ESC Peripartum Cardiomyopathy Global Registry Outcomes

Quick Takes

  • Early diagnosis and treatment of peripartum cardiomyopathy (PPCM) is important. Just under half of the deaths occurred within 30 days of diagnosis.
  • Just under half of women with PPCM experience myocardial recovery within 6 months.
  • Neonatal death occurred in 5% of women with PPCM.

Study Questions:

What are the clinical presentation, management, and 6-month outcomes in women with peripartum cardiomyopathy (PPCM) globally?

Methods:

The European Society of Cardiology (ESC) EURObservational Research Programme global registry on PPCM included participants from 49 countries. Women with PPCM were enrolled between 2012 and 2018.

Results:

Of 739 women, 33% were Caucasian, 28% were Black, 21% were Asian, 13% were Middle Eastern, 1% were Hispanic, enrolled from Europe (33%), Africa (29%), Asia-Pacific (15%), and the Middle East (22%). Mean age was 31 ± 6 years, mean left ventricular ejection fraction (LVEF) was 31 ± 10%, 67% of women had a LVEF ≤35%, and 10% had a previous pregnancy complicated by PPCM. Most women (44%) developed symptoms within 1 month of delivery. Bromocriptine was used in 15% with regional variation (Europe 15%, Africa 26%, Asia-Pacific 8%, Middle East 4%). Of the 598 (81%) women with follow-up information, average 6-month mortality was 6%, primarily due to heart failure (42%) or sudden death (30%). Re-admission occurred in 10%. Thromboembolic events occurred in 7%. Recovery (LVEF >50%) occurred in 46%. Neonatal death occurred in 5% on average.

Conclusions:

PPCM is a disease that occurs globally, but there are variations in presentations and outcomes by region. PPCM carries substantial maternal and neonatal morbidity and mortality, and just under half of women experience myocardial recovery.

Perspective:

This is a large multinational observational registry of over 700 women with PPCM from 49 countries in Europe, Africa, Asia-Pacific, and the Middle East. Several important points about PPCM are illustrated in this study:

  1. Significant morbidity and mortality can result from PPCM and early detection is important. At 6 months, the mean mortality rate was 6%, and just under half of the deaths occurred within the first 30 days of diagnosis. Less than half had recovery of LVEF to 50% or higher, and many had persistent dysfunction.
  2. The majority of deaths were sudden or related to heart failure. More research is needed to determine which patients are at highest risk of arrhythmias, potentially benefiting from wearable cardioverter defibrillators or early ICD placement.
  3. Adverse outcomes may be more prevalent than reflected in this registry. Nearly 20% of the initial cohort did not have follow-up at 6 months, women with severe illness may have died prior to enrollment or during follow-up, and participating sites that focus on PPCM may have better outcomes than sites where this diagnosis might initially be missed. Follow-up longer than 6 months would also be informative.
  4. Thromboembolic events occurred in 7% (including deep vein thrombosis, pulmonary embolism, arterial embolism, and ischemic stroke). Fewer than one sixth of the women in the registry were anticoagulated in the 6 months after discharge. Anticoagulation for PPCM is indicated for LV thrombus, and recommended for atrial fibrillation and/or severe LV dysfunction, but additional research is needed to better identify which patients should be anticoagulated. Other factors that could contribute to thromboembolic risk (i.e., deep vein thrombosis and pulmonary embolism) include immobilization due to severe heart failure or cesarean delivery.
  5. Other studies have observed that delays in diagnosis of PPCM are common, which may be related in part to the overlap of symptoms of heart failure with those of normal pregnancy. This study, however, found that fewer than two thirds of patients had pulmonary rales or peripheral edema, suggesting that the physical exam findings of pregnancy-associated heart failure may not be sensitive. In addition, one quarter of women with mild symptoms actually had severe LV dysfunction. Thus, a high index of suspicion is necessary and additional testing with B-type natriuretic peptide (BNP)/pro-BNP levels and an echocardiogram may be indicated.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Anticoagulation Management and Atrial Fibrillation, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, CHD and Pediatrics and Arrhythmias, Acute Heart Failure, Heart Failure and Cardiac Biomarkers

Keywords: Anticoagulants, Arrhythmias, Cardiac, Cardiomyopathies, Atrial Fibrillation, Brain Ischemia, Bromocriptine, Cardiomyopathies, Death, Sudden, Heart Failure, Infant, Newborn, Natriuretic Peptide, Brain, Peripartum Period, Pregnancy, Stroke, Stroke Volume, Thromboembolism, Ventricular Dysfunction, Left


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