Peripartum cardiomyopathy (PPCM) is a rare form of heart failure occurring towards the end of pregnancy or within five months postpartum, characterized by left ventricular systolic dysfunction. Its incidence varies widely globally and is influenced by factors such as maternal age and multiple gestations. Management includes symptomatic treatment, careful monitoring, and potential chronic therapy, with a focus on improving long-term outcomes even as recovery rates can vary.

1. Peripartum
cardiomyopathy
Dr. Somnath Mukhopadhay

2. It took 80 years to be recognized!
 Pregnancy associated CMP
 Meadow’s Syndrome
 Zaria Syndrome
 Postpartum myocardiosis
 Peripartum CMP

3. Definition
 2010 ESC Group defined PPCM as an idiopathic cardiomyopathy with
following:
1. Development of HF toward the end of pregnancy or within 5 months following
delivery.
2. Absence of another identifiable cause for the HF.
3. LV systolic dysfunction with an EF less than 45%. The LV may or may not be
dilated.

4. Epidemiology
 1:100 in Zaria, Nigeria (local Hausa custom of eating Kanwa, a dry lake salt
for 40 days after delivery, may lead to hypervolemia, HTN and finally PPCM)
 1:300 in Haiti.
 1:20,000 in Japan.
 1:4025 in India (acc. to latest data on 23/3/2018)
 Global incidence increased, may be due to improved diagnosis.
 Increased maternal age, Pre-eclampsia, maternal CV risk factors and multiple
gestations are other causes of raised incidence.

5. Etiology : unknown, multifactorial
 Final common pathway – increased oxidative stress, cleavage of prolactin to
angiostatic NT 16 kDA fragment and impaired VEGF signalling.
 Angiogenic imbalance : reduced VEGF in late gestation due to upregulated
sFLT1.
 Prolactin : 16K PRL causes endothelial damage and myocardial dysfunction.
 Inflammatory cytokines : increased TNF, IL-6, CRP.
 Myocarditis : role is uncertain.
 Abnormal immune response: escaped fetal cell lodge in cardiac tissue.
 Genetic predisposition: GNB3 has polymorphism of C825T
 Hemodynamic factor: exaggerated response of LV remodelling & hypertrophy.

6. Risk factors
 Age more than 30 years.
 African descent.
 Pregnancy with multiple fetus.
 History of PE/eclampsia/ postpartum HTN.
 Cocaine abuse.
 More than 4 weeks oral tocolytics with beta agonists like terbutaline.
 Relationship with DM is confounded by other risk factors.
 Selenium deficiency has conflicting data.

7. Clinical manifestation
 Timing : rare before 36 weeks.
 Sign/symptoms of HF.
 Initial diagnosis may be delayed since nonspecific fatigue, SOB and pedal
oedema simulates normal pregnancy.
 LV thrombus was seen in echocardiography in 16 out of 100 patients with
mean LVEF of 26% in a series.

8. Diagnosis
 LVEF<45% was added in definition to prevent inclusion of disorders those
mimic systolic HF like accelerated HTN, pulmonary embolism, systemic
infection, severe diastolic dysfunction, amniotic fluid embolism or pre-
eclampsia.
 BNP/CXR/CMRI/EMB/catheterization – only in selected cases.
 Viral/bacterial culture or viral titre like Coxsackie B are not indicated.
 Variable presence of LGE reflects diverse process. Persistence of LGE denotes
poor recovery.
 No pathognomonic EMB finding is seen ,till now, in PPCM.

9. Management : HF treatment
 Oxygen supplementation and assisted ventilation as per need.
 Optimization of preload.
 Hemodynamic support: inotropes, vasopressor.
 Symptomatic relief: diuretics.
 When possible, institute chronic therapies to improve long term outcome.
 15% to 20% with PPHF carry mutation known to induce CMP.
 Patient with genetic anomaly may have a predisposition to develop HF
triggered by physiological stress of pregnancy and delivery.

10. Arrhythmia management
 Arrhythmia is seen in 18.7% patients in a large inpatient database study of
9841 hospitalizations. 4.2% are having VT, 2.2% are having cardiac arrest.
 Smaller series revealed 20% to 25% cardiac arrest.
 Atrial fibrillation is usually seen in 3.1% to 11.9% of patients.
 Specific indications for ICD are not established in PPCM. 20% to 70% PPCM
usually recovers to normal by 3 to 12 months. So ICD is to be deferred, so also
CRT.
 Wearable defibrillator again is not without its risk or concern.

11. Anticoagulation issues
 The data for using anticoagulation is inconclusive for reducing events or
mortality. Exception is bromocriptine treated PPCM patients as MI/stroke
occurred with bromocriptine.
 LV systolic dysfunction with or without HF, without LV thrombus or other
indications, anticoagulation is not recommended recently.
 Definite indication of anticoagulation in PPCM and sinus rhythm is
1. Intracardiac thrombus.
2. Evidence of systemic embolism.

12. Advanced management
 MCS should be considered as bridge to bridge or bridge to recovery.
 Temporary device should always be preferred.
 Venoarterial ECMO may lead to increased prolactin. In these cases
bromocriptine (10mg BD) is helpful.
 Heart transplant in PPCM has worse outcome. Increased mortality, increased
rejection, poor graft survival and increased rate of re-implantation occurred
in different studies.
 Immunosuppression : not recommended.
 IVIg : efficacy is not confirmed.
 Apheresis and treatment with miRNA 146a are upcoming.

13. Role of bromocriptine
 Reduces prolactin production by dopamine agonist action.
 Role is controversial. May improve outcome by eliminating the cleaved formof
prolactin despite the activation of the cleaving enzyme.
 BRO-HF concluded that it was independently associated greater LV functional
recovery at 6 months and at long term follow up.
 Dose : 2.5mg twice daily for 2 weeks, followed by 2.5 mg once daily for 6
weeks.
 2019 ESC position statement from HF Association gave weak recommendation
and suggested Cabergoline as an alternative.

14. Delivery
 Hemodynamically stable : vaginal delivery with epidural anaesthesia.
 Hemodynamic instability: planned CS with central neuraxial anaesthesia.
 Early delivery is not required if maternal and fetal conditions are stable.

15. Breastfeeding
 Prevention of lactation is needed for severe HF due to harmful effects of
prolactin subfragments and high metabolic demand of lactation and
breastfeeding.
 If clinically stable then breastfeeding can be done as long as it is compatible
with heart failure treatment.
 If breastfeeding is decided then ACEI/ARB should be avoided.

16. Contraception must be reliable
 Persistent LV dysfunction can lead to high risk of recurrence.
 Sterilization may be required for patient and/or partner.
 Oestrogen containing contraceptives are avoided due to enhanced risk of
thromboembolism.

17. Prognosis
 Recovery of LV function usually takes 6 months but may take even 5 years.
 Mortality : 3% to 9.6%.
 Complete recovery-50%; persistent symptom-25%; complication-25%.
 Recovered patients may have subtle systolic or diastolic dysfunction with
reduction of maximum exercise capacity.
 Extra-cardiac morbidity may occur in the form of brain injury, CVA or
cardiopulmonary arrest.
 Persistent LVEF > 50% for more than 6 months can allow stepwise weaning of
HF therapy.
 Echocardiography should be done 6 monthly.

18. Adverse prognostic factor
 Increased NYHA classes.
 LVEF </=25%.
 Black race.
 Indigent status.
 Multiparity.
 30 to 35 years of age.

19. Predictors of persistent LV Dysfunction
 LVEF <30%.
 Fractional shortening < 20%.
 LVIDd >/= 6 cm.
 Reduced RV function.
 Raised troponin-T.
 Black race.
 Diagnosed during pregnancy.

20. Thank you