Peripartum Cardiomyopathy (PPCM)

๐Ÿ”ท Definition

Peripartum cardiomyopathy (PPCM) is a rare form of idiopathic dilated cardiomyopathy that occurs during the last month of pregnancy or within 5 months postpartum in previously healthy women, characterized by left ventricular systolic dysfunction.

๐Ÿง  Key Point: LV ejection fraction (LVEF) is <45% with or without left ventricular dilatation.


๐Ÿงช Diagnostic Criteria (as per NIH/European Society of Cardiology):

All 4 criteria must be met:

  1. Development of heart failure in the last month of pregnancy or within 5 months postpartum.
  2. Absence of another identifiable cause of heart failure.
  3. No prior history of heart disease.
  4. Echocardiographic evidence of LV systolic dysfunction:
    • LVEF <45%, or
    • LV fractional shortening <30%, or
    • LV end-diastolic dimension >2.7 cm/mยฒ

๐Ÿง  Pathophysiology

Exact cause is unknown, but multiple hypotheses:

Theory

Explanation

Prolactin hypothesis

Oxidative stress leads to cleavage of prolactin into a toxic 16 kDa fragment โ†’ endothelial dysfunction, apoptosis

Inflammatory/autoimmune

โ†‘ Inflammatory cytokines (TNF-ฮฑ, IL-6), myocarditis

Genetic susceptibility

TTN gene mutations found in some cases

Angiogenic imbalance

โ†“ VEGF and โ†‘ anti-angiogenic factors (sFLT1) โ†’ myocardial ischemia

Hemodynamic stress

Pregnancy-induced volume overload + hormonal changes precipitate dysfunction


โš ๏ธ Risk Factors

  • Age > 30
  • Multiparity
  • African ethnicity
  • Pregnancy-induced hypertension
  • Pre-eclampsia/eclampsia
  • Twin or multiple gestation
  • Prolonged tocolysis
  • Smoking, selenium deficiency, cocaine


๐Ÿ’‰ Clinical Features

System

Symptoms

Cardiovascular

Dyspnea, orthopnea, PND, palpitations, chest pain

Respiratory

Cough, hemoptysis, pulmonary edema

Systemic

Fatigue, edema, ascites

Neurological

Syncope, altered sensorium (if cerebral hypoperfusion)

Fetal

Preterm delivery, IUGR, fetal demise (in severe maternal compromise)


๐Ÿงช Investigations

Modality

Findings

ECG

Non-specific: sinus tachycardia, T wave inversion, LVH

Chest X-ray

Cardiomegaly, pulmonary edema

Echocardiography

Dilated LV, โ†“ LVEF <45%, โ†“ FS, wall motion abnormalities

BNP/NT-proBNP

Elevated

Cardiac MRI

Assesses myocardial inflammation/fibrosis

Labs

CBC, LFTs, RFTs, troponins, D-dimer (rule out PE)


๐Ÿ” Differential Diagnosis

  • Myocarditis
  • Ischemic heart disease
  • Pulmonary embolism
  • Pre-eclampsia with pulmonary edema
  • Takotsubo cardiomyopathy
  • Thyrotoxic cardiomyopathy


๐Ÿ“Š Prognosis

  • Recovery in 50โ€“70% within 6 months
  • Risk of mortality: 5โ€“20%
  • Poor prognostic markers:
    • EF <30%
    • LVEDD >6 cm
    • Delayed diagnosis
    • Persistent dysfunction at 6 months


๐Ÿง  Management Principles

Multidisciplinary approach: Cardiology + Obstetrics + Anesthesiology + Critical Care


โš•๏ธ Medical Management

Drug Class

Preferred Agent

Diuretics

Furosemide (relieves pulmonary congestion)

Vasodilators

Hydralazine, nitrates (โ†“ afterload)

Beta-blockers

Metoprolol, bisoprolol (AVOID if acute decompensation)

ACE Inhibitors/ARBs

ONLY postpartum, contraindicated during pregnancy

Digoxin

Inotropic support, rate control

Anticoagulation

Consider if EF <35%, thrombus, arrhythmia

Ivabradine

HR control if ฮฒ-blockers not tolerated

Bromocriptine

Experimental; inhibits prolactin release โ€“ shown to aid recovery in some studies


๐Ÿ’‰ Advanced Therapies

  • ICU monitoring if pulmonary edema, arrhythmia, or shock
  • Mechanical ventilation if severe respiratory distress
  • Inotropes (dobutamine, milrinone) if cardiogenic shock
  • LVAD or heart transplant for refractory cases


๐Ÿ’ฌ Viva Q&A

Q: What is the role of bromocriptine in PPCM?
๐Ÿ—จ๏ธ Inhibits prolactin secretion โ†’ reduces formation of toxic 16 kDa prolactin โ†’ may help recovery.

Q: Why avoid ACE inhibitors in pregnancy?
๐Ÿ—จ๏ธ Risk of fetal renal agenesis, oligohydramnios, IUGR, death.

Q: What is the risk of recurrence in next pregnancy?
๐Ÿ—จ๏ธ 20โ€“50% if recovered EF; >60% if EF <50% postpartum. Recurrence can be fatal.


๐Ÿ“š MCQ Pearls

  1. Earliest sign of PPCM: Fatigue and dyspnea
  2. Treatment during pregnancy: Beta-blockers + hydralazine + diuretics
  3. Postpartum ACE inhibitor of choice: Enalapril
  4. Most dangerous complication: Cardiogenic shock
  5. Definitive treatment in refractory case: Heart transplant