Peripartum cardiomyopathy (PPCM, also called pregnancy-associated cardiomyopathy)

Peripartum cardiomyopathy is defined as a condition meeting four criteria [ 4-6 ]:

  1. Development of heart failure (HF) in the last month of pregnancy or within five months of delivery.
  2. Absence of another identifiable cause for the HF. (See "Causes of dilated cardiomyopathy" .)
  3. Absence of recognizable heart disease prior to the last month of pregnancy.
  4. LV systolic dysfunction (eg, left ventricular ejection fraction [LVEF] below 45 percent or a reduced fractional shortening).

N.B. Women who develop cardiomyopathy earlier in pregnancy do not meet the definition of PPCM based upon the first and third criteria; however, the disease process is probably the same. (Hence, it is called

Etiology: unknown

Inflammatory cytokines may play a role in the pathogenesis and progression of cardiomyopathy and HF. The cytokines that are elevated in PPCM compared to controls include tumor necrosis factor (TNF)-alpha and interleukin-6 early pregnancy-associated cardiomyopathy). In addition, Fas/Apo-1, an apoptosis signaling receptor, and C-reactive protein are associated with more severe disease.

It has been suggested that a maternal immunologic response to a fetal antigen can lead to PPCM. Fetal cells may escape into the maternal circulation and remain there without being rejected due to weak immunogenicity of the paternal haplotype of the chimeric cells [ 29 ]. If these cells lodge in the cardiac tissue, they can trigger a pathologic autoimmune response.

Hemodynamic factors  — During pregnancy, there is a significant increase in blood volume and cardiac output, which results in transient LV remodeling and hypertrophy. It is possible that there is an exaggerated decrease in LV systolic function in women who develop PPCM. The hemodynamic stress of gestational hypertension, which is more common in women with PPCM, may contribute to the development of HF [ 35 ].

Role of prolactin  — Altered prolactin processing is thought to be involved in the pathogenesis of PPCM. Mice with a knockout in the cardiac tissue-specific signal transduction and activator of transcription 3 (STAT3), develop PPCM [ 36 ]. Reduction in STAT3 leads to increased cleavage of prolactin into an antiangiogenic and proapoptotic 16kDa isoform. Treatment with bromocriptine , an inhibitor of prolactin secretion, prevents the development of PPCM in these mice.

Reduced cardiac STAT3 levels have also been observed in terminally failing hearts from PPCM patients [ 36 ], but this is a nonspecific finding seen in end-stage dilated cardiomyopathy, generally [ 37 ]. It is unknown whether STAT3 levels are reduced in PPCM at an earlier stage of disease. Preliminary results of studies of bromocriptine therapy in women with PPCM suggest that prolactin could be a key target in clinical PPCM.

Risk factors  — Although the etiology of PPCM remains unclear, a number of factors have been associated with increased risk, including the following: