Pregnancy should not be ruled out for all women with pulmonary hypertension
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A patient in her early 30s with a history of severe pulmonary hypertension came into my office and expressed that she would like to start a family.
For women with pulmonary hypertension (PH), blood flow from the heart to the lungs is under dangerously high resistance, leading to right heart failure, making pregnancy very risky. In fact, it is often life-threatening, with maternal-fetal mortality rates reported between 30% and 50%.
The combination of PH and pregnancy presents unique medical challenges, owing to the additional stress that pregnancy and delivery place on the heart.
Given those challenges, women with PH are often advised to avoid pregnancy. However, not every case of PH is the same, and pregnancy shouldn’t be out of the question for all women with PH. When I first saw this patient years earlier, she had undiagnosed, untreated PH so severe that she would become short of breath doing routine household chores. She also had an atrial septal defect (ASD), which is a hole in the wall (septum) that divides the upper chambers of the heart. A previous surgery to address the ASD made the defect smaller but did not close it.
I diagnosed her PH and began medical treatment. She improved significantly, but still had symptoms with moderate activity. Once her PH symptoms improved, we turned our attention toward determining whether we could close the ASD for good. Typically, an ASD is left alone or only partially closed when a patient has significant PH. But my patient had responded so well to PH therapy, we had the needed combination of physiologic factors to safely proceed with surgery to close the ASD.
It was life-changing for her. She started living a more active life and even contemplated starting a family. Now that her PH was under control and the ASD was closed, would it be safe for her to carry a pregnancy?
We conducted two additional tests to help make this determination: an echocardiogram, which showed the right heart size and function had improved to near-normal levels, and a right heart catheterization to reassess the resistance in the pulmonary arteries. During the catheterization, we administered an inhaled PH medication that showed the resistance in the pulmonary arteries dropped to normal levels. We thus had the safety margin we needed to give the green light for pregnancy.
This patient was followed closely by our multidisciplinary team, including experts in PH, maternal fetal medicine, obstetric anesthesiology and cardiology.
As a group, we’ve developed an approach to the management of pregnant patients with PH that focuses on optimizing and monitoring right heart function throughout pregnancy and the postpartum period.
We regularly monitored the right side of the patient’s heart and used serial imaging to adjust her medications as needed to achieve a safe pregnancy.
The patient did very well and gave birth to a healthy baby boy. A few years later she carried a second pregnancy, another boy. Both boys and our patient are doing great.
In recently published research, we showed that maternal-fetal mortality in select women with PH who become pregnant can be reduced to zero through a patient-tailored management approach like ours.
It’s our hope that similar multidisciplinary efforts will be undertaken at other institutions so that outcomes can be improved for pregnant women with PH.