy. J Card Fail. 2011;17(5):426–30. Scholar
  33. 33.
    Haghikia A, Podweski 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(4):366. CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Sliwa K, Fett J, Elkayam U. Peripartum cardiomyopathy. Lancet. 2006;36(9536):687–93. Scholar
  35. 35.
    Libhaber E, Sliwa K, Bachelier K, et al. Low systolic blood pressure and high resting heart rate as predictors of outcome in patients with peripartum cardiomyopathy. Int J Cardiol. 2015;190:376–82. Scholar
  36. 36.
    Amos AM, Wissam WA, Russell SD. Improved outcomes in peripartum cardiomyopathy with contemporary treatments. Am Heart J. 2006;152:509–13. Scholar
  37. 37.
    Elkayam U. Risk of subsequent pregnancies in women with a history of peripartum cardiomyopathy. J Am Coll Cardiol. 2014;64:1629–36. Scholar
  38. 38.
    Fett JD, Fristoe KL, Welsh SN. Risk of heart failure relapse in subsequent pregnancy among peripartum cardiomyopathy mothers. Int J Gynaecol Obstet. 2010;109(1):34–6. 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;344:1567–71. Scholar
  40. 40.
    Hilfiker-Kleiner D, Haghikia A, Berliner D, Vogel-Claussen J, Schwab J, Schwarzkopf M, Ehlermann P, Pfister R, Michels G, Westenfeld R, Stangl V, Kindermann I, Kühl U, Angermann CE, Schlitt A, Fischer D, Podewski E, Böhm M, Sliwa K, Bauersachs J. Bromocriptine for the treatment of peripartum cardiomyopathy: a multicentre randomized study. Eur Heart J. 2017;38(35):2671–9. Scholar
  41. 41.
    Yamada T, Koyama T, Furuta I, Takeda M, Nishida R, Yamada T, Morikawa M, Minakami H. Effects of caesarean section on serum levels of NT-proBNP. Clin Endocrinol. 2013;78:460–5. Scholar
  42. 42.
    Sliwa K, Forster O, Libhaber E, Fett JD, Sundstrom JB, Hilfiker-Kleiner D, Ansari AA. Peripartum cardiomyopathy: inflammatory markers as predictors of outcome in 100 prospectively studied patients. Eur Heart J. 2006;27:441–6. Scholar
  43. 43.
    Burton GJ, Jauniaux E. Oxidative stress. Best Pract Res Clin Obstet Gynaecol. 2011;25:287–99. CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Hilfiker-Kleiner D, Kaminski K, Podewski E, Bonda T, Schaefer A, Sliwa K, Forster O, Quint A, Landmesser U, Doerries C, Luchtefeld M, Poli V, Schneider MD, Balligand JL, Desjardins F, Ansari A, Struman I, Nguyen NQ, Zschemisch NH, Klein G, Heusch G, Schulz R, Hilfiker A, Drexler H. A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum cardiomyopathy. Cell. 2007;128:589–600. Scholar
  45. 45.
    Bozkurt B, Villaneuva FS, Holubkov R, Tokarczyk T, Alvarez RJ, MacGowan GA, Murali S, Roenblum WD, Feldman AM, McNamara DM. Intravenous immune globulin in the therapy of peripartum cardiomyopathy. J Am Coll Cardiol. 1999;34:177–80. Scholar
  46. 46.
    Sliwa K, Skudicky D, Candy G, Bergemann A, Hopley M, Sareli P. The addition of pentoxifylline to conventional therapy improves outcome in patients with peripartum cardiomyopathy. Eur J Heart Fail. 2002;4:305–9. Scholar
  47. 47.
    Sliwa K, Blauwet L, Tibazarwa K, Libhaber E, Smedema JP, Becker A, et al. Evaluation of bromocriptine in the treatment of acute severe peripartum cardiomyopathy: a proof-of-concept pilot study. Circulation. 2010;121(13):1465–73. Scholar
  48. 48.
    Hilfiker-Kleiner D, Meyer GP, Schieffer E, Goldmann B, Podewski E, Struman I, Fischer P, Drexler H. Recovery from postpartum cardiomyopathy in 2 patients by blocking prolactin release with bromocriptine. J Am Coll Cardiol. 2007;50:2354–5. Scholar
  49. 49.
    Habedank D, Kuhnle Y, Elgeti T, Dudenhausen JW, Haverkamp W, Dietz R. Recovery from peripartum cardiomyopathy after treatment with bromocriptine. Eur J Heart Fail. 2008;10:1149–51. Scholar
  50. 50.
    Hopp L, Haider B, Iffy L. Myocardial infarction postpartum in patients taking bromocriptine for the prevention of breast engorgement. Int J Cardiol. 1996;57:227–32. Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Lee Anne Stratton
    • 1
  • Soidjon Khodjaev
    • 2
  • Srinivas Murali
    • 2
    Email author
  1. 1.Critical Care MedicineUniversity of Pittsburgh Medical CenterPittsburghUSA
  2. 2.Cardiovascular InstituteAllegheny Health NetworkPittsburghUSA

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