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Page 12 of 17 Saxena et al. Vessel Plus 2022;6:15 https://dx.doi.org/10.20517/2574-1209.2021.96

reported to be very high in these patients (16%-30% for PH and 20%-50% for Eisenmenger syndrome), but
the recent European registry results are optimistic with < 5% mortality in their cohort of 151 pregnant
women with PH [44-47] . The greatest risk of mortality is during the first six weeks after delivery. The common
causes of death include pulmonary hypertensive crisis, pulmonary thrombosis, or refractory right heart
failure. Maternal death may occur even in patients with few symptoms before pregnancy . Although there
[47]
is no safe cut-off for elevated pulmonary arterial pressure, patients with mild PH may tolerate the pregnancy
well. However, the recommendation to avoid pregnancy remains, and, if pregnancy occurs, termination is
advisable. There is increased fetal and neonatal mortality (0-30%) with the severity of reduction in maternal
cardiac output and degree of hypoxemia being the most important predictors .
[1]

If the patient chooses to continue pregnancy, extreme care during pregnancy, delivery, and in the
postpartum period must be exercised. These patients are particularly at risk for thromboembolism, bleeding
complications, and heart failure. Bed rest, meticulous fluid balance, and supplemental oxygen help.
Phosphodiesterase inhibitors are often prescribed, with the addition of prostanoids in patients with
persistent symptoms. Anticoagulation should be carefully considered as it may induce hemoptysis. Iron
deficiency is common and should be treated with supplemental iron. Vaginal delivery or planned caesarean
delivery are preferred over emergency caesarean delivery. Regional anesthesia is preferred over general
anesthesia. Delivery should be performed in an experienced tertiary center.

Cyanotic Congenital heart disease with pulmonary stenosis
In women with repaired Tetralogy of Fallot, pulmonary regurgitation is the most common residual lesion
and is generally tolerated well in pregnancy unless associated with RV dysfunction. Akagi et al. reported
[48]
data from 143 pregnancies in patients with repaired Tetralogy of Fallot. There were no maternal deaths, but
a minority of patients had heart failure (11%), worsening of arrhythmia (8%), premature birth (10%), and
miscarriage (5%). In general, patients eligible for pulmonary valve replacement or other interventions
should undergo procedure before conceiving for best outcomes.

On the other hand, in patients with uncorrected CHD, the maternal outcome is determined by the
underlying condition and ventricular function while the fetal outcome is predominantly determined by the
saturation level regardless of the anatomical complexity . Maternal complications (heart failure,
[49]
thrombosis, arrhythmias, and endocarditis) can occur in almost one-third of women and pregnancy should
be discouraged in such patients. However, in those who have already conceived, patients should be advised
to restrict physical activity and use compression stockings to prevent venous stasis (to avoid paradoxical
embolism). Thromboprophylaxis with low molecular weight heparin should be considered if there are no
contraindications. Vaginal delivery is advisable unless there is an obstetric indication for caesarean delivery.

Left ventricular outflow tract obstruction
The morbidity and mortality in left ventricular outflow tract obstruction is related to the severity of
obstruction and symptom status. The risk of obstetric complications is increased in patients with severe
aortic stenosis. Preterm delivery, fetal growth retardation, and low birth weight occur in 20%-25% of
offspring of mothers with moderate and severe AS, although miscarriages and fetal death rates are < 5% .
[50]
Severe symptomatic left ventricular outflow tract obstruction is an absolute contraindication for
[1]
pregnancy . Even asymptomatic patients with severe left ventricular outflow tract obstruction and impaired
LV function or a pathological exercise test should be counseled against pregnancy. These patients should
undergo intervention before conceiving. Asymptomatic patients with severe valvular aortic stenosis and
normal left ventricular function may tolerate pregnancy well provided they remain asymptomatic.
Guidelines recommend exercise testing in these asymptomatic patients before pregnancy to evaluate
exercise tolerance, BP response, and arrhythmias . Those with bicuspid aortic valve and associated dilated
[1]