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Page 8 of 17 Saxena et al. Vessel Plus 2022;6:15 https://dx.doi.org/10.20517/2574-1209.2021.96

Table 2. Risk of CHD recurrence in the fetus
Type of CHD in mother Risk of recurrence
Tetralogy of Fallot 2.0-4.5
Patent ductus arteriosus 4.1
Coarctation of aorta 4.1-6.3
Atrial septal defect 4.6-11
Pulmonary stenosis 5.3-6.5
Ventricular septal defect 6.0-15.6
Atrioventricular septal defect 7.9-13.9
Aortic stenosis 8.0-13.9
Marfan syndrome 50
22q11 deletion syndrome 50

CHD: Congenital heart disease.

In LMIC, women often present for the first time with clinical features of heart disease when already
pregnant. When these patients present with symptoms in the emergency room for the first time, the treating
physician should formulate a diagnostic impression based on the cursory history and focused examination,
supplemented by electrocardiogram for planning immediate management to stabilize the patient. Chest
radiography should be avoided if possible. The physician should identify the subgroup in which the patient
falls, i.e., cyanotic or acyanotic (shunt/obstructive) disease with increased or decreased pulmonary blood
flow, followed by identification of precipitating cause (anemia, polycythemia, infective endocarditis,
arrhythmia, physiological changes of pregnancy, ventricular dysfunction, thromboembolism, etc.) for
presenting symptoms. Once stabilized, patients should undergo detailed echocardiography followed by
other imaging, as deemed necessary.

These patients need complete assessment by a multidisciplinary team and risk stratification as is performed
preconception. Periodic follow-ups should be planned as per the risk stratification [Table 1A and B]. If the
assessment reveals a high-risk condition, termination of pregnancy should be recommended early in
pregnancy. However, termination of pregnancy also carries risks in women with high-risk lesions and
should be performed at a center with the necessary expertise.

MANAGEMENT OF PREGNANCY IN WOMEN WITH CONGENITAL HEART DISEASE
For each case, a clear follow-up and delivery plan should be made and discussed with the patient. The
frequency of follow-up visits depends on the risk assessment. The European Society of Cardiology has given
recommendations for management of CHD during pregnancy [Table 1B] . High-risk pregnancies are best
[1]
managed by an experienced multidisciplinary team in a tertiary care center, while those with low risk can be
managed in state-level regional centers. The patient and her family should be educated about the anticipated
symptoms and need for frequent assessment. In addition, fetal echocardiography should be offered to all
mothers with CHD between 18 and 22 weeks of gestation. It may be performed earlier if there is suspicion
of CHD on ultrasonography. The fetal echocardiography protocol should include complete sequential
segmental analysis and should not be limited to screening views. It is important to highlight that the
recurrence of CHD in the fetus may not be identical to the CHD type in the affected family member.

Timing and mode of delivery
The timing of delivery depends on maternal hemodynamic status and obstetric evaluation including cervical
assessment, fetal well-being, and fetal lung maturity. Guidelines recommend elective induction of labor at
40 weeks of gestation under controlled conditions in all women with cardiac disease as it reduces the risk of