What is the diagnosis and treatment for peripartum (postpartum) cardiomyopathy?

From the Guidelines

Peripartum cardiomyopathy should be treated with standard heart failure medications, with careful consideration of pregnancy status, and bromocriptine may be considered postpartum to enhance cardiac function recovery, as recommended by the 2022 AHA/ACC/HFSA guideline for the management of heart failure 1.

Treatment Overview

Peripartum cardiomyopathy is a rare but serious form of heart failure that occurs during the last month of pregnancy or within five months after delivery in women with no prior history of heart disease.

• Treatment involves standard heart failure medications, with careful consideration of pregnancy status.
• For pregnant women, safe medications include hydralazine, nitrates, and beta-blockers like metoprolol (starting at 12.5-25mg twice daily) 1.
• After delivery, ACE inhibitors such as lisinopril (5-40mg daily) or ARBs can be added.
• Diuretics like furosemide (20-80mg daily) help manage fluid retention.
• Anticoagulation with heparin during pregnancy or warfarin postpartum is recommended if ejection fraction is below 30-35% to prevent thromboembolism, as suggested by the European Society of Cardiology 1.

Postpartum Management

• Bromocriptine may be considered postpartum to enhance cardiac function recovery, as recommended by the 2022 AHA/ACC/HFSA guideline for the management of heart failure 1.
• Therapy with bromocriptine must be accompanied by prophylactic (or therapeutic) anticoagulation, as indicated by the 2021 narrative review on heart disease in women 1.

Follow-up and Monitoring

• Patients should restrict sodium intake to 2g daily, limit fluid intake to 1.5-2L daily, and monitor daily weights.
• Close follow-up with both cardiology and obstetrics is essential, with regular echocardiograms to track cardiac function, as recommended by the 2016 scientific statement from the American Heart Association 1.
• Recovery often occurs within 3-6 months, but some women may require long-term therapy or even heart transplantation in severe cases.
• The condition is thought to result from a combination of increased cardiac stress during pregnancy, genetic factors, and possible autoimmune or inflammatory processes, as discussed in the 2010 position statement from the Heart Failure Association of the European Society of Cardiology 1.

From the Research

Definition and Incidence

• Peripartum cardiomyopathy (PPCM) is a serious pregnancy-associated disorder of unknown etiology 2.
• It is a rare form of heart failure due to left ventricular systolic dysfunction that affects women late in pregnancy and the postpartum period 3.
• PPCM accounts for an important percentage of pregnancy-related deaths 4.

Etiopathogenesis

• The precise cellular and molecular mechanisms underlying PPCM are unclear 2.
• Theories include genetic predisposition, myocardial inflammation, and angiogenic dysregulation 3, 5.
• An increase in oxidative stress and anti-angiogenic activity play key roles in the pathophysiology of peripartum cardiomyopathy 5.

Diagnosis

• A diagnosis of exclusion, peripartum cardiomyopathy can be difficult to diagnose in the context of the normal physiologic changes of pregnancy and requires a high index of suspicion 3.
• Diagnosis should be made by clinical evaluation and echocardiography 4.
• An electrocardiogram and brain natriuretic peptide level should be obtained in patients with symptoms of dyspnea and fatigue, in addition to baseline laboratory tests 2.

Management

• Patients should be started on diuretics if volume overloaded, and beta-blockers (preferably metoprolol) if no contraindications exist 2.
• Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers should be avoided during pregnancy or lactation 2.
• Primary treatment modalities include initiation of a medication regimen aimed at the optimization of preload and reduction of afterload 3.
• Mechanical circulatory support and/or cardiac transplantation are required in some cases 5, 6.

Risk Factors and Outcomes

• Risk factors for PPCM include hypertensive disorders of pregnancy, Black race, and maternal age older than 30 years 3.
• Patients with PPCM are at increased risk of acute clinical decompensation, cardiac arrhythmias, thromboembolic complications, and death 3.
• The rate of recovery of ventricular function in PPCM is higher than in other forms of dilated cardiomyopathy 5.
• Significant improvement in ventricular function can be expected in up to 50% of patients 4.