Pregnancy in women with congenital heart disease: a focus on management and preventing the risk of complications

Figures & data

Figure 1. Estimated incidence of the common congenital heart diseases per 1,000 live births (indicated in parentheses)4.

Table 1. Gene defects causing CHD (the ones that are known till now, the list with keep changing as new gene linkages are found).

Cardiac defects	Gene mutations causing the CHD
ASD	NKX2–5 GATA4 TBX20 MYH6
VSD	NKX2–5 GATA4 TBX20
Ebstein’s anomaly	NKX2
DORV/TGA	NKX2–5 THRAP2
TOF	NKX2–5 NOTCH1
BAV	NOTCH1 SMAD6 MAT2A
HLHS	NOTCH1

Note: ASD – Atrial septal defect, VSD – Ventricular septal defect, DORV – Double outlet right ventricle, TGA – Transposition of great arteries, TOF – Tetralogy of Fallot, BAV – Bicuspid aortic valve, HLHS – Hypoplastic left heart syndrome.

Table 2. Risk of CHD in offspring of mothers with CHD according to different diseases.

Congenital disorder	Risk of inheritance
ASD, PDA, Ebstein’s anomaly, Coarctation of Aorta	4.5–6%
VSD, BAV, PV stenosis	~7%
Atrioventricular septal defect	7.5–15%
Tetralogy of Fallot	2.5–10%
Transposition of great arteries	0.5%
Marfan syndrome	50%

Note: CHD- Congenital heart disease, ASD – Atrial septal defect, PDA – Patent ductus arteriosus, VSD – Ventricular septal defect, BAV – Bicuspid Aortic valve, PV – Pulmonary valve.

Table 3. Modified WHO (m WHO) risk stratification classification.

WHO I	Pulmonary stenosis (small/mild) Patent ductus arteriosus (small/mild) Mitral valve prolapse (small/mild) Successfully repaired simple shunt defects (ASD, VSD, PDA) APVR
WHO II	Unrepaired ASD or VSD Repaired tetralogy of Fallot Turner syndrome without aortic dilatation
WHO II- III	Mild left ventricular impairment Native or tissue valve disease not considered WHO I or IV Marfan or other HTAD syndrome without aortic dilatation Aorta < 45 mm in bicuspid aortic valve Repaired coarctation AVSD
WHO III	Left ventricular impairment (30–45%) Mechanical valve Systemic right ventricle with good or mildly impaired function Fontan (if otherwise well) Unrepaired cyanotic disease Severe asymptomatic aortic stenosis Moderate aortic dilatation
WHO IV: (pregnancy not recommended)	Pulmonary arterial hypertension Severe systemic ventricular dysfunction (EF < 30%) Moderate systemic right ventricular dysfunction Severe symptomatic aortic stenosis Severe aortic dilatation Vascular Ehlers-Danlos Severe (re) coarctation Fontan with any complication

Note: APVR = anomalous pulmonary venous return, ASD = atrial septal defect, AVSD = atrioventricular septal defect, EF = ejection fraction, ESC = European Society of Cardiology, HTAD = hereditary thoracic aorta disease, PDA = persistent ductus arteriosus, VSD = ventricular septal defect, WHO = World health organization.

Adapted and modified for congenital heart disease, from the ESC 2018 “cardiovascular diseases during Pregnancy (management of) Guidelines.

Table 4. Predictors of adverse maternal cardiovascular events during pregnancy.

ZAHARA	CARPREG II
Risk Factors	Risk Factors
History of arrhythmias (1.5 points) Cardiac medications before pregnancy (1.5 points) NYHA class >II (0.75 points) LVOT with peak gradient >50 mmHg or AVA <1 cm^2 (2.5 points) Moderate or severe systemic AV valve regurgitation (0.75 points) Pulmonary AV valve regurgitation (0.75 points) Mechanical valve prosthesis (4.25 points) Cyanotic heart disease (1.0 point)	Prior cardiovascular events or arrhythmia (3 points), NYHA class >ll or cyanosis, resting oxygen saturation < 90% (3 points), mechanical valve (3 points), systemic ventricular dysfunction with LVEF < 49% (2 points), high-risk left-sided obstruction, peak LVOT >30 mmHg, AVA < 1.5 cm2; (2 points), pulmonary hypertension (2 points), coronary artery disease (2 points), high-risk aortopathy (2 points), no prior cardiac intervention (1 point), later pregnancy assessment (2points) NYHA class >ll or cyanosis, resting oxygen saturation <90% (3 points) Mechanical valve (3 points) Systemic ventricular dysfunction with LVEF < 49% (2 points) High-risk left-sided obstruction, peak LVOT >30 mmHg, AVA <1.5 cm^2; (2 points) Pulmonary hypertension (2 points) Coronary artery disease (2 points) High-risk aortopathy (2 points) No prior cardiac intervention (1 point) Later pregnancy assessment (2 points)
Score CV Risk	Score CV Risk
0–0.5→2.9%	0–1→5%
0.51–1.50→7.5%	2→10%
1.51–2.50→17.5%	3→15%
2.52–3.50→43.1%	4→22%
≥3.51→70.0%	≥4→41%

Note: AVA = aortic valve area.

Figure 2. Hemodynamic changes during pregnancy.

Figure 3. Changes in blood flow in pelvic blood vessels during pregnancy compared to non-pregnant state.

Figure 4. Graphs depicting release of anti-angiogenic factor soluble fms-like tyrosine kinase-1 (sFlt1) and soluble endoglin (eEng) and proangiogenic placental growth factor (PlGF)23.

Figure 5. Schematic representation of management issues in pregnant women with aortic stenosis.

Table 5. Delivery planning in women with CHD.

Care Plan	Multi-disciplinary team including ACHD cardiologist, obstetrician, maternal medicine specialist and anesthetist Outline timing, location, and mode of delivery by 20–32 weeks Identify monitoring needs (CTG, invasive lines, IV filters) Doses and medications considered safe for post-partum hemorrhage prophylaxis and treatment Need for ITU/HDU post delivery Post-partum thromboprophylaxis Needs for echocardiogram with cardiology review post delivery Approximate post-natal stay Discuss contraception post delivery Medications to continue
Anaesthesia	Epidural/Regional anesthesia should be considered for all women with CHD Slow titration of regional anesthesia should be considered for women with complex CHD
Mode of delivery	Vaginal delivery preferred for most women with CHD CS generally reserved for obstetric indications. Time allowed for active second stage of labor. Assisted second stage (vacuum, forceps) for LVOT obstruction (severe AS, subvalvular stenosis, HOCM) pre-load dependent lesions or critical disease (aortopathy, Fontan circulation, pulmonary hypertension). CS preferred for certain WHO III/IV patients (severe pulmonary hypertension, critical left sided valvular disease, severe aortopathy).

Note: ACHD – Adult Congenital Heart Disease, CTG – cardiotocography, IV – intravenous, WHO – World Health Organization, CS = Caesarean section, LVOT- left ventricular outflow tract obstruction, AS- aortic stenosis, HOCM- hypertrophic obstructive cardiomyopathy.