The literature has described the risk of pregnancy to the mother as very severe in patients with advanced PAH. Depending on how advanced the PAH, there may be a 50% to 80% chance of death. For patients with much milder PAH, the risks are much lower but still substantial.
More recently as our treatment options for PAH patients have improved, the reported results have shown that in very carefully selected patients with well controlled pulmonary arterial hypertension, who are managed by doctors with tremendous expertise, pregnancy can be undertaken. So that everyone understands the key message here, pregnancy is always discouraged in patients with pulmonary arterial hypertension. However, recognizing that some women will wish to proceed with pregnancy regardless of the risks, it is imperative to find a PAH team that can help minimize the risks.
Discussing Pregnancy With Your PH Team
Pregnancy in PAH patienst should never be a surprise event. Sexually active women should be using highly effective birth control such as depot provera, IUDs, or condoms with spermacide worn from start to finish. In select patients that have very mild PAH and are considering pregnancy, a discussion with their PAH physician should begin very early– long before you stop your birth control. The PH doctor will discuss with you the risks based on how you are doing. In my practice we would perform a right heart catheterization, an echo, blood tests and a six minute walk test to thoroughly understand how you are doing from a pulmonary hypertension perspective. Next we would have a very frank discussion where I would try and persuade you not to get pregnant. I would then send you to see a high risk obstetrician who would similarly try and persuade you to consider other options for becoming a parent such as adoption. If you were still intent on proceeding we would adjust your medications to remove the ones that are toxic to the fetus. We would also likely place you on continuous prostanoid therapy such as subcutaneous Remodulin. We would see you frequently and meet regularly as a team with your high risk OB. In the third trimester we would see you weekly. We would formulate a plan for delivery with the OB team. We often prefer to induce delivery to avoid delivery when the team is not available. Delivery is a big deal. We typically have the pulmonary arterial hypertension ICU nurses, the PAH doctor, the OB doctor and nurses, and an anesthesiologist all present. There might be 10 people in the delivery room. After delivery the safest place is the PAH ICU not the normal post-delivery ward. The period of great risk to the mother extends for the first few weeks post-partum (after delivery).
What If A PH Patient Accidentally Becomes Pregnant?
So what happens if you find that you are pregnant and it was not planned? This is a PH emergency. Immediately call your pulmonary hypertension doctor. After evaluating how you are doing from a PAH perspective and adjusting your medications, we would have a very frank conversation with you and your partner. The vast majority of the time, pregnancy termination is the correct course of action to prevent the mother from a high risk of dying during the pregnancy. This is always a very difficult decision.
See Pregnancy and Pulmonary Hypertension Part 1 for additional information on why pregnancy is dangerous for women with PAH.
