2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines | Circulation

New Drug, New Combinations Improve Outlook In PAH, Pulmonary Arterial Hypertension

AMBLER, Pa. (WPVI) -- High blood pressure doesn't just mean the kind measured by an arm cuff.

There's another form in the lungs that's also very dangerous.

A longtime athlete and physical education teacher explains how it impacts his life and the new medication helping him get back to his old self.

"You were in really good shape, and now all of a sudden, you're not," John Smith of Ambler, Pa., notes.

After a lifetime of sports and teaching physical education, John was blindsided 16 years ago to learn he had Pulmonary Arterial Hypertension, or PAH.

It appeared suddenly after a heart defect was corrected.

"Both sides of my heart were enlarged. After they fixed the bridge, only one side went down," he remembers.

Dr. Paul Forfia, a Temple Health heart failure specialist, says in PAH, very small blood vessels in the lungs become narrowed.

"That puts a lot of resistance back onto the right side of the heart, and a lot of resistance for the passage of blood flow from the right heart through the lungs," Dr. Forfia says.

Normal lung blood pressures balloon over time.

"It could be 10 or 15 times higher than it should be, that will cause the right side of the heart to become dysfunctional," he says.

Dr. Forfia says PAH is three to four times more common in women, usually between the ages of 30 and 60, and more common in African American and Hispanic women.

Hallmark signs include increasing shortness of breath even with light activities; fatigue; edema, or swelling; and dizziness and fainting spells.

It gets worse over time, so aggressive treatment is a must.

"Seek out a Center of Comprehensive Care where there's a dedicated program," Dr. Forfia says.

Temple Health has that certification.

A combination of drugs has been used to control PAH.

But a new drug, Winrevair, could change the landscape.

"It's the first one that can actually fix the heart," John says, adding, "I actually went and ran, which is amazing. I haven't run in 10 years."

"The outlook for patients with PAH is very, very different and much more favorable now than it was two decades ago, or even one decade ago," Dr. Forfia says.

"I have twp young kids, and I want to be around for them. That's my goal," John says.

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Understanding Altitude Hypertension And Its Effects

Not everyone will develop hypertension at high altitudes. Fitness level, genetics, rate of ascent, and hydration/nutrition can all matter.

You may be more likely to experience high altitude hypertension if you don't take time to acclimate, if you're dehydrated, and if you have pre-existing conditions. Even in the best physical health, the higher you go in elevation, the more likely you are to experience elevated blood pressure.

Screening Tool Helps Predict Death & Hospitalization In PAH

Photo Credit: Mr. Suphachai Praserdumrongchai

The Mini Nutritional Assessment was viable for assessing nutritional status and predicting mortality outcomes in patients with pulmonary arterial hypertension.

The underlying cause of pulmonary arterial hypertension (PAH) is often difficult to discern in patients. Symptoms such as shortness of breath, fatigue, chest pain, and irregular heartbeat require close monitoring, and not adhering to treatment can hinder care. Given that PAH is a progressive disease, practitioners need prognostic markers that can help them strategize optimal treatment protocols.

Studies have shown that identifying malnutrition in patients with chronic heart failure can predict poor outcomes. Hypothesizing that this correlation may also apply to patients with PAH, Maria Wieteska-Milek, MD, PhD, and colleagues developed a prospective multicenter study and published their findings in European Heart Journal.

Nutritional Assessment

The researchers used the Mini Nutritional Assessment (MNA) to gauge malnutrition in patients with PAH. The MNA was developed nearly two decades ago and has been primarily applied to older adult populations. Numerous studies have confirmed its efficacy.

The MNA has two versions: a long-form 18-question survey and a short-form 6-question survey.  Although both are effective, Dr. Wieteska-Milek and colleagues used the long form. For their statistical analysis of this study, they considered patients with an MNA score of 17 points or less to have malnutrition and patients with an MNA score of 18 to 23.5 points to be at risk for malnutrition. Both score ranges are considered abnormal.

A total of 182 participants completed the MNA while receiving treatment in an outpatient clinic during scheduled hospitalizations. The mean age was 60 ±17 years, and 73% were females. The primary endpoint of the study was all-cause mortality. The secondary endpoint was a composite of all-cause death or PAH-related hospitalizations. The cause of PAH was unknown for 57% of the participants.

Abnormal Scores

According to the MNA scale, 37% of the participants received a score identifying them as having malnutrition or being at risk for malnutrition. The median follow-up was 23 months (range, 0-32 months).

Of the participants, 18.7% died, and 29.1% underwent hospitalization because of PAH. Some 31.3% of the participants experienced the composite endpoint.

The researchers noted that when Kaplan-Meier survival curves were applied to the data, there were significant differences in overall survival rates between patients with abnormal MNA scores and those with normal MNA scores (P<0.001). There were also significant differences in combined mortality risk and PAH-related hospital admittance (P=0.02). Patients with abnormal MNA scores had higher mortality and higher combined mortality/hospitalization.

To further validate the study findings, a multivariate Cox regression analysis that included age, sex, and main risk factors confirmed that MNA scores in the abnormal range continued to show a significant association with all-cause mortality (HR, 2.86; 95% CI, 1.29–6.38; P=0.01) and the combined composite end-point of mortality risk and PAH-related hospital admittance (HR, 2.39; 95% CI, 1.05–5.42; P=0.04).

The main risk factors that were included in this analysis were the COMPERA 2.0 PAH risk calculator score, ratio of tricuspid annular plane systolic excursion to echocardiographically measured systolic pulmonary artery pressure, right atrial pressure, cardiac index, and mix oxygen saturation.

The researchers concluded that MNA was a viable tool for assessing nutritional status that could be successfully applied as an outcome predictor in patients with PAH.

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