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Table 3 Modified World Health Organization classification of maternal cardiovascular risk mWHO I mWHO II mWHO II–III mWHO III mWHO IV Diagnosis (if otherwise well and uncomplicated) Small or mild – pulmonary stenosis – patent ductus arteriosus – mitral valve prolapse Successfully repaired simple lesions (atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous drainage) Atrial or ventricular ectopic beats, isolated Unoperated atrial or ventricular septal defect Repaired tetralogy of Fallot Most arrhythmias (supraventricular arrhythmias) Turner syndrome without aortic dilatation Mild left ventricular impair- ment (EF >45%) Hypertrophic cardiomyopathy Native or tissue valve disease not considered WHO I or IV (mild mitral stenosis, moderate aortic stenosis) Marfan or other HTAD syndrome without aortic dilatation Aorta <45 mm in bicuspid aortic valve pathology Repaired coarctation Atrioventricular septal defect Moderate left ventricular impairment (EF 30–45%) Previous peripartum cardiomyopathy without any residual left ventricular impairment Mechanical valve Systemic right ventricle with good or mildly decreased ventricular function Fontan circulation. If otherwise the patient is well and the cardiac condition uncomplicated Unrepaired cyanotic heart disease Other complex heart disease Moderate mitral stenosis Severe asymptomatic aortic stenosis Moderate aortic dilatation (40–45 mm in Marfan syndrome or other HTAD; 45–50 mm in bicuspid aortic valve, Turner syndrome ASI 20–25 mm/m2, tetralogy of Fallot <50 mm) Ventricular tachycardia Pulmonary arterial hypertension Severe systemic ventricular dysfunction (EF <30% or NYHA class III–IV) Previous peripartum cardiomyopathy with any residual left ventricular impairment Severe mitral stenosis Severe symptomatic aortic stenosis Systemic right ventricle with moderate or severely decreased ventricular function Severe aortic dilatation (>45 mm in Marfan syndrome or other HTAD, >50 mm in bicuspid aortic valve, Turner syndrome ASI >25 mm/m2, tetralogy of Fallot >50 mm) Vascular Ehlers–Danlos Severe (re)coarctation Fontan with any complication Risk No detectable increased risk of maternal mortality and no/mild increased risk in morbidity Small increased risk of maternal mortality or moderate increase in morbidity Intermediate increased risk of maternal mortality or moderate to severe increase in morbidity Significantly increased risk of maternal mortality or severe morbidity Extremely high risk of maternal mortality or severe morbidity Maternal cardiac event rate 2.5–5% 5.7–10.5% 10–19% 19–27% 40–100% Counselling Yes Yes Yes Yes: expert counselling required Yes: pregnancy contrain- dicated: if pregnancy occurs, termination should be discussed Care during pregnancy Local hospital Local hospital Referral hospital Expert centre for pregnancy and cardiac disease Expert centre for preg- nancy and cardiac disease Minimal follow-up visits during pregnancy Once or twice Once per trimester Bimonthly Monthly or bimonthly Monthly Location of delivery Local hospital Local hospital Referral hospital Expert centre for pregnancy and cardiac disease Expert centre for preg- nancy and cardiac disease ASI = aortic size index; EF = ejection fraction; HTAD = heritable thoracic aortic disease; mWHO = modified World Health Organization classification; NYHA = New York Heart Association; WHO = World Health Organization. Downloaded from academic.oup.com/eurheartj/article/39/34/3165/5078465 by guest on 07 October 2026