What is postpartum cardiomyopathy (PPCM)?

From the Guidelines

Postpartum cardiomyopathy is a rare but serious heart condition that develops during the last month of pregnancy or within five months after delivery, causing the heart muscle to weaken and become less efficient at pumping blood, leading to heart failure 1.

Definition and Classification

The European Society of Cardiology defines peripartum cardiomyopathy (PPCM) as a non-familial, non-genetic form of dilated cardiomyopathy associated with pregnancy, characterized by left ventricular systolic dysfunction and heart failure 1. The diagnosis is made based on the absence of an identifiable cause of heart failure, the absence of recognizable heart disease prior to the last month of pregnancy, and left ventricular systolic dysfunction demonstrated by classical echocardiographic criteria.

Symptoms and Treatment

Symptoms of PPCM include shortness of breath, fatigue, swelling in the legs, and chest pain. Treatment typically involves standard heart failure medications such as beta-blockers, ACE inhibitors (after delivery), diuretics, and sometimes anticoagulants to prevent blood clots 1. Specific medications might include metoprolol 25-200 mg daily, lisinopril 5-40 mg daily (postpartum only), and furosemide 20-80 mg daily as needed for fluid retention.

Recovery and Risks

Recovery varies significantly, with about 30-50% of women recovering normal heart function within six months, while others may require long-term management or even heart transplantation in severe cases. Women who have experienced PPCM face increased risks in future pregnancies and should receive counseling about these risks before considering another pregnancy 1. Some key points to consider in the management of PPCM include:

• Guideline-directed medical therapy known to benefit patients with left ventricular dysfunction should be considered, taking into account both pregnancy and breastfeeding status 1.
• The duration of standard heart failure medications in patients with PPCM should be indefinite when left ventricular function fails to normalize 1.
• Patients with PPCM whose ventricular function does not normalize after pregnancy should be counseled against a subsequent pregnancy because it carries a significant risk of morbidity and mortality 1.

From the Research

Definition and Characteristics

• Postpartum cardiomyopathy (PPCM) is a rare but potentially life-threatening condition that occurs in previously healthy women during the last month of pregnancy and up to 5-6 months postpartum 2.
• It is characterized by left ventricular systolic dysfunction in the absence of any other identifiable cardiac causes 3.
• PPCM is a diagnosis of exclusion, as it shares many clinical characteristics with other forms of systolic heart failure secondary to cardiomyopathy 2.

Pathophysiology

• The etiology and pathophysiology of PPCM remain uncertain, although recent observations strongly suggest the specific role of prolactin cleavage secondary to unbalanced peri/postpartum oxidative stress 2.
• An increase in oxidative stress and anti-angiogenic activity play key roles in the pathophysiology of peripartum cardiomyopathy 4.

Clinical Presentation and Diagnosis

• PPCM typically presents in the third trimester or in the first few months postpartum 4.
• Frequent presenting symptoms of PPCM, such as prostration, shortness of breath on mild exertion, and coughing, are often initially misinterpreted as evidence of pneumonia or as physiological accompaniments of pregnancy and delivery 5.
• The clinical picture of PPCM corresponds to a dilated cardiomyopathy (DCM) with signs of severe heart failure 5.

Management and Treatment

• The management of heart failure requires a multidisciplinary approach during pregnancy, considering the possible adverse effects on the fetus 2.
• After delivery, the treatment is in accordance with the current guidelines for heart failure 2.
• Therapies that target the dysregulation of oxidative stress and anti-angiogenic activity may have a future role in treatment 4.
• Suppression of prolactin release using bromocriptine, a dopamine-receptor antagonist, has been associated with more favorable outcomes in small studies but more research is needed 4, 5.

Breastfeeding Considerations

• Breastfeeding should not routinely be ruled out if the mother is taking heart failure medication, but the consequences for the infant need to be considered 6.
• An informed risk assessment to minimize potential harm to the infant can be carried out using the evidence that is available along with a consideration of drug properties, adverse effects, pediatric use, and pharmacokinetics 6.