FRCA Notes

Peripartum Cardiomyopathy

The curriculum mentions cardiomyopathy and medical conditions affecting parturients, although not necessarily as part of the same learning objective...

This page should be viewed alongside the overview page on cardiac disease in pregnancy.

Resources

  • Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy causing heart failure secondary to LV systolic dysfunction
  • It occurs towards the end of pregnancy or in the months following delivery, in the absence of another cause of heart failure
  • The classic definition requires:
    1. Development of heart failure in the last month of pregnancy, or up to 5 months post-partum
    2. LV systolic dysfunction on echocardiography: LVEF nearly always <45%
    3. No pre-existing heart failure or other identifiable cause
  • It is a diagnosis of exclusion; there is no specific confirmatory test
  • It often affects previously healthy women and symptoms overlap with normal pregnancy, so diagnosis is frequently delayed

  • Approximately 1 in 1000 live births, but with marked geographical and ethnic variation
    • Highest rates are seen in Nigeria (1:102) and Haiti (1:300)
    • Incidence appears to be rising in Western countries

Risk factors


Maternal Pregnancy-associated
African descent Multiple gestation, including via assisted reproduction (see twin pregnancy)
Advanced maternal age (>30 years) Hypertensive disorders of pregnancy, particularly pre-eclampsia
Obesity, diabetes and essential hypertension High gravidity and parity
Family history of cardiomyopathy Oral tocolytic therapy
Cocaine use

Genetics

  • There is shared genetic susceptibility with inherited (particularly dilated) cardiomyopathies
  • These variants may be clinically silent until unmasked by the haemodynamic stress of pregnancy
  • The 2025 ESC guidelines recommend genetic counselling and testing should be considered in all women with PPCM

  • Not fully understood, but likely a combination of genetic predisposition and oxidative stress

  • Pregnancy causes a systemic inflammatory response with oxidative stress, largely from the placenta
  • This is normally counterbalanced by antioxidant pathways (e.g. mitochondrial superoxide dismutase 2)
  • Where these protective pathways fail, proteases are activated which cleave prolactin into a 16 kDa prolactin fragment
  • This fragment is anti-angiogenic; it causes endothelial dysfunction, myocyte apoptosis and down-regulation of cardioprotective factors
  • Prolactin rises in late pregnancy and during breastfeeding, explaining the timing of presentation

  • Anti-angiogenic proteins from the placenta (e.g. sFLT-1, a potent VEGF inhibitor) are also raised, as they are in pre-eclampsia, suggesting shared mechanisms between the two conditions

Clinical features

  • Non-specific features of heart failure of varying severity; may be insidious over weeks or present acutely with cardiogenic shock
  • Around two-thirds present after delivery
  • Most have severe symptoms (NYHA III/IV) at presentation
  • Orthopnoea and PND, in addition to breathlessness, are more suggestive of PPCM than normal pregnancy

Differential diagnosis


Differential Distinguishing features
Pre-eclampsia Hypertension, proteinuria; pulmonary oedema is from diastolic (not systolic) dysfunction with preserved LV function
Pulmonary embolism Hypoxaemia, tachycardia; RV strain on ECG and RV impairment on TTE
Amniotic fluid embolism Acute onset with coagulopathy (low fibrinogen and platelets)
Pre-existing cardiomyopathy or congenital heart disease History of heart failure or evidence of LV impairment before pregnancy
Myocardial infarction (inc. SCAD) Typical presentation, ischaemic ECG changes, raised troponin
Takotsubo cardiomyopathy Acute trigger (e.g. stressful labour) or catecholamine exposure; typical echo appearances
Myocarditis Viral prodrome


ECG

  • Non-specific findings e.g. bundle branch block, ST depression, T wave inversion, ectopy, brady or tachyarrhythmias
  • A prolonged QTc is associated with LVEF <35%

CXR

  • Cardiomegaly and pulmonary congestion
  • Helps exclude other causes of dyspnoea e.g. pneumonia, pleural effusions
  • A normal CXR does not exclude PPCM

TTE

  • TTE is the gold standard; LV systolic dysfunction is the key finding
  • Mean LVEF at presentation is 30%; two-thirds have an LVEF <36%
  • May show LV dilatation with functional mitral regurgitation, RV dysfunction or LV thrombus

Other

  • BNP/NT-proBNP: normal or only slightly raised in normal pregnancy but markedly raised in heart failure
    • Recommended in pregnant women presenting with orthopnoea or PND
    • Best used to rule out rather than rule in heart failure, as there is no diagnostic threshold for PPCM
  • Cardiac MRI may exclude other causes (e.g. myocarditis, infiltrative disease, LV non-compaction); gadolinium is avoided in pregnancy

  • Full LV recovery (LVEF >50-55% at 6 months) occurs in only 21-63% depending on region
  • Early mortality: sudden cardiac death from arrhythmia, acute heart failure and thromboembolism
  • Late mortality: progressive LV dysfunction and chronic heart failure

Poor prognostic factors

  • LVEF <30% at diagnosis
  • LV dilatation (LVEDD >60mm)
  • RV dysfunction
  • LV thrombus
  • Delayed diagnosis (>1 week after symptom onset)
  • Prolonged QT interval
  • African ethnicity, obesity, previous PPCM

Risk classification

  • Under the mWHO 2.0 classification (ESC, 2025):
    • mWHO III: moderate LV impairment (EF 30-45%) or previous PPCM with no more than mild residual impairment
    • mWHO IV: severe LV impairment (EF <30% or NYHA III/IV) or previous PPCM with more than mild residual impairment
  • Both require care led by a Pregnancy Heart Team in an expert centre able to provide mechanical circulatory support

  • Mirrors that of other heart failure: supportive, medical and lifestyle measures, individualised to gestation and haemodynamic stability
  • Managed by a Pregnancy Heart Team: cardiologist, obstetrician, obstetric anaesthetist, maternal foetal medicine, midwifery ± intensivist, cardiothoracic anaesthetist, neonatologist, pharmacist
  • Decompensated or shocked patients should be admitted to ICU unless delivery is imminent
  • Steroids for foetal lung maturity if preterm delivery is anticipated (typically 24-34 weeks)

Heart failure drugs in pregnancy and lactation


Drug Pregnancy Lactation
Beta-blockers (e.g. metoprolol) Yes; monitor foetal growth (IUGR, bradycardia, hypoglycaemia) Yes
Loop diuretics Yes for congestion; caution over-diuresis reducing placental perfusion Yes; may reduce milk production
Hydralazine and nitrates Yes; afterload reduction in place of ACEi/ARB Yes (ACEi usually preferred post-partum)
Digoxin Yes Yes
ACEi, ARB, ARNI No; foetotoxic (renal failure, oligohydramnios, skull and limb defects) Enalapril and captopril can be used
MRAs, ivabradine, SGLT2 inhibitors No Spironolactone can be used

  • Guideline-directed heart failure therapy should continue for at least 12 months after complete LV recovery (ESC, 2025), tapered gradually and guided by LV function

Acute decompensation

  1. Optimise preload: cautious fluid challenge if underfilled; IV diuretics if congested; GTN infusion if SBP >110mmHg (monitor placental perfusion)
  2. Optimise oxygenation: supplemental oxygen, NIV, or intubation if refractory hypoxaemia or altered mental state
  3. Haemodynamic support: inotropes and/or vasopressors; ESC 2025 names levosimendan, dobutamine and milrinone as recommended agents in cardiogenic shock
  4. Urgent delivery if instability persists despite optimal management
  5. Consider bromocriptine after delivery
  6. Consider anticoagulation (caution if delivery is imminent)

Bromocriptine

  • Dopamine agonist which inhibits prolactin secretion, thus blocking production of the 16 kDa fragment
  • Associated with improved LV recovery in small studies, but larger trials are awaited; it may be considered in newly diagnosed PPCM
  • It stops lactation, which needs discussing with the patient
  • It is prothrombotic, so anticoagulation is given for the duration of its use

Anticoagulation

  • PPCM carries a high risk of LV thrombus and thromboembolism: the hypercoagulable state of pregnancy combined with LV systolic dysfunction
  • Prophylactic anticoagulation is generally recommended
  • Therapeutic anticoagulation if intracardiac thrombus, systemic embolism or AF; ESC 2025 also lists EF <35% as an indication for therapeutic LMWH
  • LMWH or UFH is preferred in pregnancy as urgent delivery may be needed; DOACs are not recommended in pregnancy or breastfeeding

Arrhythmias and devices

  • Risk of arrhythmia and sudden cardiac death, particularly if LVEF <35%
  • Acute management is as for non-pregnant patients; cardioversion and defibrillation are safe and should not be delayed (see arrhythmias in obstetrics)
  • LV function often recovers within months, so an ICD is usually unnecessary early on; a wearable cardioverter defibrillator may bridge this period
  • ICD is indicated for persistent severe LV dysfunction more than 6 months after diagnosis despite optimal therapy

Mechanical circulatory support and transplantation

  • Should be considered early if unstable despite maximal medical therapy; around 3% need a VAD
  • Device choice depends on oxygenation:
    • Adequate oxygenation: IABP or Impella
    • Cardiogenic shock: VA-ECMO
  • ECMO in pregnancy: 30 day maternal survival approximately 75% and foetal survival 65%; bleeding, thrombosis and infection are major complications
  • Transplantation is delayed where possible, as late recovery beyond 6 months can occur and post-transplant outcomes are poorer in PPCM

Peripartum management of the parturient with PPCM


  • An individualised plan for the ante-natal period, labour and delivery and the post-partum period should be agreed by the Pregnancy Heart Team
  • Delivery should be in an expert centre with access to ICU and mechanical circulatory support
  • Review current medications: patients may be on hydralazine, nitrates and β-blockers, as ACEi/ARB/MRAs are contraindicated in pregnancy
  • Timing and dose of anticoagulation must be planned around neuraxial techniques
  • In unstable disease, delivery may need to be expedited

Timing and mode of delivery

  • There is no strong evidence that early delivery improves outcomes in stable PPCM responding to medical therapy
  • Vaginal delivery is preferred: less blood loss, infection and thrombosis
  • Caesarean delivery is reserved for obstetric indications or severe heart failure (EF <30% and/or NYHA III/IV), uncontrolled arrhythmias or haemodynamic instability
  • Induction of labour is planned to allow timely cessation of anticoagulation, with contingency plans for deterioration or caesarean delivery
  • An assisted second stage (forceps or ventouse) is often recommended to reduce maternal effort

Monitoring

  • AAGBI standard monitoring with continuous pulse oximetry and ECG
  • Low threshold for arterial line: beat-to-beat BP and arterial gases
  • CVC only if vasoactive drugs cannot be given peripherally; routine CVP monitoring has little value
  • Bedside TTE and lung ultrasound to assess function, congestion and response to treatment
  • Close fluid balance with concentrated infusions (e.g. oxytocin)

Haemodynamic goals


Cardiac feature Goal of anaesthetic management
Heart rate and rhythm Avoid bradycardia (e.g. high neuraxial block, reflex bradycardia from pure vasoconstrictors) and marked tachycardia
Maintain sinus rhythm with rapid treatment of arrhythmia
Preload Maintain normovolaemia; left lateral tilt to avoid aortocaval compression
Avoid fluid overload (e.g. autotransfusion at delivery, excessive IV fluid); consider furosemide at delivery
Afterload Avoid increases in SVR (e.g. pain, inadequate analgesia)
Avoid precipitous falls in SVR by careful titration of neuraxial block
Contractility Avoid negative inotropy ± provide inotropic support
Other Correct deranged electrolytes

Labour analgesia

  • Early neuraxial analgesia is recommended, ideally before induction or in early labour
    • Attenuates the catecholamine surge and cardiac output fluctuations of contractions
    • Can be extended for caesarean delivery, avoiding GA
  • No neuraxial technique (epidural, CSE, DPE) has been shown to be superior
  • Slow, titrated, incremental dosing (e.g. bupivacaine 0.0625-0.125% over 10-20 minutes) with close observation
  • An adrenaline-containing test dose may be harmful; an opioid test dose (e.g. fentanyl 50μg) is an alternative
  • Maintenance with standard low-dose regimens e.g. bupivacaine 0.0625% with fentanyl 2μg/ml
  • Avoid routine fluid preloading; treat hypotension predominantly with vasopressors
    • Small doses of phenylephrine are effective in most
    • Inopressors (ephedrine or noradrenaline) should be considered in severe LV dysfunction

Caesarean delivery

  • Neuraxial anaesthesia is favoured, even in severe PPCM
    • Even a carefully titrated technique can be associated with significant haemodynamic compromise (as in this case series)
  • With an epidural in situ: incremental top-up (e.g. 2% lidocaine, 0.5% bupivacaine or levobupivacaine)
  • Without an epidural: low-dose sequential CSE e.g. intrathecal heavy bupivacaine ≤5mg, then slow epidural 2% lidocaine as required
  • Alternatives: epidural alone, or CSE with intrathecal opioid only followed by slow epidural local anaesthetic
  • Single-shot spinal is avoided owing to rapid sympathectomy
  • Vasopressors: inopressors are preferred over pure vasoconstrictors; low-concentration noradrenaline (5μg/ml) can be given peripherally and maintains HR and cardiac output better than phenylephrine
  • Dobutamine or milrinone may be needed depending on clinical condition

General anaesthesia

  • Indications: patient refusal, anticoagulation, or cardiorespiratory deterioration needing intubation
  • Arterial line before induction
  • Obtund the pressor response to laryngoscopy e.g. remifentanil or alfentanil
  • Titrate induction agents cautiously to avoid myocardial depression and hypotension

Uterotonics

  • Active management of the third stage is recommended as PPH is more common and poorly tolerated
  • Oxytocin is first line, but a rapid bolus causes tachycardia, ↓ SVR and MAP, and can cause pulmonary vasoconstriction
    • Give by slow infusion (e.g. 2 IU over 10 minutes) rather than bolus
  • Second-line agents should be decided by the Pregnancy Heart Team in advance:
    • Carboprost: ↑ PVR by >100%, bronchospasm and hypoxaemia
    • Ergometrine: ↑ SVR, ↑ PVR and coronary vasospasm
    • Both are relative contraindications; may be used cautiously, possibly at reduced dose
    • Misoprostol is less effective but has fewer cardiovascular effects
  • Tranexamic acid can be used unless there is coexisting thrombosis
  • Early surgical measures e.g. compression sutures, balloon tamponade

  • Women with severe PPCM are at high risk of deterioration shortly after delivery
    • Autotransfusion and relief of aortocaval compression rapidly increase preload, which may precipitate decompensated heart failure
  • HDU or ICU care with ongoing monitoring
  • VTE prophylaxis, or restarting therapeutic anticoagulation when safe
  • Introduce or optimise guideline-directed heart failure therapy (ACEi/ARNI become available after delivery)
  • Ongoing cardiology follow-up
  • Women with PPCM are particularly vulnerable to post-partum depression

Breastfeeding

  • The decision is individualised
  • Stopping lactation (± bromocriptine) reduces prolactin levels, reduces metabolic demand in severe heart failure and allows full medical therapy
  • However, there is little evidence that breastfeeding worsens outcomes, and it has clear benefits for mother and infant

Subsequent pregnancy

  • Recurrence risk depends mainly on residual LV function, but is increased even after full recovery
  • The 2025 ESC guidelines do not recommend subsequent pregnancy if LV function has not normalised
  • Any future pregnancy requires pre-conception counselling and expert MDT care throughout, with serial echo and BNP
  • Contraceptive advice should be given