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32.27 Peripartum cardiomyopathy
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Published:July 2018
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This version:November 2019
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Abstract
Pregnancy-related heart disease is increasing worldwide and peripartum cardiomyopathy (PPCM) is an important contributor to early (<42 days postpartum) and late (up to 1 year postpartum) maternal death. PPCM is a potentially life-threatening condition presenting with heart failure secondary to left ventricular dysfunction towards the end of pregnancy, or in the months following delivery, where no other cause of heart failure is identified. It is a diagnosis of exclusion. Incidence and prognosis varies according to geography and is likely due to multiple factors including also inherited or acquired cardiomyopathy-associated mutations. It seems that PPCM also shares common pathological pathways. For example, a ‘multiple-hit’ model including systemic angiogenic imbalance that derives from the oxidative stress-cathepsin D-16 kDa prolactin cascade and additional antiangiogenic factors plays a key role in the development of PPCM in experimental models and in humans suggests that a therapeutic approach involving blockade of this pathway with bromocriptine may be a novel disease-specific approach. Despite ongoing research, numerous uncertainties regarding the incidence, pathophysiology, treatment, and prognosis of PPCM patients remain, indicating the need for further investigation. The ongoing global registry on PPCM, under the umbrella of the EuroObservational research programme, has provided novel information.
April 23, 2020: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.
Update:
The Heart Failure Association of the ...More
April 23, 2020: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.
Update:
The Heart Failure Association of the European Society of Cardiology (ESC) has published a new position statement, ‘Pathophysiology, diagnosis and management of peripartum cardiomyopathy’, which includes new treatment recommendations.
The Heart Failure Association of the ESC has published a new practical guidance paper on long-term prognosis, subsequent pregnancy, and contraception that aims to assist the practitioner who is dealing with patients diagnosed with peripartum cardiomyopathy (PPCM).
Clinical characteristics of patients from the worldwide registry on PPCM recruited via the EURObervational Research Programme demonstrated that PPCM is a global disease and highlighted the high risk of thromboembolic events.
The 2018 ESC guidelines for the management of cardiovascular diseases during pregnancy included treatment with bromocriptine in management recommendations of patients with PPCM (class IIb recommendation).
Clinical trial and registries reporting on disease-specific therapies, that is, the use of prolactin blockers, are described.
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