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Management Strategies
Preconception counseling/Contraceptive methods
Women should be advised to meet with their cardiologist prior to becoming pregnant so that their baseline status can be determined. Ideally, symptoms related to PS or moderate or severe PS with associated RV dysfunction should be addressed prior to pregnancy with a percutaneous or surgical intervention, if necessary.
The indications for surgical or interventional procedures prior to pregnancy should follow recommendations in current guidelines. (4,5,6)
Asymptomatic women with isolated PS and normal RV function are likely to tolerate pregnancy well without incident. (1,2) It may be useful perform a treadmill or cardiopulmonary exercise test as a screening test for women with underlying RV dysfunction or minimally symptomatic PS to help assess how they will tolerate the hemodynamic stress of pregnancy (3).
A discussion about contraceptive options is appropriate in women with PS. In general, most forms of contraception are safe in women with PS. (see Contraception)
Transmission of congenital heart disease to offspring should be discussed. The risk of transmission of congenital heart disease is approximately 5% for women with isolated PS, compared to a background risk of approximately 1% in the general population. For women with Williams, Noonan and Alagille syndromes, transmission to offspring differs. These syndromes have autosomal dominant transmission and confer up to a 50% chance of transmission to the fetus.
Medication use should be reviewed if a woman is contemplating pregnancy or is pregnant. The MOTHERISK website is an excellent resource.
Ante-partum Care
Women with symptoms, RV dysfunction or PS that is moderate or greater in severity should be followed at a hospital center that specializes in high-risk pregnancies. Coordinated care between a heart specialist, a high-risk obstetrician and an anesthetist should be implemented. The frequency of follow-up visits and echocardiograms should be dictated by the specialists.
In women with low risk features, depending on their preferences, antenatal care and delivery can be performed at non-specialized centers.
Although a percutaneous balloon valvotomy is preferable to perform prior to conception, when necessary, percutaneous balloon valvotomies with uterine shielding during pregnancy have been performed. (7) A surgical intervention is usually required to manage subvalvular PS and some forms of supravalvular PS.
All women with PS should be offered fetal echocardiography at 20 weeks gestation.
Labour and Delivery
For women with moderate or severe PS or RV systolic dysfunction, labor and delivery should be planned carefully with a multidisciplinary team well in advance. It is important to communicate the delivery plan to the woman and to other physicians involved in her care. The best delivery plan is not useful if information is not readily available when needed.
Generally, vaginal delivery is recommended. Good pain management for labour and delivery is important in order to minimize maternal cardiac stress.
For most deliveries of women with PS, maternal monitoring is not required.
In general, endocarditis prophylaxis at the time of labour and delivery is not recommended in women with PS. However, some experts continue to administer antibiotics because they feel that the risks of adverse reactions to antibiotics are small and the risk of developing endocarditis has major health consequences.
Post-partum Care
The hemodynamic changes of pregnancy may take up to six months to normalize. Women should be seen early after pregnancy (usually within 6-8 weeks). The frequency of additional follow up visits should be dictated by the clinical status of the women.
