| Diagnosis | Ventricular (dys)function + pulmonary hypertension |
| | Mild left ventricular impairment: EF >45%. Significantly impaired RV (subpulmonary) function. | Moderate left ventricular impairment: EF 30%–45%. Previous PPCM with not more than mild residual left ventricular impairment. | Severe left ventricular impairment: EF <30% or NYHA class III/IV. Previous PPCM with more than mild left ventricular impairment.
PAH. |
| Arrhythmias |
Atrial or ventricular
ectopic beats, isolated. | Most supraventricular arrhythmias. Bradycardia requiring pacemaker. | Low-risk LQTS: no previous events + on full dose beta-blocker therapy. Low-risk CPVT: well controlled by medical therapy. BrS with no previous events. | Sustained ventricular tachycardia from any aetiology. LQT2 (post-partum). Symptomatic CPVT and LQTS not adequately controlled by therapy. BrS with previous events. | |
| Cardiomyopathy |
| HCM: genotype-positive + phenotype-negative. | | Low-risk ARVC: genotype-positive + no or mild phenotype. HCM without complications. DCM/NDLVC with normal or mild left ventricular impairment: EF >45%. | ARVC with moderate/severe disease. HCM with arrhythmic and/or moderate haemodynamic complications. DCM/NDLVC with moderate left ventricular impairment: EF 30%–45%. | DCM/NDLVC with severe left ventricular impairment: EF <30% or NYHA class III/IV. HCM with symptomatic severe outflow tract obstruction: ≥50 mmHg. HCM with severely symptomatic LV dysfunction (EF <50%). |
| Congenital heart disease |
| Successfully repaired simple lesions without significant residual (haemodynamic) complications (atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous drainage). | Unoperated uncomplicated atrial or ventricular septal defect. Repaired tetralogy of Fallot without significant residual haemodynamic/arrhythmic lesions. Transposition of the great arteries with arterial switch without significant residual lesions. | Repaired atrioventricular septal defect without significant residual lesions. Uncomplicated Ebstein anomaly: mild to moderate TR, no tricuspid stenosis, no accessory pathway. | Unrepaired cyanotic heart disease (not Eisenmenger). Systemic RV with good or mildly decreased ventricular function. Uncomplicated Fontan circulation: good ventricular function, no significant valve disease or arrhythmias, good exercise tolerance, and normal arterial saturations. Ebstein anomaly with any complication. | Systemic RV with moderate or severely decreased ventricular function. Fontan with any complication. Eisenmenger syndrome. |
| Valvular heart disease |
Small or mild
• pulmonary stenosis
• mitral valve prolapse without significant regurgitation. | | Native, homograft or tissue valve disease not considered mWHO 2.0 I or IV: mild mitral stenosis, moderate aortic stenosis. Moderate valvular regurgitation. | Uncomplicated mechanical valve with stable well controlled INRs.
Moderate mitral stenosis. Severe asymptomatic aortic stenosis. Severe left-sided valvular regurgitation. | Severe mitral stenosis.
Severe symptomatic aortic stenosis. |
| Aortopathy |
| Non-HTAD mild aortic dilatation (<40 mm). | Turner syndrome without cardiovascular features (BAV, coarctation, AHT, aortic dilatation). | Marfan or other HTAD syndrome without aortic dilatation. Aorta <45 mm in BAV pathology. Repaired coarctation. | Moderate aortic dilatation: 40–45 mm in Marfan syndrome or other HTAD; 45–50 mm in BAV, Turner syndrome ASI 20–25 mm/m2, other aortic dilatation <50 mm. Marfan with previous aortic root replacement. Previous aortic dissection with stable diameter. | Severe aortic dilatation: >45 mm in Marfan syndrome or other HTAD, >50 mm in BAV, ASI >25 mm/m2 in Turner syndrome, other aortic dilatation >50 mm. Vascular Ehlers–Danlos syndrome. Severe (re)coarctation. Previous aortic dissection with increasing diameter. |
| Acquired + coronary heart disease + other |
| | | Prior SCAD.
Prior ischaemic cardiac
event (STEMI/NSTE
ACS).
Prior adverse pregnancy
outcome requiring
hospitalization.
Prior adverse
cardiovascular effects of cancer treatment. | |
| 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. |
| Average maternal cardiac event rates | Van Hagen et al. (2016) | 9.9% | 7.7% | 17.7% | 28.9% | 50.3% |
| Silversides et al. (2018) | 3.1% | 21.7% | 12.8% | 21.1% | 35.6% |
| Individualize each maternal risk with the modifiers below (derived from CARPREG II) |
CARPREG II score: 1 point
• No prior cardiac intervention indicated
• Late pregnancy assessment | CARPREG II score: 2 points
• Ventricular dysfunction
• High-risk left-sided valve disease or outflow
tract obstruction
• Pulmonary hypertension
• Coronary artery disease
• High-risk aortopathy | CARPREG II score: 3 points
• Prior cardiac event or arrhythmias
• Baseline NYHA III/IV or cyanosis
• Mechanical valve |
