2018 ESC Guidelines for the Management of Cardiovascular Diseases during Pregnancy

Authors, Journal, Affiliations, Type, DOI

• Vera Regitz-Zagrosek, Jolien W. Roos-Hesselink, Johann Bauersachs et al. (ESC Task Force for Management of Cardiovascular Diseases during Pregnancy)
• European Heart Journal, 2018; 39(34): 3165–3241
• Affiliations: Multi-institutional European authorship; endorsed by International Society of Gender Medicine
• Type: Clinical Practice Guideline (ESC)
• DOI: https://doi.org/10.1093/eurheartj/ehy340

Overview

The 2018 ESC Guidelines are the primary authoritative reference for managing cardiovascular disease across the reproductive cycle. The central risk stratification tool is the modified WHO (mWHO) classification (classes I–IV), with class IV carrying 40–100% cardiac event rates and constituting a contraindication to pregnancy. Key 2018 changes vs. 2011: sotalol removed from arrhythmia management; LMWH upgraded to drug of choice for VTE prevention (IB); non-selective beta-blockers mandated 40 weeks postpartum in LQTS/CPVT; bromocriptine and the BOARD framework formalised for PPCM; catheter ablation upgraded to IIaC using electroanatomical mapping; FDA letter-category drug classification system abandoned (IIIC recommendation).

Keywords

Pregnancy, cardiovascular disease, modified WHO classification, congenital heart disease, pulmonary hypertension, aortic disease, valvular heart disease, coronary artery disease, cardiomyopathy, peripartum cardiomyopathy, arrhythmia, hypertensive disorders of pregnancy, venous thrombo-embolism, anticoagulation, drugs in pregnancy

Key Takeaways

§2–3 General Principles: Physiology, Risk Stratification, Delivery

• Physiological adaptations: CO increases 40–50% by week 32 (↑HR 10–20 bpm + ↑SV); plasma volume increases 40–50%; hypercoagulable state; all changes reverse within 6 months postpartum
• mWHO Classification is the primary risk stratification tool; validated prospectively in ROPAC and ZAHARA studies; event rates:
  • Class I (2.5–5%): repaired simple lesions, isolated ectopics, mild PS
  • Class II (5.7–10.5%): unoperated ASD/VSD, repaired tetralogy of Fallot, mild LVOTO, Marfan without aortic dilatation
  • Class II–III (10–19%): mild LV impairment, HCM, native or bioprosthetic valve disease not Class I or IV
  • Class III (19–27%): mechanical valve, systemic RV, Fontan without complications, unrepaired cyanotic CHD, moderate LVOTO, moderate systemic ventricular dysfunction
  • Class IV (40–100% — pregnancy not recommended): PAH, severe systemic ventricular dysfunction (EF <30% or NYHA III–IV), prior PPCM with any residual LV impairment, severe MS or severe symptomatic AS, systemic RV with moderate/severe dysfunction, severe aortic dilatation (>45 mm Marfan or Loeys-Dietz, >50 mm BAV), vascular EDS, severe re-coarctation, Fontan with any complication
• NT-proBNP >128 pg/mL at 20 weeks is an independent predictor of adverse cardiac events later in pregnancy
• Pregnancy heart team (multidisciplinary: cardiologist, obstetrician, anesthesiologist, MFM, neonatologist) mandatory for moderate- and high-risk patients
• Delivery: vaginal delivery recommended as first choice for most; caesarean section considered for: oral anticoagulants at labour onset, aggressive aortic pathology, acute intractable HF, Eisenmenger's/severe PH, severe symptomatic AS, aortic dilatation >45 mm
• Induction at 40 weeks considered in all women with cardiac disease (IIaC); reduces emergency CS by 12% and stillbirth by 50%
• Antibiotic prophylaxis for delivery not recommended (IIIC)

§4 Congenital Heart Disease and Pulmonary Hypertension

• CHD present in 2/3 of cardiac pregnancies; most CHD well tolerated; 10% maternal cardiac complications
• PAH: maternal mortality 16–30% despite targeted therapies; pregnancy remains contraindicated (IIIB); if pregnancy occurs, termination discussed; greatest risk in puerperium; bosentan contraindicated (embryopathy) unless withdrawal greatly increases maternal risk; sildenafil often first-line
• Eisenmenger's syndrome: maternal mortality 20–50%; termination discussed; sildenafil/prostanoids used; caesarean section in severe forms
• Cyanosis without PH: if SaO₂ >90% foetal outcome better (10% foetal loss); if SaO₂ <85% high rates of foetal loss (live birth rate only 12%)
• Fontan: mWHO III–IV; high miscarriage rate (30%); therapeutic anticoagulation considered; atrial arrhythmias require prompt cardioversion

§5–6 Aortic and Valvular Disease

• Aortic dissection most common in last trimester (50%) or early postpartum (33%); all patients with known aortopathy should have complete aortic imaging pre-pregnancy
• Marfan: caesarean if aorta >45 mm; beta-blockers throughout; surgery indicated if aorta >45 mm
• Mitral stenosis (MS): restrict activity + beta-1 selective blockers if symptomatic/PH (IB); diuretics if congestion persists (IB); intervention recommended if MVA <1.0 cm² before pregnancy (IC); PMC considered during pregnancy if sPAP >50 mmHg or severe symptoms despite therapy (IIaC)
• Aortic stenosis (AS): surgery recommended pre-pregnancy if symptomatic or LVEF <50% or exercise test positive (IB); balloon valvuloplasty considered during pregnancy in severe symptomatic cases (IIaC)
• Mechanical prostheses: VKAs throughout pregnancy provide best valve thrombosis protection but carry embryopathy risk (0.45–0.9% with low-dose warfarin ≤5 mg/day), foetopathy risk; VKAs recommended in second/third trimester in women needing low dose (IC); LMWH with anti-Xa monitoring (peak 0.8–1.2 IU/mL aortic; 1.0–1.2 IU/mL mitral) when switching; switch to IV UFH ≥36 h before planned delivery (IC)
• Atrial fibrillation in valve disease: immediate anticoagulation with heparins or VKA; DOACs contraindicated throughout pregnancy

§7 Coronary Artery Disease

• AMI complicates 1.7–6.2/100,000 deliveries; accounts for >20% of maternal cardiac deaths; 3–4× higher risk vs. non-pregnant age-matched women
• Dominant aetiology differs from non-pregnant: P-SCAD 43% (most common; late pregnancy/early postpartum; multivessel LAD involvement), normal coronaries 18%, coronary thrombosis 17%
• STEMI management: primary PCI recommended (IC); radial approach preferred; radiation minimised; thrombolysis as bailout only
• Previous CAD: pregnancy considered if no residual ischaemia; delay 12 months post-AMI; SCAD recurrence risk not defined but further pregnancy discouraged

§8 Cardiomyopathies and Heart Failure

• PPCM definition: New-onset HF with LVEF <45% in last weeks of pregnancy or postpartum without prior heart disease or identifiable cause; majority diagnosed post-partum
• PPCM risk factors: multiparity, African ethnicity, smoking, diabetes, pre-eclampsia, advanced age, teenage pregnancy
• PPCM prognosis: EF recovery predominantly within 6 months; mortality 2% (Germany) to 12.6% (South Africa) to 24% (Turkey 24 months); EF <30% or LVEDD ≥6.0 cm or RV involvement → adverse outcome; subsequent pregnancy discouraged if EF <50–55%
• BOARD framework for acute PPCM management:
  • Bromocriptine — stops prolactin pathway; 2.5 mg once daily ≥1 week (uncomplicated); 2.5 mg BID ×2 weeks then once daily ×6 weeks (EF <25% or cardiogenic shock); always with anticoagulation (IIbB)
  • Oral HF therapies (ACEi/ARBs contraindicated in pregnancy, avoid during breastfeeding; hydralazine + nitrates as substitute)
  • Anticoagulants — heparin during bromocriptine; standard anticoagulation indications apply
  • Relaxing agents — vasodilators (hydralazine/nitrates); amlodipine considered
  • Diuretics — for congestion; avoid without pulmonary congestion
• WCD (Wearable Cardioverter-Defibrillator): consider for first 3–6 months in PPCM with EF <35% (limited data); avoids premature ICD implantation given high recovery rate
• ICD: early implantation in newly diagnosed PPCM NOT appropriate given high recovery rate; after 6–12 months if LVEF still reduced per standard guidelines
• Breastfeeding in HFrEF: discouraged in NYHA III/IV; stopping lactation reduces metabolic demand and enables optimal HF therapy (IIbB)
• DCM in pregnancy: NYHA III/IV and EF <40% predict maternal mortality; highly adverse risk: EF <20%, MR, RV failure, AF, hypotension; ACEi/ARBs/ARNIs/MRAs/ivabradine must be stopped pre-conception; beta-blockers continued (switch to beta-1 selective)
• HCM in pregnancy: usually well tolerated (maternal mortality 0.5% in meta-analysis); 29% worsening symptoms; beta-blockers continued (IB); start for symptoms/arrhythmia (IIaC); cardioversion for persistent AF (IIaC); therapeutic anticoagulation for paroxysmal/persistent AF

§9 Arrhythmias

• Most common arrhythmias: AF (27/100,000) and PSVT (22–24/100,000) apart from premature beats; life-threatening VT rare
• AF mortality risk: OR 13.13 (CI 7.77–22.21) for maternal death
• New-onset VT: must exclude structural heart disease; OR 40.89 (CI 26.08–64.1) for SCD; PPCM must be excluded
• LQTS: Women with congenital LQTS at substantial risk of cardiac events in postpartum period; cascade screening for channelopathies important in SCD context
• PSVT management:
  • Acute termination: vagal manoeuvres → adenosine IV (IC); cardioversion for haemodynamic instability (IC)
  • Prevention: beta-1-selective blockers or verapamil first-line if no pre-excitation (IC); flecainide/propafenone for WPW (IC); sotalol/flecainide/propafenone if nodal blockers fail (IIaC)
• AF management:
  • Rhythm control preferred during pregnancy; beta-blocker first-line (IIaC)
  • Rate control: oral beta-blocker (IC); digoxin/verapamil if beta-blockers fail (IIaC)
  • Cardioversion for haemodynamic instability or foetal risk (IC)
  • DOACs prohibited throughout pregnancy
• VT management:
  • Immediate cardioversion for unstable/stable sustained VT (IC)
  • Idiopathic RVOT VT: beta-blocker or verapamil as prophylaxis; catheter ablation if drug-refractory
  • Non-selective beta-blockers (propranolol/nadolol) throughout pregnancy and postpartum (at least 40 weeks after delivery) in LQTS and CPVT (IC); exceptions for asymptomatic LQTS patients without prior TdP who may use selective beta-blocker
  • ICD implantation recommended if indication emerges during pregnancy (IC); preferably one chamber
• Catheter ablation (IIaC): second trimester preferred; electroanatomical mapping systems mandatory to reduce radiation; appropriate for drug-refractory AVNRT/AVRT/focal AT/CTI flutter/benign right-sided VT
• Delivery surveillance tiers (ESC 2018):
  • Level 1 (low risk): PSVT/AF/idiopathic VT/low-risk LQTS/WPW — cardiology consult
  • Level 2 (medium risk): unstable SVT/VT/ICD/VT + structural disease/Brugada/moderate-risk LQTS/CPVT — tertiary centre; IV line; adenosine + beta-blocker ready; external defibrillator
  • Level 3 (high risk): unstable VT in structural disease, TdP in high-risk LQTS, SQTS, high-risk CPVT — caesarean in cardiac OR; arterial line; IV antiarrhythmics; cardiac ICU post-delivery

§10 Hypertensive Disorders

• Affect 5–10% of pregnancies worldwide; #1 medical complication; leading cause maternal/foetal morbidity
• Classifications: pre-existing HTN; gestational HTN; pre-eclampsia; superimposed gestational HTN; antenatally unclassifiable
• BP thresholds: SBP ≥170 or DBP ≥110 mmHg = emergency → hospitalise (IC); treat mild-moderate if SBP ≥150/DBP ≥95 mmHg (IC); also treat at ≥140/90 mmHg in gestational HTN/superimposed HTN/organ damage (IC)
• First-line drugs: methyldopa (IB), labetalol (IC), calcium antagonists/nifedipine (IC)
• Strict contraindications: ACE inhibitors, ARBs, direct renin inhibitors (IIIC)
• Pre-eclampsia prevention: aspirin 100–150 mg/day from week 12 to week 36–37 in high/moderate-risk women (IA)
• Eclampsia prevention/seizure treatment: IV magnesium sulfate (do not combine with CCBs — hypotension risk)
• Post-partum: methyldopa avoided postpartum (risk of depression); breastfeeding does not increase BP; all antihypertensive agents enter breast milk
• Long-term: gestational HTN and pre-eclampsia → increased risk of hypertension, stroke, and ischaemic heart disease in later life

§11 Venous Thrombo-Embolism

• VTE 0.05–0.20% of pregnancies; PE case fatality 3.5%; risk highest immediately postpartum; returns to non-pregnant level at 6 weeks
• LMWH is drug of choice for prevention and treatment (IB); lower osteoporosis, less HIT, less thrombocytopenia vs UFH
• DOACs strictly contraindicated during pregnancy (IIIC)
• Prophylaxis: weight-based LMWH; enoxaparin 0.5 IU/kg once daily for high-risk women
• Treatment dose: enoxaparin 1 mg/kg BID, aiming peak anti-Xa 0.6–1.2 IU/mL
• D-dimer: physiologically elevated throughout pregnancy (mean rise 39% per trimester); negative D-dimer may not exclude VTE in pregnancy — imaging remains gold standard
• DVT: left-sided in >85% (iliac vein compression); compression ultrasound first-line; MR venography for pelvic DVT
• Peripartum anticoagulation management: therapeutic LMWH → omit for planned delivery (low-risk: 24 h; high-risk: convert to UFH 36 h before); UFH stopped 4–6 h before delivery; restart 6 h after vaginal delivery / 12 h after CS (if no bleeding)

§12 Drugs during Pregnancy and Breastfeeding

• FDA letter categories no longer recommended (IIIC); replaced by PLLR narrative labelling; consultation of electronic databases (www.safefetus.com) recommended
• ACE inhibitors/ARBs/ARNIs: teratogenic, strictly contraindicated; up to 48–87% foetal complications in exposure data
• RAAS drugs: spironolactone not advised; eplerenone only if clearly needed
• Beta-blockers: generally safe; beta-1-selective (metoprolol/bisoprolol) preferred for most indications; non-selective agents (propranolol/nadolol) preferred for LQTS/CPVT (superior QTc shortening); atenolol associated with higher foetal growth retardation — best avoided; growth restriction/hypoglycaemia/bradycardia possible
• CCBs: nifedipine safe (some neonatal seizure risk if used in third trimester); verapamil safe in pregnancy — second-line for AF rate control and idiopathic sustained VTs; diltiazem teratogenic in animals
• Amiodarone: foetal hypothyroidism, growth retardation, premature labour — avoid in pregnancy
• Sotalol: removed from 2018 ESC arrhythmia recommendations; potential TdP risk; may be considered only if other agents fail
• Flecainide/propafenone: safe in structurally normal heart; first-line for WPW SVT prevention
• Digoxin: can be considered for rate control if beta-blockers fail; verapamil alternative
• VKAs: embryopathy in first trimester (0.45–0.9% low-dose warfarin ≤5 mg); foetopathy 0.7–2% second/third trimester; vaginal delivery while on VKA contraindicated (foetal intracranial haemorrhage risk)
• Statins: should not be prescribed in pregnancy or breastfeeding for hyperlipidaemia
• Bromocriptine: included as adjunct PPCM treatment; always with prophylactic/therapeutic anticoagulation

Limitations of the Document

• The majority of recommendations are Class C (expert consensus/retrospective data) given the ethical impossibility of RCTs in pregnancy; very few IB or IA recommendations
• mWHO classification event rate data primarily from ROPAC registry (observational, selection bias possible)
• Limited data on novel therapies (e.g., NOAC safety post-delivery in breastfeeding; gene therapy in inherited arrhythmias)
• 2018 vintage: does not include newer data on ERA safety in PAH pregnancy, updated VTE risk stratification models, or newer arrhythmia ablation data

Key Concepts Mentioned

• concepts/mWHO-Classification — central risk stratification framework
• concepts/LQTS-Pregnancy-Management — arrhythmia section §9 expands LQTS/CPVT management
• concepts/Cardio-Obstetrics — pregnancy heart team model
• entities/Peripartum-Cardiomyopathy — BOARD framework, bromocriptine dosing
• concepts/Hypertensive-Disorders-of-Pregnancy — §10 classification and management
• concepts/Preeclampsia — prevention, management, delivery
• concepts/Adverse-Pregnancy-Outcomes — mWHO IV conditions

Key Entities Mentioned

• entities/CPVT — non-selective beta-blockers Class I throughout pregnancy + 40 weeks postpartum
• entities/Long-QT-Syndrome — postpartum high-risk period; non-selective beta-blockers; delivery risk tiers
• entities/Atrial-Fibrillation — most common arrhythmia in pregnancy; management algorithm
• entities/Pulmonary-Hypertension — mWHO IV; termination discussed; PAH therapies
• entities/Heart-Failure — acute HF management; contraindicated drugs

Wiki Pages Updated

• wiki/sources/cv-pregnancy-esc-2018.md — created
• wiki/concepts/mWHO-Classification.md — created
• wiki/entities/Peripartum-Cardiomyopathy.md — updated
• wiki/entities/CPVT.md — updated (pregnancy section added)
• wiki/concepts/LQTS-Pregnancy-Management.md — updated (source added)
• wiki/concepts/Cardio-Obstetrics.md — updated (source added, mWHO specifics)
• wiki/sourceindex.md — updated
• wiki/wikiindex.md — updated
• log.md — updated