Peripartum cardiomyopathy

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

PPCM is a diagnosis of exclusion in a high-risk physiological period. Because symptoms overlap normal pregnancy and thromboembolism, diagnosis depends on urgent clinical assessment, natriuretic peptide testing and echocardiography rather than reassurance from age or an uncomplicated pregnancy.

Care should be led by a Pregnancy Heart Team spanning cardiology, obstetric medicine, maternal-fetal medicine, anaesthesia and neonatology. Stabilisation, delivery timing and medication compatibility are considered together.

Prognosis tracks initial LV/RV severity and recovery. Persistent dysfunction requires chronic HF/device care; even complete recovery requires future-pregnancy counselling and surveillance.

Key points

PPCM is otherwise-unexplained heart failure from LV systolic dysfunction, usually LVEF below 45%, towards the end of pregnancy or in the months after birth.
Orthopnoea, PND, resting tachycardia, raised JVP, hypoxia and pulmonary oedema are not normal pregnancy discomforts.
TTE confirms ventricular dysfunction and identifies RV involvement, valve regurgitation and intracardiac thrombus.
Always exclude pre-eclampsia-related pulmonary oedema, PE, ACS/SCAD, myocarditis, sepsis and previously unrecognised cardiomyopathy or valve disease.
Treatment during pregnancy differs from postpartum HFrEF: ACE inhibitors, ARBs, ARNIs, MRAs and SGLT2 inhibitors are avoided during pregnancy.
Severe dysfunction and the postpartum state increase thromboembolic risk; anticoagulation is individualised by LVEF, thrombus, AF and bromocriptine use.
Recovery may take months and does not abolish relapse risk; continue follow-up and counsel before any later pregnancy.
Bromocriptine is not routine lactation suppression and has important MHRA hypertension/cardiovascular contraindications; any PPCM use is specialist and off-label.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

Proposed pregnancy-related myocardial stress

Peripartum cardiomyopathy is otherwise-unexplained systolic heart failure arising late in pregnancy or postpartum. Vascular, hormonal and oxidative stresses are thought to injure a susceptible myocardium.

Hypertensive pregnancy disorders

Pre-eclampsia and other hypertensive disorders are associated with greater risk and may add afterload and endothelial stress. They may also independently cause pulmonary oedema, so coexistence needs careful assessment.

Greater pregnancy load

Multiple gestation increases plasma volume, cardiac output and hormonal exposure. This greater haemodynamic burden may reveal limited myocardial reserve during late pregnancy or after delivery.

Underlying susceptibility

Some patients appear to share genetic or familial susceptibility with dilated cardiomyopathy. Pregnancy then acts as a physiological stressor, although no single predisposition establishes the diagnosis.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Proposed stress-susceptibility interaction
PPCM probably reflects several interacting pathways. Late-pregnancy and postpartum haemodynamic, vascular and hormonal changes may trigger or reveal systolic dysfunction in a susceptible myocardium, but no single mechanism explains every case.
2
Left-ventricular contraction falls
Reduced myocardial contractility lowers ejection fraction, with or without ventricular dilatation. Stroke volume falls and compensatory sympathetic and neurohormonal pathways become activated.
3
Filling pressure rises
Reduced forward output and neurohormonal sodium and water retention raise left-sided filling pressure. Pulmonary venous congestion produces orthopnoea and oedema, while annular dilatation can cause functional mitral regurgitation.
4
Severe disease impairs perfusion
Extensive left or biventricular failure reduces maternal cardiac output. Hypotension, organ injury, malignant arrhythmia and cardiogenic shock can develop during an already vulnerable physiological period.
5
Stasis promotes thrombosis
Severe ventricular dysfunction causes intracardiac stasis, compounded by the hypercoagulable peripartum state. Left-ventricular thrombus may embolise systemically, while concurrent venous thrombosis may cause pulmonary embolism.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Heart-failure symptoms

Progressive exertional or resting breathlessness, orthopnoea, PND, cough, oedema, abdominal congestion and profound fatigue.

Objective red flags

Persistent resting tachycardia, low oxygen saturation, raised JVP, gallop, pulmonary crackles, hypotension or new functional MR.

Timing

Typically late pregnancy or early postpartum, but modern definitions recognise presentation across the peripartum months; timing never replaces exclusion of another cause.

Risk context

Multiple gestation, hypertensive disorders, pre-eclampsia and some ancestry/geographical settings increase risk but are not diagnostic.

Acute dangerRed flag

Pulmonary oedema, hypoperfusion, malignant arrhythmia, embolic neurology or severe biventricular failure requires critical care and Pregnancy Heart Team activation.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
12-lead ECG and continuous monitoring when unwellFirst step
Why
Detect arrhythmia, conduction disease and ACS clues.
Interpretation and limitations
ECG is often abnormal but non-specific; a normal tracing does not exclude PPCM.
02
NT-proBNP/BNP and troponin
Why
Support pathological HF and assess myocardial injury.
Interpretation and limitations
Natriuretic peptide is not normally markedly elevated in uncomplicated pregnancy; troponin prompts ACS/SCAD/myocarditis evaluation but may rise in PPCM.
03
Urgent TTE
Why
Measure LVEF, LV/RV size/function, valves, filling, pulmonary pressure and thrombus.
Interpretation and limitations
LVEF below 45% supports PPCM in the correct timing after alternatives are excluded; LV need not be dilated.
04
FBC, U&E/eGFR, LFT, CRP, TSH, glucose and urine protein/BP assessment
Why
Find precipitants, treatment constraints and hypertensive disease.
Interpretation and limitations
Anaemia, infection, renal/hepatic injury or pre-eclampsia may coexist and alter delivery and drug decisions.
05
CXR and lung ultrasound
Why
Confirm pulmonary congestion and identify alternatives.
Interpretation and limitations
Do not withhold clinically necessary ionising imaging in pregnancy; use shielding/protocol optimisation according to modality.
06
CTPA/VQ, coronary imaging or CMR when indicated
Why
Exclude PE, ACS/SCAD or myocarditis/inherited disease.
Interpretation and limitations
Choose by the competing emergency and pregnancy stage; postpartum gadolinium/contrast decisions are individualised.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Normal pregnancy symptoms

Mild exertional breathlessness and dependent oedema are common. Resting hypoxia, persistent orthopnoea, raised JVP, crackles or objective ventricular dysfunction should not be attributed to normal pregnancy without urgent assessment.

Pre-eclampsia-related pulmonary oedema

Hypertension, proteinuria or other maternal-organ dysfunction support pre-eclampsia. Echocardiography may show preserved systolic function, although pre-eclampsia and peripartum cardiomyopathy can coexist.

Pulmonary embolism

Pleuritic pain, haemoptysis, disproportionate hypoxaemia and right-heart strain favour embolism. Pregnancy and the postpartum period increase thromboembolic risk, so assessment remains urgent.

Acute coronary syndrome or dissection

Prominent chest pain, regional ECG change or regional wall-motion abnormality raises concern for coronary ischaemia, including spontaneous coronary artery dissection in the peripartum setting.

Myocarditis or pre-existing cardiomyopathy

Symptoms or documented dysfunction predating late pregnancy, or familial and genetic clues, support pre-existing cardiomyopathy. Cardiac magnetic resonance may identify an alternative injury pattern, but myocarditis can overlap and timing alone cannot establish peripartum cardiomyopathy.

Additional chapter-specific clues

Alternative emergencyRed flag

Pleuritic pain, haemoptysis or disproportionate hypoxia suggests PE; chest pain/ECG change requires ACS and SCAD assessment.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01AcuteSuspected PPCMFirst stepPeripartum HF symptoms with objective abnormality.

1Assess ABCDE, oxygen only for hypoxaemia, ECG/monitoring, IV access, labs including NT-proBNP/troponin and urgent echo; involve obstetrics and cardiology immediately.
2Treat pulmonary congestion with cautious loop diuretic and position/ventilatory support; manage unstable arrhythmia by standard emergency principles with fetal considerations.
3AlternativeActively test for PE, ACS/SCAD, pre-eclampsia, sepsis, valve disease and alternative cardiomyopathy according to findings.
4If shock or severe biventricular failure, transfer early to a centre with Pregnancy Heart Team, mechanical support, advanced HF and neonatal capability.

02PregnancyHF treatment before deliveryPPCM diagnosed while still pregnant.

1Use pregnancy-compatible symptom and afterload treatment selected by the Pregnancy Heart Team, such as beta-1-selective blockade and hydralazine/nitrate when haemodynamics permit.
2Avoid ACE inhibitors, ARBs, ARNIs, MRAs and SGLT2 inhibitors during pregnancy; use diuretic only for congestion to avoid unnecessary placental hypoperfusion.
3Assess anticoagulation when severe LV dysfunction, intracardiac thrombus, AF or another indication is present; choose heparin/VKA timing around gestation and delivery with obstetric specialists.
4Choose delivery timing and mode by maternal haemodynamics and obstetric indication; stable HF does not automatically require caesarean birth.

03PostpartumOptimise after birthHaemodynamically stable postpartum PPCM.

1PreferredTransition to NICE HFrEF disease-modifying therapy, checking lactation compatibility and infant factors medicine-by-medicine; enalapril is the preferred ACE inhibitor during breastfeeding of a full-term healthy infant.
2Continue careful diuresis, rhythm surveillance and anticoagulation when indicated; image for LV thrombus when severe apical dysfunction is present.
3Consider bromocriptine only in a specialist PPCM protocol after explicit MHRA contraindication, BP and thrombosis review—not as routine lactation suppression.
4EscalationArrange early HF follow-up and repeat echo; escalate persistent severe dysfunction for wearable/implantable defibrillator or advanced-HF assessment at the appropriate interval.

04FutureRecovery and another pregnancyPost-discharge or pre-conception review.

1Continue HF therapy and serial imaging until durable recovery; never stop teratogenic medicines simply to conceive without a supervised substitution plan.
2Refer before conception to a Pregnancy Heart Team for current LVEF, contractile reserve where appropriate, recurrence risk and medication review.
3Strongly discourage pregnancy when LV dysfunction persists; if pregnancy proceeds after recovery, provide shared decision-making and close echo/biomarker surveillance through postpartum.

Key medicines and prescribing safety2 treatments · regimens, roles and cautions

Relieves pulmonary/systemic congestion during pregnancy or postpartum.

Furosemide

Common oral starting dose 20-40 mg once daily; in acute pulmonary oedema use an individualised IV dose based on severity and prior exposure.

Use the minimum effective dose: hypovolaemia, electrolyte loss and renal injury can reduce maternal and placental perfusion. Monitor weight, BP, U&E and fetal context.

Postpartum HFrEF disease-modifying treatment; NHS SPS identifies enalapril as the preferred ACE inhibitor during breastfeeding of a full-term healthy infant.

Enalapril (postpartum only)

After delivery, start 2.5 mg orally once daily for symptomatic heart failure under close BP supervision; titrate over 2-4 weeks toward the usual maintenance 20 mg/day in one or two doses, to a maximum 40 mg/day in two divided doses, as tolerated.

Contraindicated during pregnancy. Check BP, renal function and potassium before and after initiation/titration; review hypovolaemia, renal-artery stenosis, hyperkalaemia and previous ACE-inhibitor angioedema. During breastfeeding monitor the infant for poor feeding, lethargy, pallor, inadequate weight gain and, especially when young, reduced wet nappies.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Pulmonary oedema and shock

Rising left-sided filling pressure floods the lungs, while severe pump failure lowers organ perfusion. Critical illness may require ventilatory, circulatory and specialist pregnancy-heart-team support.

Ventricular arrhythmia

A dysfunctional or acutely injured ventricle can become electrically unstable. Sustained ventricular tachycardia or fibrillation can cause syncope, collapse or sudden cardiac death.

Intracardiac thrombus and embolism

Low ventricular flow and peripartum hypercoagulability promote thrombus formation. Left-sided thrombus may cause stroke or limb ischaemia, whereas venous or right-sided thrombus may cause pulmonary embolism.

Persistent chronic heart failure

Incomplete recovery leaves ventricular dilatation and reduced systolic function. Ongoing heart-failure treatment, rhythm surveillance and sometimes device assessment may be required after pregnancy.

Relapse in a later pregnancy

A subsequent pregnancy can provoke recurrent ventricular dysfunction even after apparent recovery. Persistently reduced function carries greater risk and makes pre-conception specialist counselling essential.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

During instability, monitor ECG, oxygenation, BP, urine output, lactate/perfusion and fetal status according to gestation.
Check renal function and electrolytes after diuresis and each postpartum HF-drug initiation or titration.
Repeat TTE before discharge when severe and at early follow-up, then until stable recovery or an advanced-HF decision.
Screen for AF/ventricular arrhythmia and LV thrombus when severe dysfunction or symptoms warrant.
For breastfeeding, review maternal medicine exposure and monitor a full-term healthy infant as specified by SPS; seek specialist advice for premature or unwell infants.
Keep follow-up beyond EF recovery and document contraception and pre-conception counselling.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

Normal pregnancy has limits

Resting hypoxia, orthopnoea, raised JVP or pulmonary oedema must never be normalised as late-pregnancy symptoms.

LV dilatation is optional

PPCM can meet the systolic-dysfunction definition without a dilated ventricle.

Postpartum is still high risk

Haemodynamic shifts and thrombosis risk continue after delivery; birth is not the end of cardiac monitoring.

Bromocriptine has a regulatory tension

Some specialist PPCM strategies consider prolactin blockade, but MHRA advises against routine suppression and lists uncontrolled/hypertensive pregnancy and severe cardiovascular contraindications; specialist risk review is essential.

Recovered EF does not mean zero recurrence

Later pregnancy can relapse even after normalisation, while persistent dysfunction confers substantially greater risk.

11Common pitfallsFrequent interpretation and management errors.

01
Reassuring severe orthopnoea or persistent tachycardia as normal pregnancy.
02
Starting an ACE inhibitor, ARB, ARNI, MRA or SGLT2 inhibitor before delivery.
03
Diagnosing PPCM without excluding PE, pre-eclampsia, ACS/SCAD and pre-existing disease.
04
Using bromocriptine routinely without checking MHRA contraindications, BP and anticoagulation strategy.
05
Discharging after symptomatic improvement without repeat echo and pre-conception follow-up.