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PH Can Impose Physical Restrictions, But Don't Limit Yourself

It's a beautiful day in the country for what appears to be the beginning of autumn. There are acres of trees, mostly green except for a few that have changed to gold. They're casting a shadow over a lake, but on the other side sun is glistening on the water and the land is covered in lush, green grass. It appears to be a wide-open field except for the ruins of an old fence. All that remains is a small gate held up by two standing pillars. Peering longingly through the wrought-iron bars are three clueless sheep.

This picture (photographer unknown) of sheep confined by an imaginary fence has been making the rounds on social media. People are sharing it with their personal view of its symbolism.

When I look at the picture I think of the pulmonary hypertension (PH) community and how physical restrictions caused by the disease can lead to life-altering mental fatigue in both patients and caregivers. We can become so focused on what we can't do that our minds neglect to seek ways of living past the gates that PH puts up.

Take that first step

Prior to his heart and double-lung transplant 10 years ago, my son Cullen, 25, lived within the confines of PH. When he was diagnosed in 2008, it seemed the more his dad and I learned about this rare, life-threatening condition, the less we could envision Cullen living any sort of a normal life outside the safety of our home.

Thanks to an excellent PH specialist, compassionate staff, and the experiences shared by other PH families, we became aware that it was as important for Cullen to explore what he could do as it was for him to stop when faced with something he couldn't.

Cullen was placed on triple therapy, and one of those PH treatments was continuous intravenous Flolan (epoprostenol GM). It required a central venous catheter placed into a large vein leading to his heart, which would emerge from a small opening in Cullen's chest.

He spent weeks in the hospital as his body adjusted to this metaphorical new appendage while his parents learned how to care for it and prepare the medicine going into it. When he was released to return home, that first step back into the real world was intimidating.

We were facing the beginning of the school year and were frozen with worry about allowing Cullen to attend. But we pressed on by meeting with his principal and teachers to educate them about PH, develop a plan of how to keep him safe at school, and explain that some days he might not be well enough to attend. Our efforts were successful and, years later, he graduated from the eighth grade with his class.

Due to his Flolan treatment and the pump needed to administer it, bathing was a challenge and swimming was a no-go — until we gifted Cullen with a dry suit. That was yet another gate opened back up for him.

Some days Cullen found himself on the shady side of life and on others, the sunny, but no matter which side he was on, we did our best to keep him free to wander and experience what he could.

My advice to the newly diagnosed and their caregivers is to seek friendship and understanding from the PH community, but don't limit yourself to them. We spread PH awareness so that those affected by the disease can go out into the world and live in it to the best of their ability. We are always here for you, but we will never hold you back from enjoying a life of quality. I invite you to join the PH News Forums where we share our experiences, both PH-related and not.

The fourth sheep The sun sets behind wispy clouds that are sitting close to the horizon. The silhouettes of trees reach up toward the sky.

The sun sets on New Year's Eve 2024. (Photo by Colleen Steele)

In the picture of the restrained sheep, there is a fourth one, standing next to the gate, vision unobstructed by the bars but still frozen in place. As a caregiver, I can relate to that sheep. I'm not physically limited in moving forward, but I often feel unable to because of my caregiving responsibilities.

On New Year's Eve, the setting sun reminded me of how unnecessarily tethered I have become to helping others. I was out running errands and allowed myself quick glimpses at the sky. Its beauty encouraged me to pull over into a nearby park and enjoy watching the sun go down.

Taking that moment to step away from my responsibilities and enjoy a few moments of peace was so refreshing that I've made it a part of my daily routine in 2025.

Sometimes all it takes is a setting sun to herd us in a more inviting direction.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary hypertension.

CTEPH Predictors Following Pulmonary Embolism

Risk for chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism (PE) is associated with gender, delayed PE diagnosis, hypoxemia, heart load, D-dimer levels, and PE etiology. These findings were published in the Journal of the American Heart Association.

One of the most serious post-PE syndromes is CTEPH. A large variation in the rate of CTEPH has been reported and risk factors are not well understood.

The Contemporary Management and Outcomes in Patients With Venous Thromboembolism (COMMAND VTE) Registry-2 is a large cohort that recruited patients in Japan. In this study, patients (N=5197) with acute symptomatic venous thromboembolism between 2015 and 2020 at 31 sites were evaluated for CTEPH after acute PE (n=2787). The primary outcomes were the incidence of and risk factors for CTEPH.

Following a diagnosis of acute PE, the cumulative rates of CTEPH increased from 1.0% at 180 days to 1.7% at 1 year, 2.0% at 2 years, 2.3% at 3 years, and 2.4% at 4 and 5 years.

Several independent risk factors for CTEPH were identified, which could be useful for screening a high-risk population for CTEPH after acute PE.

The patients with (n=48) and without (n=2739) CTEPH comprised 77.1% and 56.3% women (P =.004), their mean ages were 65.2±15.0 and 66.6±15.4 years, and they had a BMI of 23.4±4.4 and 23.8±4.7, respectively.

At PE, patients who went on to develop CTEPH were more likely to have unprovoked PE (79.2% vs 40.2%; P <.001), to present with hypoxemia (70.8% vs 44.0%; P <.001), they had higher right heart load (91.7% vs 40.5%; P <.001), and they had lower D-dimer levels (median, 6.0 vs 11.4 mg/mL; P <.001) compared with patients who did not develop CTEPH.

Most patients with CTEPH (97.9%) were diagnosed within 3 years of PE. A total of 7 patients with CTEPH died, due to malignant diseases (n=4), infection (n=1), chronic obstructive pulmonary disease (n=1), and unknown reasons (n=1). The survival rates after CTEPH were 95.7%, 93.5%, 88.4%, 85.0%, 85.0%, and 72.9% at years 1 through 6, respectively.

Risk for CTEPH was associated with right heart load (adjusted hazard ratio [aHR], 9.28; 95% CI, 3.19-27.00; P <.001), unprovoked PE (aHR, 2.77; 95% CI, 1.22-6.30; P =.02), hypoxemia (aHR, 2.52; 95% CI, 1.26-5.04; P =.009), female gender (aHR, 2.09; 1.05-4.14; P =.04), per day delay in diagnosis from symptom onset (aHR, 1.04; 95% CI, 1.01-1.07; P =.01), and D-dimer levels per 1 mg/mL (aHR, 0.96; 95% CI, 0.92-0.99; P =.02).

This study was limited by the lack of diversity in the study cohort, which may limit the generalizability of these findings.

The study authors concluded, "In this large real-world VTE registry in the DOAC [direct oral anticoagulation] era, the cumulative detection of CTEPH after acute PE was 2.3% at 3 years. Several independent risk factors for CTEPH were identified, which could be useful for screening a high-risk population for CTEPH after acute PE."

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors' disclosures.

This article originally appeared on The Cardiology Advisor

How I Struggle To Manage Chronic Pain During The Holidays

Are you managing chronic pain alongside pulmonary hypertension (PH)? Did it disrupt your holiday plans?

If you answered yes, you're not alone. Many folks in the PH and broader rare disease communities share similar frustrations with having to adjust plans because of unrelenting pain. Dealing with chronic pain is an invisible battle that affects not only the body but also the mind. Pain shows up uninvited, especially during moments I look forward to, such as when I'm spending time with loved ones. And pain doesn't like to share control.

Over the holidays, I had many plans — things I was excited to do and tasks I wanted to tackle — but most of them didn't happen. If I had to sum up my Christmas and New Year's break in one word, it would be "sleep." Wrapping myself in my heated blanket, taking medication, and searching for relief became my routine.

Navigating this season while managing chronic pain and the side effects of my many medications is grueling. The heaviness and throbbing in my legs and back can be attributed to osteoarthritis, and when my chronic kidney disease progresses, my back pain intensifies. I've been told I have a high pain tolerance, which sometimes allows me to avoid taking medication for it. But when the pain becomes unbearable, I have to give in.

Lately, I've found myself needing more medication and rest than usual, which leaves me feeling defeated. I understand this reality, but I struggle to explain it to those closest to me.

Navigating life with daily pain

After dealing with chronic pain for so long, I've learned to accept it as part of my daily life, although that hasn't been easy. Talking about it is even more difficult. There's a stigma associated with managing chronic pain, which often leaves me pushing through it in silence. And that's exhausting.

If I have plans or other things to do, I'll often delay taking my medication until the pain is overwhelming. I don't want to seem weak or like a burden to others, so I push through. But inevitably, the pain catches up, leaving me too drained to enjoy time with loved ones.

Friends and family often overlook the emotional toll of this constant battle. The physical pain is accompanied by a mental and emotional burden that I carry every day. This ongoing battle has caused me to feel isolated and disconnected from everyday life. It's difficult for people who don't experience chronic pain to understand its impact. Still, I keep going, using multifaceted approaches to find relief. Even on my most challenging days, hope and faith remain.

Yet I wonder: Do my loved ones notice the changes I experience with PH and my increased pain? Do they see my physical abilities fading while my PH progresses? How could they, when I often appear fine on the outside?

But that's not the whole story. Some days, the weight of it all feels overwhelming, and I want to give in. But then I remember what I've already overcome since my PH diagnosis almost 20 years ago, and that fuels me to keep going. I hope others will acknowledge and admire this resilience of mine.

I've distanced myself from many people. The one person who sees it all, especially on unbearable days, is my husband and caregiver, Manny. He sees my pain even when I wish he didn't have to. I know he struggles with it, too, and I try to support him on this journey as well. What I need most is understanding and support, not just from Manny but from everyone around me.

Pain can rearrange priorities, and I'm still learning to navigate this reality. Too bad pain and PH don't take a break during the holiday season, too.

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