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

Table 1 Physiological changes during pregnancy and delivery

Changes	Variables	First trimester	Second trimester	Third trimester	Delivery
Hemodynamic	CO	Small increase	Moderate increase	Moderate increase	Significant increase
	SVR	Mild decrease	Moderate decrease	Moderate decrease	-
	PVR	Mild decrease	Mild decrease	Mild decrease	Mild increase
	HR	Small increase	Moderate increase	Significant increase	Very significant increase
	BP	Mild decrease	Mild decrease	No change	Mild increase
WBC	WBC count	-	-	-	High
RBC	RBC mass	Small increase	Moderate increase	Moderate increase	-
Blood volume	Plasma volume	Moderate increase	Moderate increase	Very significant increase	Extremely significant increase
Remodeling in heart	LV mass	Small increase	Small increase	Small increase	-
	Chamber sizes	-	-	4-chamber enlargement	-
Aorta	Distensibility	Increase	-	-	-
Respiratory minute ventilation	O2 saturation1	Small increase	Moderate increase	Moderate increase	-
Cardiac biomarkers	BNP	No change	-	-	Mild increase
	cTn	No change	-	-	-
	CK-MB	Increase	-	-	-
	D-dimer	Small increase	Moderate increase	Significant increase	-
ECG changes	P wave	Small increase	Plateau	-	-
	Q wave	-	-	Prominent Q wave in inferior and anterolateral leads	-
	QTc	-	-	Mild increase	-
	ST changes	-	-	-	ST depression after cesarean section delivery
Arrhythmia	APC	Common	-	-	-
	PVC	Common	-	-	-

¹O₂ saturation < 95% is abnormal during pregnancy and needs investigation.

CO: Cardiac output; SVR: Systemic vascular resistance; PVR: Pulmonary vascular resistance; HR: Heart rate; BP: Blood pressure; WBC: White blood cells; RBC: Red blood cells; LV: Left ventricle; BNP: Brain natriuretic peptide; CK-MB: Creatine kinase-isoenzyme MB; ECG: Electrocardiography; cTn: Cardiac troponin; APC: Atrial premature contraction; PVC: Premature ventricular contraction.

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.