fatigue,blood pressure,hypertension,high blood pressure,pulmonary problems,difficulty breathing,pregnancy stage,con-00062,newbie mom,greener,Pulmonary Arterial Hypertension Can Be Fatal,pulmonary arterial hypertension, PAH, high blood, hypertension, difficulty in breathing, alta presyon,Pulmonary Arterial Hypertension is a rare disorder that does not get much attention. That's what doctors find alarming.
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Konting Lakad, Hinahapo Na? You're Thinking COVID-19 But It Could Be A Rare Disease

Women in their reproductive years are more affected by this rare and progressive disease.
PHOTO BYShutterstock/Boyloso

With the COVID-19 pandemic, people who experience difficulty in breathing may automatically think they have caught the virus. But a breathing problem is a symptom of many underlying health problems. One such condition is pulmonary arterial hypertension (PAH).

What is pulmonary arterial hypertension

Pulmonary arterial hypertension is a “rare and progressive disorder characterized by high blood pressure (hypertension) in the arteries of the lungs (pulmonary artery) for no apparent reason,” according to the National Organization for Rare Disorders (NORD). In the United States, it is labeled as an orphan disease, which means it affects fewer than 200,000 people.

The fact that PAH is uncommon and does not get much attention alarms medical specialists. If not properly diagnosed and addressed with the necessary therapy, PAH can lead to early death.

That’s why the Philippine College of Chest Physicians (PCCP) has partnered with multinational healthcare company Johnson & Johnson embarked on an awareness campaign last November 2020. The organization wants to improve diagnostic rates and increase the number of patients treated for pulmonary arterial hypertension.

PCCP president Dr. Ivan Villespin and J&J Philippines head of medical affairs Dr. Erwin Benedicto led an online media roundtable discussion on the importance of early detection and proper diagnosis of PAH.

Difference between pulmonary hypertension and pulmonary arterial hypertension 

The doctors clarified that PAH is a more specific type of Pulmonary Hypertension (PH). It occurs when the walls of the arteries leading from the right side of the heart to the lungs narrow and tighten. Consequently, pressure in the lungs rises and causes symptoms like fatigue and shortness of breath.  

The two doctors also differentiated the symptoms between pulmonary arterial hypertension and the more common systemic hypertension, or alta presyon in Filipino.

Dr. Villespin pointed out that PAH and systemic hypertension are not interchangeable. He explained, “Merong as high as 200/100 sa systemic blood pressure, pero hindi sa pulmonary. Meron namang mataas sa pulmonary, pero wala namang high blood.”

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Pulmonary arterial hypertension symptoms

Dr. Villespin noted that his PAH patients have these complaints: “Konting lakad o binubuksan lang ang bibig, nahahapo na. Madaling mapagod. May chest pains, nawawalan ng malay, nahihilo, ubo nang ubo.”

But he and his fellow doctor-panelist agree that such symptoms can also be because of a host of other ailments, particularly the more common tuberculosis and chronic obstructive pulmonary disease (COPD). Those two are among the leading causes of death in the Philippines.

Systemic hypertension can be detected through an instrument called sphygmomanometer, which measures the blood pressure and see if the patient is below or beyond the usual normal reading of 120/80.

PAH, however, can only be diagnosed through a test called right heart catheterization or pulmonary artery catheterization. Cleveland Clinic explains this test is used to see how well the heart is pumping, like how much it pumps per minute. Another function is to measure the blood pressure in the heart as well as the main vessels in the lungs.

Pulmonary arterial hypertension diagnosis

Dr. Benedicto’s advice is to immediately see a doctor when find it hard to breath. He said, “In every dyspnea or breathlessness, they suspect PAH may be one of them. Kung ma-eliminate ’yan, then good. Referral system is what we advocate. Those patients who wish to be treated properly, they need specialists.”

In the Philippines, there are no exact figures available that determine the current prevalence of PAH. The only known data is from the Philippine Heart Center’s 2009-2012 single-center study. It showed that 82 of 104 Filipino patients were found to have PAH within that time period, and they were mostly from the 20 to 40 age group.

Dr. Villespin added that based on his experience as a pulmonologist and his research on the subject, more women in their reproductive ages are affected — these include pregnant women. He cautioned, though, that “pregnancy itself is not a risk factor for pulmonary arterial hypertension.” 

Women can be more susceptible to PAH if:

  • She’s had repeated abortion.
  • She’s taking illicit drugs
  • She’s suffering from any autoimmune disorder

Dr. Villespin said PAH can also happen to newborns and older kids. The little ones will have to undergo treatment that’s “a bit similar” to what their adult counterparts take, but still under pediatric care.

Currently, there’s no cure for pulmonary arterial hypertension, only modern advanced therapies that can markedly improve a patient’s symptoms and slow the rate of clinical deterioration. But “with advancement of technology,” Dr. Villespin pointed out, “there’s optimism.”

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