Navigating women with congenital heart disease during pregnancy: Management strategies and future directions

Table 2 Common congenital and valvular heart disease encountered in pregnancy

ACHD type	Maternal risk	Fetal risk	Key clinical considerations
ASD	Low risk (< 5% arrhythmia, endocarditis, TE)	Low fetal mortality	DVT prophylaxis; consider anticoagulation if high-risk; aspirin in select cases
VSD	Similar to ASD	Low fetal mortality, CHD recurrence 27%	Standard management; low risk overall
Tetralogy of Fallot (repaired)	Low cardiac event rate, arrhythmia (2%-6%)	Low fetal risk	Elective PVR if RV dysfunction or dilation
CoA	HTN (5%-30%), rare dissection	Low fetal mortality, CHD recurrence 4%	Avoid pregnancy in severe CoA (mWHO IV); control BP carefully
Ebstein anomaly	HF 3%, arrhythmia 4%	Preterm 22%	Assess cyanosis, degree of TR, and RV function
d-transposition of great arteries s/p atrial switch	HF 10%, arrhythmia 15%	Preterm 34%-38%, low CHD recurrence	Assess systemic ventricular function and TR
ccTGA/l-TGA	HF 10%, cardiac event 2%	Preterm 9%, CHD recurrence 36%	Assess systemic RV, TR, and heart block risk
Cyanotic CHD (unrepaired)	High maternal risk (HF 19%, TE 3.6%)	Fetal mortality 12%, preterm 45%	Contraindicated for pregnancy; require thromboembolism prophylaxis, iron support
Eisenmenger syndrome	Very high maternal mortality (33%), TE 18%	Fetal mortality up to 30%, preterm 65%	Pregnancy is contraindicated; PDE-5i/prostanoids may be used, endothelin antagonists contraindicated
Fontan circulation	HF 3%-11%, arrhythmia up to 37%	Preterm 28%-59%, live birth only 45% of evidence of Fontan failure, postpartum hemorrhage 14%	Avoid pregnancy in complicated Fontan; anticoagulation recommended
Severe mitral stenosis	Mortality 3%, HF 37%, arrhythmia 16%	Fetal mortality 6%, preterm 18%	Severe MS = mWHO IV (contraindicated); moderate = mWHO III
Severe aortic stenosis	Mortality 2%, HF 9%, arrhythmia 4%	Fetal mortality 5%, preterm 4%	Severe symptomatic AS = mWHO IV; assisted delivery may be considered
Severe pulmonary stenosis	Generally well tolerated; worsening function possible	No significant fetal effects observed	Monitor for worsening symptoms; limited data
Moderate/severe AV valve regurgitation	Mortality < 1%, HF 8%-11%, arrhythmia 6%-8%	Fetal mortality 0%-1%, preterm 12%-15%	Worse prognosis with pulmonary hypertension or LV dysfunction
Moderate/severe semilunar valve regurgitation	Mortality < 1%, HF 1%-3%, arrhythmia 0%-3%	Fetal mortality 1%-8%, preterm 5%-10%	Same considerations as AV regurgitation

TE: Thromboembolism; DVT: Deep vein thrombosis; PVR: Pulmonary valve replacement, RV: Right ventricle; CoA: Coarctation of the aorta; mWHO: Modified world health organization classification; HTN: Hypertension; HF: Heart failure; TR: Tricuspid regurgitation; PDE-5i: Phosphodiesterase 5 inhibitor; AV: Atrioventricular valve regurgitation; s/p: Status post; CHD: Congenital heart disease; BP: Blood pressure; ACHD: Adult congenital heart disease; ASD: Atrial septal defect; ccTGA: Congenitally corrected transposition of the great arteries; l-TGA: L-transposition of great arteries; VSD: ventricular septal defect; MS: Mitral stenosis; AS: Aortic stenosis; LV: Left ventricle.