There are some patients you come across during medical training who you never forget. Ms. W, a postpartum woman I met during anesthesiology residency, was one such patient. I hope that by sharing her story and reviewing what is known about the diagnosis and management of peripartum cardiomyopathy, you will be better prepared to care for future patients with this complex disease.
Ms. W was a 31 year old woman of Cameroonian descent, who I first met during an ICU rotation as a CA-2/PGY-3. Her pregnancy was complicated only by preeclampsia at 38 weeks, necessitating an induction of labor. She had an uncomplicated vaginal delivery, and was discharged home on postpartum day two with a healthy baby boy.
Like most postpartum patients, her focus at home was on her newborn, and despite mild shortness of breath, cough and palpitations at home, she and her providers were quick to attribute her symptoms to sleep deprivation, generalized fatigue, and a passing upper respiratory illness. Four weeks postpartum, she was unable to walk to the bathroom without becoming extremely short of breath and brought to the emergency room, where she was found to be in extremis. Her respiratory rate was over 30 breaths/minute, her oxygen saturation was 85% on room air, and she had 4+ pitting edema to both thighs. Ms. W was rapidly triaged with all the tests and consults, and found to have a left ventricular ejection fraction of 25% with a failing right ventricle. She was diagnosed with peripartum cardiomyopathy and admitted to the ICU.
I first met Ms. W one week after admission to the ICU, and at first glance, she didn't appear ill. Unlike most of the other patients on the unit who were sedated or sleeping, she was sitting up in bed, reading Pride and Prejudice. However, a quick chart review (and the continuous hum of a machine) told me she was probably feeling better due to aggressive medical management and the presence of a right ventricular assist device (RVAD).
She was the first patient I ever had with PPCM, and one of the few who required an RVAD to support right heart function. In spite of what she endured in the weeks that followed - erosion of the device into her abdominal organs and endless infections that prevented her from becoming a transplant candidate - Ms. W could always be found sitting up, glasses on, reading the classics. She bravely endured hundreds of tests and procedures, and the worst pain of all - separation from her newborn. Tragically, she never made it home to her child, despite aggressive management of her heart condition... once it was recognized.
Ms. W was taken far too soon, by a disease we are often late to diagnose.
Peripartum Cardiomyopathy
The following definition of PPCM was proposed by the European Society of Cardiology in 2010: non-ischemic cardiomyopathy with reduced ejection fraction (EF), usually <45%, presenting toward the end of pregnancy or in the months after delivery in a woman without previously known structural heart disease. [1]
"The fact that we are yet to determine a precise definition for PPCM reflects our incomplete understanding of the disease process, its underlying etiology, and variable phenotypes." [2]
There are several theories about the etiology of PPCM. Though studies are ongoing, the most likely explanation is that the onset and severity are multifactorial, attributed to genetic predisposition, inflammation, vascular abnormalities and hormonal changes during pregnancy.
Presentation of PPCM
Most patients with PPCM present with signs and symptoms of heart failure, including but not limited to dyspnea, lower extremity edema, orthopnea, and paroxysmal nocturnal dyspnea. These symptoms can be confused with those of normal pregnancy (especially in late gestation), which often leads to missed or delayed diagnosis of PPCM and underestimation of the incidence of this condition.
Risk Factors for PPCM [3]
- Age > 30 (>50% of cases)
- Race (black women have a greater prevalence of PPCM and are at risk for a more severe phenotype)
- Preeclampsia & hypertension (22% of PPCM cases co-exist with PIH)
- Multiple gestation (the average rate of twin gestations in cases of PPCM across 16 studies was 9%) [4]
Patients with a severe phenotype of PPCM at presentation may have symptoms of advanced heart failure, necessitating rapid diagnosis and escalation of supportive care. Patients with PPCM should be managed by a multidisciplinary team that includes cardiology, ICU, MFM, and OB Anesthesiology if the patient is still pregnant.
In the absence of a single defined disease pathway, PPCM management strategies prioritize supporting forward flow, minimizing pulmonary edema and third spacing, and reversing pathologic cardiovascular remodeling. [5] Key features include optimizing volume status, neutralizing maladaptive neurohormonal responses, and preventing thromboembolic and arrhythmic complications. [6]
Bromocriptine: Helpful or Not?
Bromocriptine remains an experimental treatment, and there is no strong evidence supporting its use in PPCM.
Why did we start using it?
As we have discussed extensively, the pathogenesis of PPCM is incompletely understood. The prolactin theory is based on a mouse model that demonstrated how oxidative stress led to cleavage of the lactation hormone, prolactin. The prolactin breakdown products in these mice were vasculotoxic and led to significant myocardial dysfunction. Mice treated with the dopamine D2 agonist bromocriptine had complete reversal of their cardiomyopathy. [7]
Where are we now?
Subsequent human studies based on this hypothesis are inconsistent at best. Therefore, bromocriptine is not currently recommended in the United States for treatment of PPCM. Larger trials are under way with no outcomes yet reported.
Bromocriptine suppresses lactation and is associated with thromboembolic events, so if patients are started on this experimental therapy, anticoagulation is recommended. Bromocriptine should not be given without consideration for the potential benefits of breastfeeding.
[8]
Prognosis and Predictors of Adverse Outcomes & Recovery in PPCM:
Our understanding of short and long term survival and recovery of cardiac function is based on only a few studies, namely, the IPAC (United States, 100 patients, 2015) and Soweto (South Africa, 176 patients, 2013) studies. [9]
Prognosis
Most patients (>70%) with PPCM will recover fully, meaning, return to an LVEF >50% within 6 months, but some may take as long as 2-3 years.
Patients who present with an LVEF >30% at the time of diagnosis are more likely to make a full recovery than those with LVEF < 30%. African ancestry is associated with a reduced likelihood of recovery, possibly related to a greater proportion presenting with severe LV dysfunction at the time of diagnosis. Though recovery occurs in the majority of cases, one study of 66 PPCM patients from Germany demonstrated that >20% of patients experienced other persistent cardiovascular conditions such as arterial hypertension or arrhythmias in spite of recovery of their LV function. [10]
Mortality rates at 12 months after diagnosis have been reported to be between 4-14%, with racial variation [11].
Predictors of Adverse Outcomes [12]
- Severe LV dysfunction at diagnosis (EF <30%).
- Reduced EF is not a specific predictor of failure to recover and should not be considered an indication for premature device implantation or cardiac transplantation.
- Greater degree of LV remodeling, LV dilatation
- Obesity
- Preeclampsia
- RV systolic dysfunction
- LV thrombus
There are times when no matter what we do, how early we diagnose the problem, or how rapidly we intervene, we cannot prevent a negative outcome. It is impossible to say whether anything could have prevented Ms. W's tragic death. However, for many patients, early recognition and intervention can prevent disease progression, improve heart function, and reduce complications like heart failure, arrhythmias, and death.
For help differentiating normal pregnancy/postpartum symptoms from pathologic symptoms, consider using the CDPH/CMQCC Task Force Algorithm for recognizing Cardiovascular Disease in Pregnancy.
To learn the specifics of managing PPCM and other cardiac problems during pregnancy and labor, come to our upcoming three day in-person educational event, OB Shock 2026 from March 19-21, 2026 in Houston, TX!
1. Sliwa K, Hilfiker‐Kleiner D, Petrie MC, et al. Current state of knowledge on diagnosis, management, and therapy of peripartum cardiomyopathy: A position from the Heart Failure Association of the European Society of Cardiology Working on peripartum cardiomyopathy. European Journal of Heart Failure. doi:10.1093/eurjhf/hfq120
2. Oyenubi O, Benjamin P, Chan MA, et al. Abstract 15353: Identifying different clinical phenotypes in peripartum cardiomyopathy by latent class analysis. Circulation. 2022;146. doi:10.1161/circ.146.suppl_1.15353
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10. Moulig V, Pfeffer TJ, Ricke‐Hoch M, et al. Long‐term follow‐up in peripartum cardiomyopathy patients with contemporary treatment: Low mortality, high cardiac recovery, but significant cardiovascular co‐morbidities. European Journal of Heart Failure. 2019;21(12):1534-1542. doi:10.1002/ejhf.1624
11. 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: practical guidance paper from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy. Eur J Heart Fail. 2018 Jun;20(6):951-962. doi: 10.1002/ejhf.1178. Epub 2018 Mar 26. PMID: 29578284.
12. 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. Journal of Cardiac Failure. 2011;17(5):426-430. doi:10.1016/j.cardfail.2011.01.007.