Pregnant Women with PAH Require Careful Monitoring, Management, Says Review

Women with pulmonary arterial hypertension (PAH) are discouraged from getting pregnant due to a high risk of morbidity and mortality for both mother and child. Yet, some patients with PAH choose to become pregnant despite the guidelines. A new review article says such patients can successfully bring a child to term, but only with careful management by a team of physicians.

Corresponding author Wilbert S. Aronow, M.D., of New York Medical College, and colleagues, explained that pregnancy sparks a series of physiological changes, including increased blood volume, heart rate, and cardiac output (CO).

“These changes are necessary to support fetal development, but can exacerbate the already compromised cardiovascular function in PAH patients,” they wrote in Cardiology in Review.

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DetectionRight ventricular failure, cardiac arrest, pulmonary hypertensive crisis, preeclampsia and sepsis are the primary causes of morbidity and mortality, Aronow and his colleagues noted, citing a 2022 meta-analysis. That study found that while the majority of pregnancies among patients with PAH advanced past 20 weeks, 58% of the pregnancies resulted in premature deliveries, most by cesarean section. They calculated a maternal mortality rate of 12%, a stillbirth rate of 3% and a neonatal mortality rate of 1%.

In the new review, Aronow and colleagues said management of PAH during pregnancy begins by making patients aware of the risks before they decide to become pregnant, including the risk that PAH can worsen even after the pregnancy ends.

“For those who choose to continue with the pregnancy, meticulous planning and continuous monitoring are imperative,” they wrote.

They suggest patients be cared for by a multidisciplinary team including obstetricians, cardiologists, anesthesiologists, hematologists and neonatologists. It is common, the authors said, for women to be admitted to the hospital in the second trimester to allow for more careful monitoring, and then again 24 to 48 hours prior to a planned delivery.

"On the day of delivery, patients are taken to the intensive care unit for the insertion of a central venous catheter and an arterial line to facilitate continuous monitoring of central venous pressure, blood pressure, and CO,” they wrote.

Additional careful monitoring in an intensive care unit is necessary for several days following the birth, they said, due to the risk of decompensation, among other concerns.

When it comes to treating the PAH itself, the authors said the ideal situation is for the patient to be taking PAH therapy prior to the pregnancy. However, they added that certain PAH therapies — including Adempas (riociguat), endothelin receptor agonists, and guanylate cycle stimulates — should be stopped because they can interfere with fetal development. Prostacyclin analogs and phosphodiesterase type 5 (PDE-5) inhibitors like Viagra (sildenafil) appear to be safe in pregnant patients, the authors said.

William S. Aronow, M.D.

During pregnancy, prevention of right ventricular failure should be a key focus, the authors said, and warrants therapies like intravenous epoprostenol and inhaled Ventavis (iloprost), among others. Most patients with PAH are also given an anticoagulant during and after pregnancy, the investigators noted, due to the risk of thromboembolic events. However, they said anticoagulants are typically paused in the period just before and after delivery. They said there is insufficient evidence to say which particular anticoagulants are ideal.

Aronow and colleagues said their review of existing literature showed there are significant gaps in data regarding the safety and efficacy of specific PAH therapies. They added that the development of newer therapies, like Winrevair (sotatercept) and gene therapies, offer mechanistic insights into PAH that might help with patient management.

“Ultimately, enhancing our understanding of PAH in the context of pregnancy will lead to better management strategies, reduced morbidity and mortality, and improved quality of life for affected women,” the investigators concluded.