CARPREG and ZAHARA maternal cardiovascular and offspring risk scores, and modified WHO classification of maternal risk
| Predictor | Risk points (maternal risk) | Risk points (offspring risk) |
|---|---|---|
| CARPREG | ||
| Prior cardiac event (heart failure, transient ischaemic attack, stroke, arrhythmia) | 1 | – |
| NYHA functional class III/IV or cyanosis (SpO2<90%) | 1 | 1 |
| Left heart obstruction (mitral valve area <2 cm2 or aortic valve area <1.5 cm2 or peak LVOT gradient >30 mm Hg (echocardiography) | 1 | 0.75 |
| Reduced systemic ventricular systolic function (EF <40%) | 1 | – |
| Multiple gestation | – | 3 |
| Smoking | – | 1 |
| Heparin/warfarin during pregnancy | – | 1 |
| ZAHARA | ||
| Prior arrhythmia | 1.50 | – |
| NYHA functional class III/IV | 0.75 | – |
| Left heart obstruction (peak LVOT gradient >50 mm Hg or aortic valve area <1.0 cm2 | 2.50 | – |
| Mechanical valve prosthesis | 4.25 | 2.50 |
| Systemic atrioventricular valve regurgitation (moderate/severe) | 0.75 | – |
| Pulmonary atrioventricular valve regurgitation (moderate/severe) | 0.75 | – |
| Cardiac medication before pregnancy | 1.50 | 0.75 |
| Cyanotic heart disease (corrected and uncorrected) | 1.00 | 0.75 |
| Twin or multiple gestation | – | 1.75 |
| Smoking during pregnancy | – | 0.50 |
| Modified WHO Classification | ||
| Conditions in which maternal risk is WHO class I | ||
| Uncomplicated, small or mild pulmonary stenosis | ||
| Successfully repaired simple lesions (atrial or ventricular septal defect, patent ductus arteriosus, anomalous pulmonary venous drainage) | ||
| Conditions in which maternal risk is WHO class II or III | ||
| WHO class II (if otherwise well and uncomplicated) | ||
| Unoperated atrial or ventricular septal defect, repaired tetralogy of Fallot | ||
| WHO class II–III (depending on individual) | ||
| Native or tissue valvular heart disease not considered WHO I or IV; repaired coarctation; Marfan syndrome without aortic dilatation, bicuspid valve with aorta <45 mm; mild ventricular impairment | ||
| WHO class III | ||
| Mechanical valve; systemic RV; Fontan circulation; unrepaired cyanotic heart disease; other complex congenital heart disease; Marfan syndrome with aorta 40–45 mm; bicuspid aortic valve with aorta 45–50 mm | ||
| Conditions in which pregnancy risk is WHO class IV (contra-indicated) | ||
| Pulmonary hypertension/Eisenmenger syndrome; systemic ventricular EF <30% or systemic ventricular dysfunction with NYHA class III–IV; severe mitral stenosis, severe symptomatic aortic stenosis, Marfan syndrome with aorta >45 mm; bicuspid aortic valve with aorta >50 mm; native severe coarctation | ||
| CARPREG Risk Score: For each CARPREG predictor that is present, a predictor-specific number of points is assigned for maternal cardiovascular risk or offspring risk, according to the table. The risk score (either maternal or offspring) is the total number of points. The risk of maternal cardiovascular complication is 5% with 0 points, 27% with 1 point and 75% with ≥1 point. The risk of offspring complications is higher with a higher risk score; no percentages are assigned to the score. ZAHARA risk score: for each ZAHARA predictor that is present, a predictor-specific number of points is assigned to the pregnancy, according to the table. The risk of maternal cardiovascular complications is 2.9% with <0.50 points, 7.5% with 0.51–1.50 points, 17.5% with 1.51–2.50 points, 43.1% with 2.51–3.50 points and 70% with >3.51 points. The risk of offspring complications is 19.9% with <0.50 risk points, 33.3% with 0.50–0.99 risk points, 46.7% with 1.0–1.49 risk points, and 59.6% with ≥1.50 risk points. Modified WHO classification: Class I: no detectable increased risk of maternal mortality and no/mild increase in morbidity; Class II: small increased risk of maternal mortality or moderate increase in morbidity; Class III: significantly increased risk of maternal mortality or severe morbidity; Class IV: extremely high risk of maternal mortality or severe morbidity, pregnancy is contra-indicated. | ||
CARPREG, CARdiac disease in PREGnancy; LVOT, LV outflow tract; NYHA, New York Heart Association; SpO2, oxygen saturation as measured by pulse oximetry; ZAHARA, Zwangerschap bij Aangeboren HARtAfwijkingen (pregnancy in congenital heart disease).