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. 2024 Mar 14;20(2):24–35. doi: 10.14797/mdcvj.1306

Table 2.

High-risk congenital heart disease in pregnancy. CHD: congenital heart disease; LV: left ventricle; mWHO: modified World Health Organization; NYHA: New York Heart Association; CARPREG: Cardiac Risk in Pregnancy Study; BAV: bicuspid aortic valve; HCTD: high risk connective tissue disease


CONGENITAL HEART DISEASE PHYSIOLOGICAL VARIABLE mWHO RISK (III vs IV)

Congenital severe aortic stenosis Asymptomatic III

Symptomatic IV

Unrepaired CHD Hypoxemia (O2 < 94%) III (degree of hypoxemia not addressed in mWHO or CARPREG 2)

Severe hypoxemia

Systemic right ventricle Good or mildly reduced RV function III

Moderate to severely reduced RV function IV

Fontan circulation Well and uncomplicated III

Arrhythmias IV

Degree of hypoxemia IV

Moderate to severe systemic ventricular dysfunction IV

NYHA functional class IV

Re-coarctation Not severe II-III

Severe IV

Any anatomically simple, moderate, or complex congenital heart disease Pulmonary hypertension (including Eisenmenger syndrome) IV

Moderate LV dysfunction (EF 30-45%) III (Unless classified as IV due to NYHA FC III-IV)

Severe LV dysfunction (EF < 30%) IV

NYHA Functional Class II-IV IV

Arrhythmia Not addressed in mWHO, use CARPREG for risk assessment

Mechanical valve III

Aortopathy Moderate aortic dilatation (40-45 mm in Marfan syndrome or other HCTD; 45–50 mm in BAV, 20–25 mm/m2 in Turner syndrome) III

Severe aortic dilatation (> 45 mm in Marfan syndrome or other HCTD, > 50 mm in BAV, > 25 mm/m2 in Turner syndrome) IV