The New England Journal of MedicinePeripartum Cardiomyopathy

Deciphering the Enigma of Peripartum Cardiomyopathy: Unraveling Genetic and Hormonal Underpinnings in Maternal Heart Health

Peripartum cardiomyopathy (PPCM) emerges as a critical and complex condition in maternal health, characterized by acute cardiac degeneration leading to heart failure during pregnancy or shortly after childbirth. This article presents the multifaceted aspects of PPCM, elucidating its prevalence, risk factors, clinical presentation, differential diagnosis, pathogenesis, management strategies, and outcomes. It provides a comprehensive overview tailored for physicians, highlighting the latest findings on genetic predispositions, hormonal influences, and the pressing need for more targeted treatments and long-term management strategies in PPCM.

Key Points:

1. Prevalence and Risk Factors: PPCM affects approximately 1 in 2,000 births globally, with significant regional variations. It is notably more prevalent in Black women in the US, with a higher risk associated with hypertensive pregnancy disorders, multiple gestations, advanced maternal age, and anemia.
2. Clinical Presentation: Symptoms mimic those of late pregnancy, causing diagnostic delays. These include dyspnea, orthopnea, and signs of heart failure. The disease often presents post-delivery, with Black women in the US typically presenting later in the postpartum period.
3. Diagnosis and Differential Diagnosis: PPCM is a diagnosis of exclusion. Echocardiography is key, revealing systolic dysfunction without other structural heart diseases. Differential diagnosis includes preexisting heart conditions, preeclampsia-induced pulmonary edema, and pulmonary embolism.
4. Pathogenesis Insights: Recent studies suggest a hormonal model, with PPCM potentially triggered by hormones from the pituitary and placenta, in combination with individual cardiac susceptibility. The exact mechanisms, however, remain elusive.
5. Genetic Factors: Approximately 15% of PPCM cases involve genetic variants associated with dilated cardiomyopathy, suggesting a possible genetic predisposition in some patients.
6. Management and Therapies: Treatment largely follows guidelines for nonischemic dilated cardiomyopathy, with specific considerations for the peripartum period. Bromocriptine therapy, aimed at suppressing prolactin, is under investigation.
7. Outcomes and Racial Disparities: Recovery is common, but a subset of patients experience chronic heart failure or require heart transplantation. Significant racial disparities exist, especially in the US, where Black women face poorer outcomes.
8. Future Directions: The need for more research is evident, particularly regarding long-term outcomes, withdrawal of medications post-recovery, and understanding racial disparities.

Preeclampsia is associated with a 4-fold increase in future incident heart failure and a 2-fold increased risk in coronary heart disease, stroke, and death because of coronary heart or cardiovascular disease (AHA).

More on Contraception/Pregnancy