There are five types of pulmonary hypertension (PH), a kind of blood pressure that affects your lungs and the right side of your heart. Pulmonary arterial hypertension (PAH) is the type that occurs when the high blood pressure in your lungs comes from narrowing, injury, or other obstructions of the small pulmonary arteries. (These arteries carry blood from the right side of your heart to your lungs.) Pregnancy can be extremely dangerous for people with PAH. In fact, due to the high rate of complications and mortality in pregnant people with PAH, it’s recommended that people with PAH avoid becoming pregnant.

Pregnancy for people with PAH is complex. In some cases, you may not know you have PAH before becoming pregnant, and in all cases, the decision to have or not have children is deeply personal. To better understand how pulmonary hypertension (PH) — and specifically PAH — is managed during pregnancy, myPHteam recently spoke with Dr. Anjali Vaidya. Dr Vaidya is an associate professor of medicine at the Lewis Katz School of Medicine at Temple University and serves as the co-director of the Pulmonary Hypertension, Right Heart Failure & CTEPH/PTE Program at Temple University Hospital.

Dr. Vaidya explained the risks of becoming pregnant, the inherent complications, and how pregnancy is managed for people with PAH.

Pregnancy Is Hard on the Heart

Even in people without PAH, pregnancy impacts a person’s heart function. “In any normal, healthy pregnancy, we would expect the woman to experience changes in her overall hemodynamics, which includes things like increased blood volume which can cause strain on the heart,” Dr. Vaidya explained. “When there's also pulmonary hypertension, the right side of the heart is particularly vulnerable to those changes of pregnancy.”

PAH is when you have an elevation of blood pressure in the blood vessels of your lungs (pulmonary arteries) that puts stress on your heart. It can thus lead to heart failure. Throughout any pregnancy, your heart rate increases to account for a 30 percent to 50 percent increase in blood volume. Heart failure, therefore, for people with PAH is even more likely during pregnancy as your already taxed heart may not be able to adapt to the increase in your blood volume.

Many people are newly diagnosed with PAH during pregnancy. A nine-year-study from Beijing that looked at 36 people with PAH who became pregnant noted as many as 72 percent of them did not have PAH symptoms and/or were unaware of their condition before they became pregnant. In other cases,they may not have noticed the condition prior to becoming pregnant because their symptoms were not serious prior to their pregnancy.

Pregnancy Complications Related to Pulmonary Arterial Hypertension

Treating your PAH becomes difficult as a result of pregnancy — and the maternal mortality rate among pregnant people with PAH indicates this. One study looked at the published case studies with PAH patients over the decades. The authors found 73 pregnant people with PAH in the pool, and among them, there was a maternal mortality rate of 28 percent.

“The risk of heart failure and not surviving the pregnancy are unfortunately quite high,” Dr. Vaidya said. “In addition to that, so many of the medications that are required to keep our patients alive and well are also high-risk during pregnancy. In other words, they have the risk of causing abnormal effects on the baby.”

Dr. Vaidya explained that it is a difficult balance, as PAH is difficult to treat without medication, and without treatment a pregnant person with PAH will suffer.

Even if PAH medications continue to be prescribed, Dr. Vaidya emphasized there are fewer options for pregnant people. Many cannot be prescribed unless you have a recent negative pregnancy test, and available PAH medicines can cause birth defects, disrupt your baby’s development, or negatively impact them.

Pregnancy Is Not Recommended for People With PAH

Specialists generally suggest people with PAH avoid becoming pregnant. That is due to the difficulty in treating your PAH during pregnancy and because of the higher risk of both morbidity and maternal death among pregnant people with PAH. Given the consequences, when you are first diagnosed with PAH ask your doctor about the associated risks of pregnancy. If you are newly diagnosed with PAH, you should be told the medical guidelines, which recommend not becoming pregnant if you have pulmonary arterial hypertension, said Dr. Vaidya.

Contraceptive Options for People With Pulmonary Arterial Hypertension

Ask your doctor for guidance on contraception when you have been diagnosed with PAH. This is especially important because some forms of estrogen-based oral contraceptives increase your likelihood of developing blood clots, something people with PAH should avoid. Instead, you might consider progesterone-based birth control options, which are safer for people with PAH, and non-hormonal options as well.

Termination of the Pregnancy Is Generally Advised

Although some people with PAH know the risks and still choose to become pregnant, others may not know that they have PAH until they become pregnant. Such newly diagnosed people are faced with a difficult choice. “I have seen patients that have been told they have pulmonary hypertension at the time of pregnancy, and they have been advised by their doctors to terminate their pregnancy,” Dr. Vaidya explained.

The guidelines followed by specialists state that a pregnant person with PAH should be informed of the significant risk involved, and they should be advised to terminate. That said, pregnancy termination if you have PAH carries an even greater risk than it does with the general population. An abortion for a person with PAH should be performed by experienced doctors.

Dr. Vaidya emphasized that even though guidelines recommend termination, the decision about whether or not to continue the pregnancy is complicated and personal. “I try to be very supportive and give all the facts and the guidelines and the information, so the patients can make their own individual decisions,” Dr. Vaidya explained. “Understandably, very often the decision is to continue with pregnancy.”

Ultimately, it is up to you to make the decision you feel is best. No matter your decision, working out a plan with your care team to either terminate the pregnancy as safely as possible or to give you the best chance for the healthiest pregnancy is ideal.

Management of Pregnancy With Pulmonary Arterial Hypertension

It is possible to have a safe pregnancy with PAH — with the right kind of care. If you choose to remain pregnant, you will require a team of experts to monitor your condition to maximize your chance of survival and potential for a successful pregnancy.

Dr. Vaidya stated that her hospital has an excellent success rate among pregnant people with pulmonary hypertension. She attributes that to the hospital’s multidisciplinary team, which is composed of pulmonary hypertension cardiologists, maternal fetal medicine obstetricians, and anesthesiologists with obstetric expertise specifically for pregnant people with PAH.

Monitoring by a cardiologist with expertise in PH, in addition to obstetric care, is necessary to keep an eye on your heart throughout your pregnancy. A cardiologist will likely order blood tests to assess if your heart is under strain. They will also likely evaluate the structure and function of the right side of the heart with echocardiograms.

Right-heart catheterization might be necessary. This procedure tells you about your intracardiac filling pressures and pulmonary artery pressure (and stiffness), and how much blood the heart is pumping. Dr. Vaidya stated that this information helps to ensure your treatment plan keeps up with the physiological changes that take place throughout your pregnancy. And it allows your health care team to adjust medications as necessary.

“Some patients, depending on how severe their pulmonary hypertension is and the severity of their right heart failure, might be admitted to the hospital for days or weeks to keep them in a safe range up until their delivery,” Dr. Vaidya said.

“The mode of delivery should be based on what the OB-GYN and the maternal fetal medicine experts recommend,” Dr. Vaidya explained. There isn’t a recommendation that all people with PH delivery vaginally or via cesarean section (c-section).

Rather, Dr. Vaidya said, at the time of delivery it is important that your obstetric anesthesiologist and cardiologist work together to determine the best way to support how your pulmonary hypertension affects your cardiovascular system. The involvement of your cardiologist throughout your pregnancy can ensure that you are in the best health possible for the delivery, and that the type of delivery is most appropriate for you.

Although some doctors feel c-sections have lower risks than vaginal delivery for people with PAH, Dr. Vaidya pointed out that the anesthesia and bleeding involved in a c-section come with risks as well, and with that, this type of delivery should be evaluated on an individual basis.

Postpartum Care Should Not Be Overlooked

Heart monitoring must continue for people with PAH in the days and weeks after you’ve given birth. Dr. Vaidya explained that in her facility, people with PAH are monitored closely by PH cardiologists, and anyone who is not stable may require an extended hospital stay. Even after you are discharged from the hospital, if you have PH, you should follow up with your health care team in the weeks following birth.

And while everyone’s pre-pregnancy blood volume returns fairly quickly following birth, hemodynamics (blood flow) can take weeks to return to normal. Dr. Vaidya noted that sometimes fluid build-up needs to be eliminated and requires intravenous diuretics. Depending on the severity of your condition, a person with PAH may continue to be at risk for mortality following their pregnancy — another reason why continued close monitoring is important.

Talk With Others Who Understand

On myPHteam, the social network for people with pulmonary hypertension and their loved ones, nearly 40,000 members come together to ask questions, give advice, and share their stories with others who understand life with pulmonary hypertension.

Are you living with pulmonary arterial hypertension while pregnant? Do you have PAH and want to bear a child? Share your experience in the comments below, or start a conversation by posting on your Activities page.