Postpartum Cardiomyopathy

Article Author:
Ateeq Mubarik
Article Editor:
Arshad Muhammad Iqbal
Updated:
3/14/2019 5:37:21 PM
PubMed Link:
Postpartum Cardiomyopathy

Introduction

Postpartum cardiomyopathy, also known as peripartum cardiomyopathy (PPCM), is defined as new onset of heart failure between the last month of pregnancy and 5 months post delivery with no determinable cause. First described in a case series in 1937. Postpartum cardiomyopathy is a rare cause of heart failure. Its incidence and prevalence are highly variable depending on the race and geographic regions.[1] Its clinical outcome is also diverse from complete recovery to death. Postpartum cardiomyopathy is a diagnosis of exclusion. 

Etiology

The exact mechanism of disease is unknown; however, different hypotheses have been described regarding its etiology comprising viral myocarditis, nutritional deficiencies, autoimmunity, microchimerism, hemodynamic stresses, vascular dysfunction, hormonal insults, and underlying genetics.[2]

Epidemiology

The nationwide population-based study showed the incidence of 10.3 patients per 10,000 live births. The incidence increases with age with a maximum incidence of 40 to 54 years.[3] This incidence is highest in African Americans and people living in the southern United States, but the incidence is lowest in Hispanics. The disease is more prevalent in Asia and Africa. One study showed that the incidence of heart failure due to pregnancy is higher among Nigerian women; affecting nearly 1 out of 100 live births.[4] Data showed that every 1 in 837 to 1 in 1374  deliveries suffers from peripartum cardiomyopathy in south Asia.[5],[6] Due to the advent of new techniques for the diagnosis and more awareness regarding postpartum cardiomyopathy, its incidence has increased from 8.5 to 11.8 in the last few years.[7]

Pathophysiology

There are approximately 11 hypotheses suggested as a possible causal factor for the development of heart failure.[2] 

  1. Viral myocarditis during a peripartum period possibly due to echovirus, coxsackievirus, parvovirus B19, human herpesvirus 6, Epstein bar virus, or human cytomegalovirus[8]
  2. Unable to clear cardiac antigen autoantibodies due to reduced humoral immunity[9]
  3. Inadequate response to hemodynamic stress of pregnancy[10]
  4. Aggressive cardiac cell apoptosis[11]
  5. Inflammation due to cytokines[12]
  6. Selenium deficiency due to malnutrition[13]
  7. Familial predisposition due to a genetic mode of transmission[14]
  8. Increased prolactin effect on the heart[15]
  9. Estrogen and progesterone effect[16]
  10. Myocardial stunning due to adrenergic surge[16]
  11. Myocardial ischemia[17]

Histopathology

The diagnosis is based on clinical presentation and echocardiographic findings. A biopsy is not needed for the diagnosis however when done in rare cases; it shows patchy fibrosis, myofiber hypertrophy, degeneration, and necrosis with inflammatory endomyocardial infiltrates. It also sometimes reveals viral genomes such as herpesvirus 6, parvovirus B19, and Epstein-Barr virus.[18]

History and Physical

Most patients present soon after delivery especially in the first week postpartum. The symptoms are suggestive of heart failure, for example, orthopnea and paroxysmal nocturnal dyspnea. These symptoms are usually attributed to normal pregnancy and that is why a diagnosis of postpartum cardiomyopathy can be easily missed. Physical examination findings include tachycardia, elevated jugular venous pressure, bilateral pulmonary crackles due to pulmonary edema, third heart sound (S3) and displaced apical pulse. Severe cases may present with acute respiratory failure or cardiogenic shock and a need for close monitoring in the intensive care unit. 

Some of the most common risk factors for the development of postpartum cardiomyopathy are as follows:

  • Advanced maternal age (more cases reported in both extremes of age) 
  • High parity (71% of women diagnosed with PPCM had three or more prior pregnancies)[19]
  • High gravidity
  • Twin pregnancy (more endemic in women with twin pregnancies)
  • Use of tocolytic therapy (greater than 4 weeks can cause silent ischemia)[20]
  • African descent (more prevalent in the African population)
  • Poverty
  • Hypertension
  • Cocaine abuse

Evaluation

ECG may show non-specific changes like sinus tachycardia, interventricular delay and sometimes, LBBB pattern.

Echocardiography suffices to differentiate it from other causes and usually shows left ventricle dilatation of variable degrees, left ventricle systolic dysfunction, right ventricular and bi-atrial enlargement, mitral and tricuspid regurgitation, and pulmonary hypertension.[21]

Echocardiography criteria to diagnose PPCM includes ejection fraction less than 45%, end-diastolic diameter greater than 2.7 cm/m2 and/or M-mode fractional shortening less than 30%[21].

Cardiac MRI can also be used to diagnose when an accurate estimation of the ejection fraction (EF) is required.

Treatment / Management

Treatment is usually supportive and directed toward the management of the heart failure symptoms.[22] Standard heart failure therapy is used to optimize the patient's volume status. Beta-blockers and ACEIs are the most commonly used drugs and have shown to lower the mortality.[23] However, an ACEI is contraindicated in pregnant patients. Diuretics are often used to ease symptoms related to heart failure. Novel anti-heart failure medications, such as sacubitril/valsartan have been reported to improve heart failure symptoms in pregnancy-related cardiomyopathies.[24]

Recent data suggest that an increase in oxidative stress during the peripartum period increases the formation of abnormal 16-kDa prolactin which induces toxic effects on cardiac myocyte. Bromocriptine, a dopamine receptor agonist with prolactin-blocking properties, decreases the effect of 16-kDa prolactin on cardiac myocyte and has been associated with better outcomes in small studies.[25]

Cardiac resynchronization therapy has also shown to improve ejection fraction and outcomes when medical therapy alone is ineffective.[26]

Differential Diagnosis

  • Takotsubo cardiomyopathy[27]
  • Normal physiological effects of pregnancy
  • Pulmonary embolism
  • Acute pulmonary edema from prolonged tocolysis or preeclampsia
  • Myocardial infarction
  • Pneumonia

Prognosis

Long-term outcomes are variable. Fifty percent of the patients improve with standard medical treatment. However, 25% of patients develop chronic heart failure, and the remainder dies during the course of the disease.[28] The patient should be advised against pregnancy if the EF remains low, as mortality is high in such scenarios. However, if pregnancy is desired, the patient should wait for at least 5 years after the ejection fraction has normalized.

Complications

Following complications have been associated with PPCM:

  • Progression to severe heart failure
  • Cardiogenic shock
  • Arrhythmias
  • Thromboembolic events such as stroke, TIA, or LV apical clots
  • Death

Enhancing Healthcare Team Outcomes

An interprofessional approach to peripartum cardiomyopathy is recommended.

Because of the high morbidity and mortality of peripartum cardiomyopathy, the prenatal nurse, obstetrician and nurse practitioner working in obstetrics must be fully aware of this disorder. Only through a prompt and appropriate referral to a cardiologist can the mortality be improved.

An interprofessional approach should be used to manage moderate-to-severe cases. The patient may require close monitoring in the ICU and repeat ultrasounds may be required to assess the viability of the fetus. All patients should be warned about the potential toxicity of the cardiac medications on the fetus.

Finally, the patient should be educated to avoid future pregnancies if ejection fraction fails to improve as it increases the mortality up to 50%. Despite optimal therapy, the condition still carries a mortality rate of more than 10% to 20%.[29][30]


References

[1] Brar SS,Khan SS,Sandhu GK,Jorgensen MB,Parikh N,Hsu JW,Shen AY, Incidence, mortality, and racial differences in peripartum cardiomyopathy. The American journal of cardiology. 2007 Jul 15     [PubMed PMID: 17631087]
[2] Ntusi NB,Mayosi BM, Aetiology and risk factors of peripartum cardiomyopathy: a systematic review. International journal of cardiology. 2009 Jan 9     [PubMed PMID: 18722678]
[3] Kolte D,Khera S,Aronow WS,Palaniswamy C,Mujib M,Ahn C,Jain D,Gass A,Ahmed A,Panza JA,Fonarow GC, Temporal trends in incidence and outcomes of peripartum cardiomyopathy in the United States: a nationwide population-based study. Journal of the American Heart Association. 2014 Jun 4     [PubMed PMID: 24901108]
[4] Isezuo SA,Abubakar SA, Epidemiologic profile of peripartum cardiomyopathy in a tertiary care hospital. Ethnicity     [PubMed PMID: 17682350]
[5] Hasan JA,Qureshi A,Ramejo BB,Kamran A, Peripartum cardiomyopathy characteristics and outcome in a tertiary care hospital. JPMA. The Journal of the Pakistan Medical Association. 2010 May     [PubMed PMID: 20527612]
[6] Pandit V,Shetty S,Kumar A,Sagir A, Incidence and outcome of peripartum cardiomyopathy from a tertiary hospital in South India. Tropical doctor. 2009 Jul     [PubMed PMID: 19535757]
[7] Mielniczuk LM,Williams K,Davis DR,Tang AS,Lemery R,Green MS,Gollob MH,Haddad H,Birnie DH, Frequency of peripartum cardiomyopathy. The American journal of cardiology. 2006 Jun 15     [PubMed PMID: 16765131]
[8] Farber PA,Glasgow LA, Viral myocarditis during pregnancy: encephalomyocarditis virus infection in mice. American heart journal. 1970 Jul     [PubMed PMID: 4316715]
[9] Warraich RS,Sliwa K,Damasceno A,Carraway R,Sundrom B,Arif G,Essop R,Ansari A,Fett J,Yacoub M, Impact of pregnancy-related heart failure on humoral immunity: clinical relevance of G3-subclass immunoglobulins in peripartum cardiomyopathy. American heart journal. 2005 Aug     [PubMed PMID: 16086928]
[10] Lampert MB,Weinert L,Hibbard J,Korcarz C,Lindheimer M,Lang RM, Contractile reserve in patients with peripartum cardiomyopathy and recovered left ventricular function. American journal of obstetrics and gynecology. 1997 Jan     [PubMed PMID: 9024112]
[11] Wencker D,Chandra M,Nguyen K,Miao W,Garantziotis S,Factor SM,Shirani J,Armstrong RC,Kitsis RN, A mechanistic role for cardiac myocyte apoptosis in heart failure. The Journal of clinical investigation. 2003 May     [PubMed PMID: 12750399]
[12] Drexler H, Changes in the peripheral circulation in heart failure. Current opinion in cardiology. 1995 May     [PubMed PMID: 7612976]
[13] 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. International journal of cardiology. 1992 Jul     [PubMed PMID: 1428253]
[14] Sliwa K,Damasceno A,Mayosi BM, Epidemiology and etiology of cardiomyopathy in Africa. Circulation. 2005 Dec 6     [PubMed PMID: 16330699]
[15] Hopp L,Haider B,Iffy L, Myocardial infarction postpartum in patients taking bromocriptine for the prevention of breast engorgement. International journal of cardiology. 1996 Dec 13     [PubMed PMID: 9024910]
[16] Lampert MB,Lang RM, Peripartum cardiomyopathy. American heart journal. 1995 Oct     [PubMed PMID: 7572598]
[17] Koide T,Saito Y,Sakamoto T,Murao S, Peripartal cardiomyopathy in Japan. A critical reappraisal of the concept. Japanese heart journal. 1972 Nov     [PubMed PMID: 4539938]
[18] Bültmann BD,Klingel K,Näbauer M,Wallwiener D,Kandolf R, High prevalence of viral genomes and inflammation in peripartum cardiomyopathy. American journal of obstetrics and gynecology. 2005 Aug     [PubMed PMID: 16098856]
[19] Demakis JG,Rahimtoola SH, Peripartum cardiomyopathy. Circulation. 1971 Nov     [PubMed PMID: 4255967]
[20] Bassett JM,Burks AH,Levine DH,Pinches RA,Visser GH, Maternal and fetal metabolic effects of prolonged ritodrine infusion. Obstetrics and gynecology. 1985 Dec     [PubMed PMID: 3934609]
[21] Hibbard JU,Lindheimer M,Lang RM, A modified definition for peripartum cardiomyopathy and prognosis based on echocardiography. Obstetrics and gynecology. 1999 Aug     [PubMed PMID: 10432149]
[22] Khan A,Paré E,Shah S, Peripartum Cardiomyopathy: a Review for the Clinician. Current treatment options in cardiovascular medicine. 2018 Sep 29     [PubMed PMID: 30269217]
[23] Ma G,Fang Q,Wang F, The effect of beta-blockers on mortality in patients with heart failure and atrial fibrillation: A meta-analysis of observational cohort and randomized controlled studies. Cardiology journal. 2018 Jul 16     [PubMed PMID: 30009373]
[24] Gaddipati VC,Patel AA,Cohen AJ, The Use of a Novel Heart Failure Agent in the Treatment of Pregnancy-Associated Cardiomyopathy. Case reports in cardiology. 2017     [PubMed PMID: 28890834]
[25] Sliwa K,Blauwet L,Tibazarwa K,Libhaber E,Smedema JP,Becker A,McMurray J,Yamac H,Labidi S,Struman I,Hilfiker-Kleiner D, Evaluation of bromocriptine in the treatment of acute severe peripartum cardiomyopathy: a proof-of-concept pilot study. Circulation. 2010 Apr 6     [PubMed PMID: 20308616]
[26] Mouquet F,Mostefa Kara M,Lamblin N,Coulon C,Langlois S,Marquie C,de Groote P, Unexpected and rapid recovery of left ventricular function in patients with peripartum cardiomyopathy: impact of cardiac resynchronization therapy. European journal of heart failure. 2012 May     [PubMed PMID: 22447949]
[27] Citro R,Giudice R,Mirra M,Petta R,Baldi C,Bossone E,Piscione F, Is Tako-tsubo syndrome in the postpartum period a clinical entity different from peripartum cardiomyopathy? Journal of cardiovascular medicine (Hagerstown, Md.). 2013 Aug     [PubMed PMID: 23519095]
[28] Yaméogo NV,Samadoulougou AK,Kagambèga LJ,Kologo KJ,Millogo GRC,Thiam A,Guenancia C,Zansonré P, Maternal and fetal prognosis of subsequent pregnancy in black African women with peripartum cardiomyopathy. BMC cardiovascular disorders. 2018 Jun 18     [PubMed PMID: 29914408]
[29] Dayoub EJ,Datwani H,Lewey J,Groeneveld PW, One-Year Cardiovascular Outcomes in Patients With Peripartum Cardiomyopathy. Journal of cardiac failure. 2018 Oct     [PubMed PMID: 30194024]
[30] Honigberg MC,Sarma AA, Pregnancy Among Survivors of Childhood Cancer: Cardiovascular Considerations. Current treatment options in cardiovascular medicine. 2018 Jun 19     [PubMed PMID: 29923132]