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Pulmonary Arterial Hypertension
New Developments in Pulmonary Arterial Hypertension: A Clinician’s Perspective

Released: October 27, 2023

Expiration: October 27, 2024

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Key Takeaways
  • Although an echocardiogram is the best screening tool we have today, an echocardiogram alone will not help define which grouping of pulmonary hypertension a person may have. 
  • The most common side effect seen with pulmonary arterial hypertension medications is vasodilation, which in turn may lead to headaches. 
  • A cardiologist or pulmonologist who is passionate about pulmonary arterial hypertension and has staffing to support the complex needs of this population is who the patient needs to see.

Pulmonary arterial hypertension (PAH) is a rare, progressive disorder characterized by high blood pressure in the arteries of the lungs with no specific cause. In January 2023, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) published the 2022 Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension (PH). Hemodynamic assessment by right heart catheterization provides the basis for the definition of PH. PH is now defined as a mean pulmonary arterial pressure (mPAP) >20 mm Hg, a pulmonary arterial wedge pressure (PAWP) of at most 15 mm Hg, and a pulmonary vascular resistance (PVR) >2 Wood units (WU). The 6th World Symposium on PH Classification gives us the 5 different groups of PH, with Group 1 being PAH, defined as mPAP >20 mm Hg, PAWP of >15mm Hg, and a PVR >3 WU. 

This expert commentary, provided by Melisa Wilson, DNP, expands on answers to questions that learners submitted at a presentation on PAH at the Cardiology and Pulmonary Summit for APPs (CAPSAPP).

Do you define right ventricular hypertension as >60 mm Hg?

PAH is deemed to be “likely” if the systolic pulmonary arterial pressure is greater than 40 mm Hg, and there is a high probability of PAH once that pressure reaches 60 mm Hg. Therefore, a pressure greater than 60 mm Hg would likely define right ventricular hypertension and be a potential indicator for underlying pulmonary hypertension. Although an echocardiogram is the best screening tool we have today, there are flaws and limitations that may not allow PAH to be properly diagnosed. There is a lot more to be learned in this area, but looking at an echocardiogram comprehensively beyond just that one number is going to help raise the level of suspicion for PAH. However, an echocardiogram alone will not help define which grouping of PH a person may have. 

At what age can PAH be diagnosed?

PAH can really happen at any age—from birth to older than age 65. PAH is certainly concerning in patients of advanced age since they often have comorbid conditions. PAH can be traced back to some underlying congenital heart disease at birth or present as persistent PAH of a newborn. 

What are the common side effects of agents used to treat PAH?

There are 3 drug pathways to treat PAH that all ultimately lead to vasodilation: the prostacyclin pathway, the endothelial pathway, and the nitrous oxide pathway. With vasodilation comes headache. The next most common side effect is nausea because these medications can affect the GI tract. However, the route of administration makes a difference, so diarrhea and reflux may be associated with these medications as well. 

Do you have concerns about telangiectasia and thrombocytopenia with sotatercept?

We have seen in trials that the telangiectasias are more of the cosmetic type, but we are concerned about the ones that are not visible. There is potential for telangiectasias to develop within the abdomen and cause bleeding, but that is something monitored in patients. Right now, telangiectasias are being well documented as superficial. As for thrombocytopenia, this is something that is seen often with PAH therapies. Again, this is one of those situations where you must monitor patients and identify their underlying etiology. As we learn more through studies of sotatercept, we will better understand the degree to which patients develop thrombocytopenia or other adverse events on the therapy vs placebo. 

Is oxygen helpful for patients with PAH?

Oxygen is one of the most challenging things for patients to be compliant with due to its visibility and the stigma associated with it. Oxygen is a very potent vasodilator, and no medication is going to replace the body’s need for oxygen. Patients with PAH may need both medication and oxygen; however, oxygen is predominantly used in patients who have oxygen desaturation. 

Is the cause of PAH in patients with HIV due to the virus itself or due to side effects from antiretroviral use?

With HIV, we have not found that PAH is linked to viral loads or related to any available treatments, it just tends to be more aggressive in that patient population in general. 

If you suspect a patient has PAH, would you refer them to pulmonary, cardiology, or both?

It does not matter what their specialty; a cardiologist or pulmonologist who is passionate about PAH is who the patient needs to see. Patients with PAH need a provider who is going to keep going and provide the support needed to get through treatment. Referral to and collaboration with PH experts and accredited centers is strongly encouraged. 

How much would you expect a general pulmonologist to do before referring the patient to a PH center?

Before referral to a specialist center, a pulmonologist can perform pulmonary function tests, 6-minute walk test, or obtain lung imaging like a chest CT or x-ray for evaluation and review by the expert PAH center. Right heart catheterization or echocardiograms are not required prior to referral to a PH center. It is imperative that the right heart catheterization be done comprehensively, include all the appropriate hemodynamic measurements, and vasodilator testing when indicated.

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Have you managed patients with PAH in your practice? Are you familiar with current treatment options? Leave a comment below to join the discussion.

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