Peripartum Cardiomyopathy (PPCM)

Definition

Peripartum cardiomyopathy is an idiopathic cardiomyopathy presenting with heart failure due to left ventricular systolic dysfunction toward the end of pregnancy or in the months following delivery, without any other identifiable cause of cardiomyopathy.

Diagnostic Criteria (Classical)

All must be present:

  1. Heart failure develops in last month of pregnancy or within 5 months postpartum
  2. No identifiable cause of heart failure
  3. No pre-existing heart disease
  4. LV systolic dysfunction
    • LVEF <45%, or
    • Fractional shortening <30%, or
    • LV end-diastolic dimension >2.7 cm/m²

(ESC now accepts presentation anytime from late pregnancy to several months postpartum)


Epidemiology

  • Incidence varies widely:
    • India/Africa: higher incidence (1:300–1:1000 deliveries)
    • Western countries: ~1:2500–1:4000
  • High mortality and morbidity
  • Major cause of maternal heart failure–related death


Risk Factors

Maternal

  • Advanced maternal age (>30–35 yrs)
  • Multiparity
  • Multiple gestation
  • African/Asian ethnicity
  • Obesity
  • Smoking

Obstetric

  • Preeclampsia / eclampsia
  • Gestational hypertension
  • Prolonged tocolysis
  • Cesarean section

Medical

  • Viral myocarditis
  • Nutritional deficiencies (selenium)
  • Genetic predisposition (TTN mutations)


Pathophysiology (HIGH-YIELD)

PPCM is multifactorial, involving vascular, hormonal, inflammatory, and genetic mechanisms.


1. Prolactin Hypothesis (MOST EXAM-IMPORTANT)

  • Late pregnancy → oxidative stress
  • Activates cathepsin D
  • Cleaves prolactin (23 kDa) into 16-kDa prolactin fragment
  • 16-kDa prolactin is:
    • Anti-angiogenic
    • Pro-apoptotic
    • Pro-inflammatory
  • Causes:
    • Endothelial dysfunction
    • Myocyte apoptosis
    • Microvascular ischemia

👉 Basis for bromocriptine therapy


2. Anti-angiogenic Imbalance

  • Increased sFlt-1 (anti-VEGF factor)
  • Decreased VEGF signaling
  • Impaired myocardial capillary density

(Explains association with preeclampsia)


3. Inflammatory & Autoimmune Mechanisms

  • Elevated cytokines: TNF-α, IL-6
  • Autoantibodies against cardiac proteins
  • Myocarditis-like injury


4. Genetic Predisposition

  • Overlap with dilated cardiomyopathy
  • Truncating mutations in TTN gene
  • Explains recurrence and familial clustering


Clinical Presentation

Symptoms

  • Dyspnea (rest/exertion)
  • Orthopnea, PND
  • Fatigue
  • Palpitations
  • Chest pain (rare)
  • Syncope (severe cases)

Signs

  • Tachycardia
  • Hypotension
  • Elevated JVP
  • S3 gallop
  • Pulmonary crepitations
  • Peripheral edema
  • Cardiogenic shock (severe PPCM)

Often misdiagnosed as normal pregnancy-related dyspnea


Investigations

1. ECG

  • Sinus tachycardia
  • Nonspecific ST-T changes
  • Atrial or ventricular arrhythmias
  • QRS prolongation (poor prognosis)


2. Chest X-ray

  • Cardiomegaly
  • Pulmonary edema
  • Pleural effusions


3. Echocardiography (KEY DIAGNOSTIC TOOL)

  • LVEF <45%
  • Global LV hypokinesia
  • LV dilatation (variable)
  • RV dysfunction (poor prognostic sign)
  • Functional MR/TR
  • Intracardiac thrombus (common)


4. Biomarkers

  • BNP / NT-proBNP ↑
  • Troponin: usually normal or mildly elevated


5. Cardiac MRI

  • Helps exclude myocarditis
  • Late gadolinium enhancement:
    • Usually absent or minimal in PPCM
    • Extensive LGE → poor recovery


Differential Diagnosis

  • Dilated cardiomyopathy
  • Acute myocarditis
  • Valvular heart disease
  • Pulmonary embolism
  • Amniotic fluid embolism
  • Severe preeclampsia-related heart failure


Management

General Principles

  • Multidisciplinary: cardiology + obstetrics + ICU
  • Treat as acute HFrEF
  • Balance maternal benefit and fetal safety


Medical Management

A. Standard Heart Failure Therapy

Postpartum

  • ACE inhibitors / ARBs
  • Beta-blockers (metoprolol, carvedilol)
  • Mineralocorticoid receptor antagonists
  • Loop diuretics
  • Vasodilators

Antepartum

Drug

Use

Loop diuretics

(cautious)

Beta-blockers

(metoprolol preferred)

Hydralazine + nitrates

✔

ACEI / ARB / ARNI

contraindicated

MRA

contraindicated


B. Bromocriptine (HIGH-YIELD)

  • Dopamine agonist → blocks prolactin
  • Improves LV recovery
  • ESC recommends adjunctive therapy

Requires anticoagulation (↑ thrombotic risk)

Typical regimen:

  • 2.5 mg BID × 2 weeks → 2.5 mg OD × 6 weeks (varies by protocol)


C. Anticoagulation

Indications:

  • LVEF <35%
  • LV thrombus
  • Bromocriptine use

Options:

  • LMWH (antepartum)
  • Warfarin (postpartum)
  • DOACs: limited data


Advanced & ICU Management

1. Acute Decompensated HF / Shock

  • Oxygen / NIV / mechanical ventilation
  • Inotropes (dobutamine, milrinone)
  • Vasopressors if needed


2. Mechanical Circulatory Support

  • IABP
  • Impella
  • VA-ECMO
  • LVAD (bridge to recovery or transplant)


3. Arrhythmia Management

  • AF, VT common
  • Temporary pacing / ICD if persistent severe LV dysfunction (>3–6 months)


Prognosis

Recovery

  • ~50–70% recover normal LV function within 6 months
  • Recovery may continue up to 1–2 years

Poor Prognostic Factors

  • LVEF <30%
  • LVEDD >6 cm
  • RV dysfunction
  • Delayed diagnosis
  • African/Asian ethnicity
  • High NT-proBNP
  • Presence of LGE on MRI


Future Pregnancy Counseling 

If LV function recovers

  • Still risk of recurrence (20–30%)
  • Requires close monitoring

If LV dysfunction persists

  • Pregnancy contraindicated
  • High risk of:
    • Maternal death
    • Refractory HF
    • Fetal loss


Key Exam Pearls

  • PPCM is diagnosis of exclusion
  • 16-kDa prolactin is central to pathogenesis
  • Bromocriptine + anticoagulation is unique to PPCM
  • Major cause of maternal HF postpartum
  • Recovery possible → unlike idiopathic DCM
  • Strong association with preeclampsia