Peripartum Cardiomyopathy. Update 2016

Peripartum cardiomyopathy Potentially life-threatening pregnancy-associated disease that typically arises in the peripartum period and is marked by left ventricular dysfunction and heart failure. Relatively uncommon, but its incidence is rising. Women often recover cardiac function, but long-lasting morbidity and mortality are not infrequent. (Circulation. 2016;133:

Defination An idiopathic cardiomyopathy frequently presenting with heart failure secondary to LV systolic dysfunction (LVEF <45%) towards the end of pregnancy or in the months following delivery, if no other cause of heart failure is found

Epidemiology Data in Africa and Asia suggest an incidence of ≈1 in 1000 live births In Haiti, the incidence of PPCM may be as much as 1 in 300 live births In northern Nigeria, the incidence of PPCM has been reported as high as 1 in 100 live births Recent multi-institutional estimates of peripartum cardiomyopathy (PPCM) incidence in the United States. (Circulation. 2016;133:

Associated Conditions Age – >50% of cases occur in women >30 years of age, Preeclampsia and Hypertension – Preeclampsia and hypertension strongly predispose to PPCM. Multiple Gestations – PPCM frequently presents in cases of multigestational status Other – substance abuse, anemia, asthma, prolonged tocolysis, diabetes mellitus, obesity, and malnutrition. (Circulation. 2016;133:

Comparison of timing during and after pregnancy of hemodynamic changes, exemplified as cardiac output (CO; in black), elevations in prolactin and soluble Fms-like tyrosine kinase 1 (sFlt1) hormones (red), and incidence of peripartum cardiomyopathy (PPCM; blue bars). (Circulation. 2016;133:

Vasculo-hormonal hypothesis of the pathophysiology of peripartum cardiomyopathy (PPCM). (Circulation. 2016;133:

J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Defination The Working Group on PPCM of the Heart Failure Association (HFA) of the European Society of Cardiology (ESC) recently proposed a new simplified definition of PPCM as an idiopathic cardiomyopathy frequently presenting with heart failure secondary to LV systolic dysfunction (LVEF <45%) towards the end of pregnancy or in the months following delivery, if no other cause of heart failure is found. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Clinical presentation of acute peripartum cardiomyopathy Most patients admitted with PPCM present typical symptoms of AHF associated with signs of congestion

Evaluation of acute peripartum cardiomyopathy As for any AHF, initial evaluation of patients with suspected acute PPCM includes two parts, : – Evaluation of cardiopulmonary distress; – Confirmation of the diagnosis with additional tests. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Evaluation of cardiopulmonary distress The presence of criteria defining cardiopulmonary distress should lead to intensive cardiac care unit admission: Haemodynamic instability (systolic blood pressure 130 b.P.M. Or <45 b.P.M.), Respiratory distress (respiratory rate >25/min; peripheral oxygen saturation <90%), signs of tissue hypoperfusion with abnormal cellular oxygen metabolism (increased blood lactate >2.0 mmol/L; low central–venous oxygen saturation <60%, if available; altered mental state; cold, clammy, mottled skin; oliguria <0.5 mL/kg/h) J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Confirmation of the diagnosis An ECG s in all suspected PPCM (has high negative predictive value) Echocardiography is indicated in all cases of suspected PPCM ; – To confirm the diagnosis, – Assess concomitant or pre-existing cardiac disease, – Exclude complications of PPCM (e.G. LV thrombus), – Obtain prognostic information. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Peculiarities in the management of acute heart failure caused by peripartum cardiomyopathy Multidisciplinary approach with focus on health of mother and foetus. Avoidance of heart failure (HF) drugs with foetal toxicity during pregnancy (i.e. ACE inhibitors/ARBs, mineralocorticoid receptor antagonists) and breastfeeding; thereafter standard HF therapy. Consideration of bromocriptine (2.5 mg twice daily for 2 weeks, followed by 2.5 mg per day for 6 weeks) in addition to standard HF therapy. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Peculiarities in the management of acute heart failure caused by peripartum cardiomyopathy Anticoagulation with heparin to avoid cardio-embolic complications in patients with LVEF ≤35% or treated with bromocriptine (if no contraindication exists). In the case of cardiogenic shock, consideration of levosimendan (0.1 μg/kg/min for 24 h) instead of catecholamines as first-line inotropic drug. Early transfer to experienced centre. Early evaluation of mechanical circulatory support according to the centre’s experience. Prevention of sudden cardiac death, early consideration of wearable cardioverter-defibrillator devices in patients with LVEF ≤35%. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Example of prespecified protocol of interdisciplinary work-up for acute heart failure (AHF) during pregnancy

Algorithm for initial management. J. Bauersachs et al. Current management of patients with severe acute peripartum cardiomyopathy. European Journal of Heart Failure (2016)

Example of the management of a peripartum cardiomyopathy (PPCM) patient with cardiogenic shock in the intensive care unit (ICU).

Young woman with severe cardiac decompensation 22 years of age without known previous diseases She had given spontanaeous birth to a healthy girl 4 months earlier (primigravida, primapara) Fatigue and progressive dyspnea at exertion since delivery and since 4 days she is suffering from dyspnea at rest Visited her GP several times: „No acute need for action “ No clinical signs of infections in recent days/weeks Presentation with cardiac decompensation to rural hospital with rapid development of cardiogenic shock, necessity of intubation and mechanical ventilation, start of catecholamine therapy Transfer to our hospital On admission BP 50/20 mmHG with high dose catecholamine therapy (dobutamin, suprarenin, noradrenalin)

Young woman with severe cardiac decompensation Echocardiography at admission

Young woman with severe cardiac decompensation Angiography ruled out coronary heart disease

Diagnosis: Peripartum Cardiomyopathy

Management & counseling in PPCM Women with HF during pregnancy should be treated according to current major society guidelines for HF generally, except for avoiding contraindicated medications such as – Angiotensin converting enzyme inhibitors, – Angiotensin II receptor blockers, – Aldosterone antagonists All women with PPCM should receive counseling on the potential risk of recurrence with future pregnancies. Women with peripartum cardiomyopathy (PPCM) or history of PPCM should receive counseling regarding risk of recurrence and family planning and contraception options. Breastfeeding be avoided because of the potential effects of prolactin subfragments