Learning objectives

• Describe the risk factors and symptoms of peripartum cardiomyopathy
• Diagnose peripartum cardiomyopathy
• Manage patients with peripartum cardiomyopathy

Background

• Peripartum cardiomyopathy (PPCM) is a rare cause of cardiomyopathy occurring during late pregnancy or in the early postpartum period
• Characterized by significant left ventricular dysfunction and heart failure in the peripartum period in the absence of other identifiable causes of heart failure
• Potentially life-threatening condition
• Left ventricle ejection fraction is nearly always less than 45%
• Etiology is unclear but likely multifactorial (hormonal, inflammatory, genetic,…)

Risk factors

• African descent
• Increasing age
• Pregnancy-related hypertension
• Multiparity
• Multiple gestations
• Obesity
• Chronic hypertension
• Chronic tocolytics use
• Cocaine use

Signs & Symptoms

• Paroxysmal nocturnal dyspnea
• Pedal edema
• Orthopnea
• Dyspnea on exertion
• Dry cough
• Palpitations
• Increase in abdominal girth
• Lightheadedness
• Chest pain
• Jugular venous distentions
• Displaced apical impulse
• Third heart sound
• Mitral regurgitation murmurs

Diagnosis

• Diagnosis is based on exclusion
• Differential diagnoses:
  • Pulmonary embolism 
  • Severe sepsis 
  • Amniotic fluid embolism 
  • Preeclampsia/pregnancy-induced hypertensive disease 
  • Arrhythmias 
  • Severe anemia 
  • Myocardial infarction 
  • Dilated cardiomyopathy of other etiologies 
• Diagnostic tests:
  • Routine blood tests 
    • Evaluate for anemia, electrolyte abnormalities, endocrine conditions, renal or liver dysfunction
    • Brain natriuretic peptide (BNP) is commonly elevated in patients with heart failure and PPCM
  • Chest radiography
    • Cardiomegaly and/or pulmonary edema are suggestive of heart failure but nonspecific to PPCM
  • ECG 
    • Sinus tachycardia, supraventricular tachycardia, ventricular tachycardia, ST segment and T wave abnormalities, dilation of chambers, and QRS prolongation may be observed but are nonspecific
  • Echocardiography
    • Left ventricular ejection fraction <45%: Requirement for PPCM diagnosis
    • Evaluation of other etiologies such as valvular diseases or structural abnormalities
    • Ventricle/atrium dilation and left ventricular thrombus or atrial thrombosis may be present
  • Cardiac MRI
    • Evaluation of other causes of heart failure
  • Cardiac catheterization
    • Only for selected patients
    • Left heart catheterization is indicated in patients with suspected ischemic cardiomyopathy

Management

Management is similar to that of other causes of heart failure

• Pharmacological
  • Angiotensin-converting enzyme (ACE) inhibitors: First-line treatment postpartum, contraindicated during pregnancy
  • Hydrazaline and nitrate therapy can be used safely during pregnancy
  • Beta-blockers
  • Digoxin (carefully monitor plasma levels)
  • Loop diuretics (e.g., furosemide)
  • Avoid calcium channel blockers and aldosterone antagonists
  • Thromboprophylactic low molecular weight heparin
• Non-pharmacological:
  • Non-invasive ventilation or intubation
  • Inotropic support
  • Intra-aortic balloon pump, left ventricular assist device or extracorporeal membrane oxygenation may be required in severe cases
  • Heart transplantation in severe cases who do not respond to therapy
  • Implantable defibrillator or cardiac resynchronization in patients with chronic functional impairment

Prognosis

Complications

• Thromboembolism
• Arrhythmias
• Progressive heart failure
• Misdiagnosis as preeclampsia
• Fetal distress from hypoxia

Anesthetic management

Suggested reading

• Rodriguez Ziccardi M, Siddique MS. Peripartum Cardiomyopathy. [Updated 2022 Jul 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482185/
• Honigberg MC, Givertz MM. Peripartum cardiomyopathy. BMJ. 2019;364:k5287. Published 2019 Jan 30. doi:10.1136/bmj.k5287
• Thompson L, Hartsilver E. Peripartum cardiomyopathy. WFSA. https://resources.wfsahq.org/atotw/peripartum-cardiomyopathy/#:~:text=Titrated%20neuraxial%20anaesthesia%2C%20by%20incremental,agents%20that%20reduce%20myocardial%20contractility. Published February 24, 2015. Accessed February 13, 2023. 

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