Abstract
According to current definition, peripartum cardiomyopathy (PPCM) is a rare disorder in which left ventricular dysfunction and symptoms of heart failure occur in the last month of pregnancy. It has been reported that the incidence of PPCM is 1 in 3,000–4,000 live births. The pathogenesis is poorly understood, however, infectious, immunologic, and nutritional causes have been hypothesized. Clinical presentation includes usual signs and symptoms of heart failure, and unusual presentations such as thromboembolism. Diagnosis is based upon the clinical presentation of congestive heart failure and the objective evidence of left ventricular systolic dysfunction. Early diagnosis and initiation of treatment are essential to optimize pregnancy outcome. Patients with systolic dysfunction during pregnancy are treated similar to patients who are not pregnant. The mainstays of medical therapy are digoxin, loop diuretics, sodium restriction and afterload reducing agents (hydralazine and nitrates). Due to a high risk for venous and arterial thrombosis, anticoagulation with subcutaneous heparin should be instituted. Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be avoided during pregnancy because of severe adverse neonatal effects. Effective treatment reduces mortality rates and increases the number of women who fully recover left ventricular systolic function. The prognosis is poor in patients with persistent cardiomyopathy. Subsequent pregnancies are often associated with recurrence of left ventricular systolic dysfunction.
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Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, Ansari A, Baughman KL (2000) Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. JAMA 283:1183–1188
Richie C (1849) Clinical contribution to the pathology, diagnosis and treatment of certain chronic diseases of the heart. Edinb Med Surg J 2:333–342
Gouley B, McMillan T, Bellet S (1937) Idiopathic myocardical degeneration associated with pregnancy and especially the puerperium. Am J Med Sci 19:185–199
Hull E, Hidden E (1938) Postpartal heart failure. South Med J 31:265–270
Demakis JG, Rahimtoola SH, Sutton GC, Meadows WR, Szanto PB, Tobin JR, Gunnar RM (1971) Natural course of peripartum cardiomyopathy. Circulation 44:1053–1061
Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, Shotan A (2005) Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 111:2050–2055
Whitehead SJ, Berg CJ, Chang J (2003) Pregnancy-related mortality due to cardiomyopathy: United States, 1991–1997. Obstet Gynecol 102:1326–1331
Fett JD, Christie LG, Carraway RD, Murphy JG (2005) Five-year prospective study of the incidence and prognosis of peripartum cardiomyopathy at a single institution. Mayo Clin Proc 80:1602–1606
Desai D, Moodley J, Naidoo D (1995) Peripartum cardiomyopathy: experiences at King Edward VIII Hospital, Durban, South Africa and a review of the literature. Trop Doct 25:118–123
Mielniczuk LM, Williams K, Davis DR, Tang AS, Lemery R, Green MS, Gollob MH, Haddad H, Birnie DH (2006) Frequency of peripartum cardiomyopathy. Am J Cardiol 97:1765–1768
Alvarez Navascues R, Marin R, Testa A, Paneda F, Alvarez Grande J (2001) Preeclampsia and peripartum cardiomyopathy: infrequent association. Nefrologia 21:84–87
Fett JD (2005) Peripartum cardiomyopathy (PPCM) in both surrogate and biological mother. Hum Reprod 20:2666–2668
Veille JC (1984) Peripartum cardiomyopathies: a review. Am J Obstet Gynecol 148:805–818
Demakis JG, Rahimtoola SH (1971) Peripartum cardiomyopathy. Circulation 44:964–968
Mendelson MA, Chandler J (1992) Postpartum cardiomyopathy associated with maternal cocaine abuse. Am J Cardiol 70:1092–1094
Pierce JA, Price BO, Joyce JW (1963) Familial occurrence of postpartal heart failure. Arch Intern Med 111:651–655
Pearl W (1995) Familial occurrence of peripartum cardiomyopathy. Am Heart J 129:421–422
Melvin KR, Richardson PJ, Olsen EG, Daly K, Jackson G (1982) Peripartum cardiomyopathy due to myocarditis. N Engl J Med 307:731–734
Felker GM, Jaeger CJ, Klodas E, Thiemann DR, Hare JM, Hruban RH, Kasper EK, Baughman KL (2000) Myocarditis and long-term survival in peripartum cardiomyopathy. Am Heart J 140:785–791
Midei MG, DeMent SH, Feldman AM, Hutchins GM, Baughman KL (1990) Peripartum myocarditis and cardiomyopathy. Circulation 81:922–928
Cenac A, Gaultier Y, Devillechabrolle A, Moulias R (1988) Enterovirus infection in peripartum cardiomyopathy. Lancet 2:968–969
Bultmann BD, Klingel K, Nabauer M, Wallwiener D, Kandolf R (2005) High prevalence of viral genomes and inflammation in peripartum cardiomyopathy. Am J Obstet Gynecol 193:363–365
Cenac A, Djibo A, Chaigneau C, Velmans N, Orfila J (2003) Are anti-Chlamydia pneumoniae antibodies prognosis indicators for peripartum cardiomyopathy? J Cardiovasc Risk 10:195–199
Sliwa K, Skudicky D, Bergemann A, Candy G, Puren A, Sareli P (2000) Peripartum cardiomyopathy: analysis of clinical outcome, left ventricular function, plasma levels of cytokines and Fas/APO-1. J Am Coll Cardiol 35:701–705
Brown CS, Bertolet BD (1998) Peripartum cardiomyopathy: a comprehensive review. Am J Obstet Gynecol 178:409–414
Coulson CC, Thorp JM Jr, Mayer DC, Cefalo RC (1996) Central hemodynamic effects of recombinant human relaxin in the isolated, perfused rat heart model. Obstet Gynecol 87:610–612
Kothari SS (1997) Aetiopathogenesis of peripartum cardiomyopathy: prolactin-selenium interaction? Int J Cardiol 60:111–114
O’Connell JB, Costanzo-Nordin MR, Subramanian R, Robinson JA, Wallis DE, Scanlon PJ, Gunnar RM (1986) Peripartum cardiomyopathy: clinical, hemodynamic, histologic and prognostic characteristics. J Am Coll Cardiol 8:52–56
Carvalho A, Brandao A, Martinez EE, Alexopoulos D, Lima VC, Andrade JL, Ambrose JA (1989) Prognosis in peripartum cardiomyopathy. Am J Cardiol 64:540–542
Hilfiker-Kleiner D, Sliwa K, Drexler H (2008) Peripartum cardiomyopathy: recent insights in its pathophysiology. Trends Cardiovasc Med 18(5):173–179
Hilfiker-Kleiner D, Kaminski, Podewski E, Bonda T, Schaefer A, Sliwa K, Forster O et al (2007) A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum cardiomyopathy. Cell 128(3):589–600
Abboud J, Murad Y, Chen-Scarabelli C, Saravolatz L, Scarabelli TM (2007) Peripartum cardiomyopathy: a comprehensive review. Int J Cardiol 118:295–303
Ferrero S, Colombo BM, Fenini F, Abbamonte LH, Arena E (2003) Peripartum cardiomyopathy. A review. Minerva Ginecol 55(139–151):151–158
Lee W (1991) Clinical management of gravid women with peripartum cardiomyopathy. Obstet Gynecol Clin North Am 18:257–271
Homans DC (1985) Peripartum cardiomyopathy. N Engl J Med 312:1432–1437
Brandenburg RO, Chazov E, Cherian G, Falase AO, Grosgogeat Y, Kawai C, Loogen F, Martin Judez V, Goodwin JF, Olsen EGJ, Oakley CM, Pisa Z (1980) Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies. Br Heart J 44:672–673
Yacoub A, Martel MJ (2002) Pregnancy with primary dilated cardiomyopathy. Obstet Gynecol 99:928–930
Meadows WR (1957) Idiopathic myocardial failure in the last trimester of pregnancy and the puerperium. Circulation 15:903–914
Walsh JJ, Burch GE, Black WC, Ferrans VJ, Hibbs RG (1965) Idiopathic myocardiopathy of the puerperium (postpartal heart disease). Circulation 32:19–31
Sanderson JE, Adesanya CO, Anjorin FI, Parry EH (1979) Postpartum cardiac failure—heart failure due to volume overload? Am Heart J 97:613–621
Mann MS, Cossham PS, Baker JL, Hurley PA (1987) Left atrial myxoma in the second trimester of pregnancy. Case report. Br J Obstet Gynaecol 94:592–593
Hibbard JU, Lindheimer M, Lang RM (1999) A modified definition for peripartum cardiomyopathy and prognosis based on echocardiography. Obstet Gynecol 94:311–316
Hauck AJ, Kearney DL, Edwards WD (1989) Evaluation of postmortem endomyocardial biopsy specimens from 38 patients with lymphocytic myocarditis: implications for role of sampling error. Mayo Clin Proc 64:1235–1245
Murali S, Baldisseri MR (2005) Peripartum cardiomyopathy. Crit Care Med 33:S340–S346
Lindheimer MD, Katz AI (1973) Sodium and diuretics in pregnancy. N Engl J Med 288:891–894
Cohn JN, Johnson G, Ziesche S, Cobb F, Francis G, Tristani F, Smith R, Dunkman WB, Loeb H, Wong M et al (1991) A comparison of enalapril with hydralazine-isosorbide dinitrate in the treatment of chronic congestive heart failure. N Engl J Med 325:303–310
Packer M, O’Connor CM, Ghali JK, Pressler ML, Carson PE, Belkin RN, Miller AB, Neuberg GW, Frid D, Wertheimer JH et al (1996) Effect of amlodipine on morbidity and mortality in severe chronic heart failure. Prospective Randomized Amlodipine Survival Evaluation Study Group. N Engl J Med 335:1107–1114
Stempel JE, O’Grady JP, Morton MJ, Johnson KA (1982) Use of sodium nitroprusside in complications of gestational hypertension. Obstet Gynecol 60:533–538
Mastrobattista JM (1997) Angiotensin converting enzyme inhibitors in pregnancy. Semin Perinatol 21:124–134
Uretsky BF, Young JB, Shahidi FE, Yellen LG, Harrison MC, Jolly MK (1993) Randomized study assessing the effect of digoxin withdrawal in patients with mild to moderate chronic congestive heart failure: results of the PROVED trial. PROVED Investigative Group. J Am Coll Cardiol 22:955–962
Packer M, Gheorghiade M, Young JB, Costantini PJ, Adams KF, Cody RJ, Smith LK, Van Voorhees L, Gourley LA, Jolly MK (1993) Withdrawal of digoxin from patients with chronic heart failure treated with angiotensin-converting-enzyme inhibitors. RADIANCE Study. N Engl J Med 329:1–7
White WB (1984) Management of hypertension during lactation. Hypertension 6:297–300
Anderson JL, Gilbert EM, O’Connell JB, Renlund D, Yanowitz F, Murray M, Roskelley M, Mealey P, Volkman K, Deitchman D et al (1991) Long-term (2 year) beneficial effects of beta-adrenergic blockade with bucindolol in patients with idiopathic dilated cardiomyopathy. J Am Coll Cardiol 17:1373–1381
Andersson B, Blomstrom-Lundqvist C, Hedner T, Waagstein F (1991) Exercise hemodynamics and myocardial metabolism during long-term beta-adrenergic blockade in severe heart failure. J Am Coll Cardiol 18:1059–1066
Packer M, Bristow MR, Cohn JN, Colucci WS, Fowler MB, Gilbert EM, Shusterman NH (1996) The effect of carvedilol on morbidity and mortality in patients with chronic heart failure. U.S. Carvedilol Heart Failure Study Group. N Engl J Med 334:1349–1355
Mason JW, O’Connell JB, Herskowitz A, Rose NR, McManus BM, Billingham ME, Moon TE (1995) A clinical trial of immunosuppressive therapy for myocarditis. The Myocarditis Treatment Trial Investigators. N Engl J Med 333:269–275
Bozkurt B, Villaneuva FS, Holubkov R, Tokarczyk T, Alvarez RJ Jr, MacGowan GA, Murali S, Rosenblum WD, Feldman AM, McNamara DM (1999) Intravenous immune globulin in the therapy of peripartum cardiomyopathy. J Am Coll Cardiol 34:177–180
Box LC, Hanak V, Arciniegas JG (2004) Dual coronary emboli in peripartum cardiomyopathy. Tex Heart Inst J 31:442–444
Nishi I, Ishimitsu T, Ishizu T, Ueno Y, Suzuki A, Seo Y, Ohtsuka S, Iida K, Yamaguchi I (2002) Peripartum cardiomyopathy and biventricular thrombi. Circ J 66:863–865
Jha P, Jha S, Millane TA (2005) Peripartum cardiomyopathy complicated by pulmonary embolism and pulmonary hypertension. Eur J Obstet Gynecol Reprod Biol 123:121–123
Bennani SL, Loubaris M, Lahlou I, Haddour N, Badidi M, Bouhouch R, Cherti M, Arharbi M (2003) Postpartum cardiomyopathy revealed by acute lower limb ischemia. Ann Cardiol Angeiol (Paris) 52:382–385
Pettifor JM, Benson R (1975) Congenital malformations associated with the administration of oral anticoagulants during pregnancy. J Pediatr 86:459–462
Rasmusson KD, Stehlik J, Brown RN, Renlund DG, Wagoner LE, Torre-Amione G, Folsom JW, Silber DH, Kirklin JK (2007) Long-term outcomes of cardiac transplantation for peri-partum cardiomyopathy: a multiinstitutional analysis. J Heart Lung Transplant 26:1097–1104
Hilfiker-Kleiner D, Meyer GP, Schieffer E, Goldmann B, Podewsky E, Struman I et al (2007) Recovery from postpartum cardiomyopathy in 2 patients by blocking prolactin release with bromocriptine. J Am Coll Cardiol 50(24):2354–2355
Jahns BG, Stein W, Hilfiker-Kleiner D, Pieske B, Emons G (2008) Peripartum cardiomyopathy—a new treatment option by inhibition of prolactin secretion. Am J Obstet Gynecol 199(4):e5–e6
Sutton MS, Cole P, Plappert M, Saltzman D, Goldhaber S (1991) Effects of subsequent pregnancy on left ventricular function in peripartum cardiomyopathy. Am Heart J 121:1776–1778
Amos AM, Jaber WA, Russell SD (2006) Improved outcomes in peripartum cardiomyopathy with contemporary. Am Hearth J 152(3):509–513
Fett JD, Christie LG, Murphy JG (2006) Brief communication: outcomes of subsequent pregnancy after peripartum cardiomyopathy: a case series from Haiti. Ann Intern Med 145:30–34
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We do not have a financial relationship with any organization; we have full control of all primary data and we agree to allow the journal to review our data if requested.
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Moioli, M., Valenzano Menada, M., Bentivoglio, G. et al. Peripartum cardiomyopathy. Arch Gynecol Obstet 281, 183–188 (2010). https://doi.org/10.1007/s00404-009-1170-5
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DOI: https://doi.org/10.1007/s00404-009-1170-5