Understanding primary pulmonary arterial hypertension


Delve into the complexities of primary pulmonary arterial hypertension, exploring its clinical manifestations, recommended management strategies, and the pressing need for further research, particularly in the context of pregnancy.

Understanding primary pulmonary arterial hypertension | Image Credit: © Yakobchuk Olena - © Yakobchuk Olena - stock.adobe.com.

Understanding primary pulmonary arterial hypertension | Image Credit: © Yakobchuk Olena - © Yakobchuk Olena - stock.adobe.com.

In a recent review, investigators discussed the clinical presentation, management methods, and research needed related to primary pulmonary arterial hypertension (PAH)


  1. Pregnant women with pulmonary arterial hypertension (PAH) face significant risks, with mortality rates ranging from 9% to 17%, highlighting the importance of careful management and counseling against pregnancy.
  2. Symptoms of PAH overlap with those of pregnancy, potentially leading to delayed diagnosis and treatment, while risks include right ventricular stress, failure, and death.
  3. While Right Heart Catheterization is the gold standard for diagnosing PAH, its use during pregnancy is limited because of risks to the fetus, necessitating careful consideration of alternative diagnostic approaches.
  4. Multidisciplinary management involving experienced teams is crucial for pregnant PAH patients, with risk stratification, close monitoring, and tailored treatment plans essential for optimizing outcomes.
  5. PAH poses risks not only to maternal health, including right heart failure and hypertension crisis, but also increases the likelihood of adverse fetal outcomes such as low birth weight and neonatal mortality, emphasizing the need for comprehensive care strategies.

Pulmonary hypertension (PH), defined by a resting mean pulmonary artery pressure of 20 mm Hg or greater, can be primary or secondary to other conditions. PH presents as elevated pulmonary pressures that can cause abnormal cellular, prothrombotic, and vascular constriction outcomes. Risks of PH include right ventricular (RV) stress and RV failure and death.

PAH is 4 times more common in women vs men, making it the most common PH type among women of reproductive age. Pregnant patients with PAH have a mortality rate from 9% to 17%, with most guidelines recommending against pregnancy. However, data has indicated promise for improved outcomes.

PH evaluation often occurs after right ventricular systolic pressure detection, with a complete diagnostic evaluation and risk stratification recommended. Staff from an experienced center should perform multidisciplinary management among pregnant patients.

Symptoms of PAH include dyspnea, fatigue, hepatomegaly, syncope, ascites, and ankle edema. These symptoms might occur during pregnancy but are also signs of increased RV stress. Investigators noted the need for research about the source of PAH during pregnancy, which could be heritable, idiopathic, or other disease associated.

Pregnant patients should be referred to a PAH experienced center when PAH is confirmed, and early termination is recommended. Additionally, PAH patients should receive counseling about contraception and breastfeeding. Risk stratification is vital for PAH patients seeking to continue pregnancy.

Significant changes in the cardiovascular system occur during pregnancy, including a 30% to 50% increase in cardiac output and blood volume during the third trimester. Hormonal changes during pregnancy may also cause an accumulation of fluid in the interstitial space, further impacting the circulation system during delivery.

These changes, as well as a potential 30% decrease in pulmonary vascular resistance during pregnancy, indicate a risk of increased severity from PAH symptoms. Shared symptoms between pregnancy and PAH also lead to delayed diagnosis and treatment.

When evaluating for PAH, a careful history and physical examination should be conducted. This includes gathering data about the age of onset and specific disease type and severity. Risk factors including family history of PAH, exposure to PAH-inducing drugs or toxins, HIV, portal hypertension, congenital heart diseases, and connective tissue diseases should be thoroughly considered.

The World Health Organization functional class and REVEAL risk score can be used for risk stratification. However, there has not been a tool validated among pregnant women.

Data has indicated favorable outcomes among pregnant women with class 1 and class 2 PAH, with a 4% death rate and all deaths among women with class 4 reported. Investigators recommended monthly symptom and functional status monitoring during the first and second trimester and weekly monitoring during the third trimester.

Authors referred to Right Heart Catheter as, “the diagnostic gold standard for PAH, the parameters of which help classify PAH into several subgroups, assess the risk of adverse events or mortality, and make therapeutic decisions.” However, this procedure should be avoided during pregnancy because of a risk of radiation to the fetus.

Management of PAH among women of reproductive age should be performed routinely by an experienced multidisciplinary team. Pre-conceptive counseling and monitoring can be utilized to treat patients, as well as general and supportive care, medication and immune therapy, delivery and postpartum care, contraception and breastfeeding counseling, and cardiac surgery.

In general care, providers should optimize care, avoid teratogenic drugs, employ close monitoring, and utilize a multidisciplinary team approach. Treatment will often be tailored to the individual patient.

An expert multidisciplinary team for PAH patients undergoing delivery should include obstetricians, PH specialists, anesthesiologists, neonatologists, and critical care professionals. Lower-risk patients can be managed using oral phosphodiesterase inhibitors and inhaled prostacyclin analog, while intravenous medication may be necessary in higher-risk patients.

Mothers with PAH are at risk of right heart failure, hypertension crisis, cardiac arrest pulmonary, premature labor or birth, and stroke. Fetal outcomes such as low birth weight, week of delivery, neonatal preterm birth, small for gestational age, neonatal asphyxia, and neonatal mortality are also increased.

These guidelines indicate risk factors, symptoms, and treatment methods of PAH. Investigators recommended further research to explore the relationship between PAH, oligohydramnios, and preeclampsia.


Teng Y, Zong L, Ding J, Wu M, Li X. Management of pulmonary arterial hypertension: before, during and after pregnancy. Int J Cardiol Cardiovasc Risk Prev. 2024;21:200252. doi:10.1016/j.ijcrp.2024.200252

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