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PAH: Changing Approaches To Diagnosis, Treatment, And The Patient Journey
In recent decades, there's been a sea change in the understanding of pulmonary arterial hypertension (PAH), which in turn has affected the patient journey in this disease. The prognosis of PAH has evolved from impending death with few treatment options to a chronic condition notable for improved survival.1
Improved patient survival has been realized due to the development of effective therapies and the expansion of risk-stratification scores, which aid clinicians in clinical decision-making. Although PAH can now be better controlled — with improved quality of life and clinical course for patients — patients still face barriers to care. The treatment burden is cumbersome and hinders quality of life, and many with the condition initially present with advanced or end-stage disease.1
Changes and Challenges in Disease DiagnosisA focus on early disease detection and new clinical outcomes findings have changed the definitions of PH and PAH.2-4 According the 2022 ESC/ERS Guidelines for Pulmonary Hypertension, patients with a mean pulmonary artery pressure of more than 20 mmHg measured by right heart catheterization are now considered to have PH. A pulmonary vascular resistance or more than 2.0 Wood units and pulmonary arterial wedge pressure of at least 15 mm Hg further implies the existence of PAH.3,4
"These lowered thresholds aim to identify patients early in the disease course, which is important because delay to diagnosis of PH is common and linked to elevated morbidity and shortened lifespan," according to the author of a review published in the Journal of the American Heart Association.2
"
Once patients understand the disease itself and the medications/therapies, the next step really would be to help them understand risk assessment and goals of low-risk status.
Although the 2022 ESC/ERS Guidelines for Pulmonary Hypertension cover the whole spectrum of PH, these updated guidelines include "an emphasis on diagnosing and treating pulmonary arterial hypertension."4
Within the 2022 guidelines, the principal diagnostic algorithm for PH has been streamlined to a 3-step approach3 that includes:
In an exclusive interview, Jean Elwing, MD, FCCP, Chair of the Pulmonary Vascular and Cardiovascular Network with the American College of Chest Physicians, provided guidance on how to best recognize PH in patients, and to then identify PAH.
"Pulmonary hypertension can present with multiple nonspecific symptoms and can be very challenging to diagnose," she said. "When patients have progressive dyspnea, exercise limitation, and exertional chest discomfort, we have to think about potential causes that are both pulmonary and cardiac. When we are thinking of cardiac causes, one of the best tools to use is an echocardiogram. The echocardiogram will give us insight into the likelihood of pulmonary hypertension."
She advised that PH should be a differential diagnosis in anyone with unexplained dyspnea/exercise limitation or other nonspecific symptoms such as intermittent chest discomfort with exertion, dizziness, lightheadedness, fatigue, and swelling. The index of suspicion should be high in patients with a higher risk of developing the disease, and their clinical trajectory should be followed closely.
For instance, if a patient with HIV, liver disease with portal hypertension, or connective tissue disease develops shortness of breath, an echocardiogram should be performed followed by right-heart catheterization, if needed, to rule out PH.
Socioeconomic Factors Affecting PAHDr. Elwing also remarked on the impact of socioeconomic factors in the recognition of PH. "Social challenges and socioeconomic status can play a significant negative role in pulmonary hypertension diagnosis and lead to delays in care. This is an area where we need to be very mindful and consider all patients equally when assessing symptoms and pursuing further work-up of pulmonary hypertension," she stated.
For instance, women and men present with PH and PAH in a similar fashion, but due to a lower prevalence of the disease in men, symptoms could be overlooked.
As for race and ethnicity, she said, "We believe that different races/ethnicities present with similar symptoms of pulmonary hypertension but the etiologies of pulmonary hypertension in these populations may differ. Hispanic patients are more at risk for congenital heart disease-associated pulmonary arterial hypertension and have an increased risk of viral hepatitis increasing their risk of portal hypertension and portopulmonary hypertension. Connective tissue disease-associated pulmonary hypertension has been shown to be more common in Black patients in available registry data."
The Evolution of PAH TreatmentOver the past 3 decades, much has been clarified regarding pulmonary hypertension treatment; 30 years ago, the only treatment available involved high dosages of calcium-channel antagonists, which improved survival only in a handful of those with pulmonary arterial hypertension.5
Approved medical treatments for PAH (along with their routes of administration) include the following2:
In the aggregate, these agents help improve outcomes such as exercise tolerance and capacity, as well as mitigating clinical deterioration.2
In addition to pharmacologic therapies, there are also nonpharmacologic interventions that may improve patient well-being. These revolve around diet, sleep, and exercise, said Dr. Elwing.
"A balanced diet with monitoring of salt and fluid intake can have a major impact on right heart-failure management and other comorbidities. Exercise can improve dyspnea and tolerance of many health conditions. This is important for exercise tolerance as well as overall well-being," she said.
"Sleep is imperative for mood and resilience. Good sleep hygiene and assessment of sleep disorders is critical for patients to feel well and have the reserve to care for themselves with this complex medical condition," she added.
The Patient Journey in PAHHelping patients with PAH through the rigors of diagnosis and treatment requires empathetic conversations by specialists. Unfortunately, due to time constraints, it is hard for clinicians to make patients comfortable enough to open up about their goals and to revisit these goals over time. Instead, expedient visits pinpoint clinical factors, therapy, and medication adherence/tolerability.1
Outside of clinicians, there are a few options patients and their caregivers may be encouraged pursue for further information and support, including Living with PAH, an online support and discussion community, and the American Lung Association Patient & Caregiver Network.
Dr. Elwing recommends that specialists take time to partner with patients to improve care.
"Knowledge is power, especially in complex diseases like pulmonary hypertension. Taking the time to educate patients will pay off for the patient and the provider in multiple ways," she stressed. "Successful treatment and best outcomes can be achieved with joint efforts of the care provider as well as the patient. In order to achieve this, patients need to be educated on the disease and made aware of the importance of medication adherence, close follow-up, testing, and potential for medication changes."
She added, "Once patients understand the disease itself and the medications/therapies, the next step really would be to help them understand risk assessment and goals of low-risk status. Calculating a risk score in PAH is not just a number. Using a risk assessment tool helps guide medical therapy and provides some insight in terms of prognosis for that individual."
On a final note, Dr. Elwing exhorted providers to take time to consider the patient in a holistic sense.
"We need to understand their level of medical literacy, ability to communicate, social support, and the severity of the illness. We assess patients' risk status and design a treatment approach based on that. We choose the type of medications and the delivery system based on what the patient is able to tolerate and use safely," Dr Elwing noted.
"We should closely monitor every PAH patient and provide opportunities to use our most aggressive therapies but may have to tailor medications based on that individual patient's needs for the patient's safety and ability for them to use medications consistently," she added.
This article originally appeared on Pulmonology Advisor
My GP Told Me I Just Had A Chest Infection – To This Day I Have No Idea Why I Nearly Died
WHEN healthy Rachael Stewart started suffering from discomfort when breathing, she thought it was an innocent chest infection - and her GP backed this up.
But when her chest pain got worse ten days later in February 2019 - after five days of antibiotics - her doctor sent her to A&E.
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Rachael Stewart, 28, from Glasgow, started suffering from discomfort when breathingCredit: Make The Headlines5
Her GP told her it was just a chest infection, but her condition began to worsenedCredit: Make The Headlines5
In A&E, the now beauty queen, was told she had a blood clot in her lung, which was collapsingCredit: Make The HeadlinesWithin 24 hours, Rachael, 28, from Glasgow, who had previously run a half marathon, was told she had a blood clot and her lung had started to collapse and she needed urgent treatment.
Rachael's world was turned "upside down" - she spent the next few days in hospital and the next two years building up her strength again.
Now, the commercial account handler competes in pageants and raises awareness of blood clots as she accepts she'll be a hospital patient for the rest of her life.
Rachael said: "The last healthy breath I took was some time in January 2019, and I never even realised it.
"There were points where I thought, am I going to die? It was shocking to go from healthy, to being told life might never be the same.
"To this day, doctors have no idea what caused this to happen to me.
"My right lung is still partially collapsed and is at 60 per cent capacity - doctors are confident it will never improve, but I accept my health and have adapted my life and routine to suit my needs.
"I've realised that by sharing my story I can help others to know what life looks like on the other side - to give them hope."
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Shocking five-figure calorie amount Brits consume on holidays revealed OUT OF THE BLUERachael had always prided herself on being fit and healthy.
She had previously gone to the gym regularly, ran a half marathon in 2016, and walked 10K multiple times per week.
So when she first felt unwell and had pains in her chest in February 2019, she visited her GP who prescribed a five-day course of antibiotics for a chest infection.
When her symptoms worsened, her doctor referred her to A&E at Monklands Hospital, where an X-ray revealed Rachael's lung was collapsing and she would need to be kept in.
It also showed a pulmonary embolism - a sudden blockage in your pulmonary arteries, the blood vessels that send blood to your lungs - had appeared.
Rachael said: "I had gone from having my mind put at ease by my GP, to within hours of arriving at the hospital, finding out I had started losing blood supply to my lung because there was a sizable blood clot in my body.
"I just sat there in silence as they explained everything to me.
"At 23 I was told I'm losing blood supply to an organ that's pretty much required for everything.
"They also found that I had an inflamed lymph node and an unknown growth.
"Obviously being told that your lung has collapsed and you're losing blood supply, I knew it wasn't good.
"I messaged my dad and said I was in hospital and it was more serious than I thought."
Rachael questioned if she was going to lose her lung, but she wasn't given a definitive answer.
Her doctors were confident they'd caught the blood clot in time, so she went straight onto blood thinning injections and was prescribed a course of blood thinners for six months to take daily.
She stayed in hospital for two days before returning home to continue her recovery.
LIFE NEVER THE SAME AGAINBut life was very different when she returned home, as things she used to do easily, like go to the bathroom or walk downstairs, were now difficult.
She felt tired, breathless and the pains in her chest remained for months.
She stopped attending the gym and running and instead was limited to short walks to build her stamina back up.
Rachael's recovery took a year as she kept catching chest infections and she required two weekly check ups at respiratory, GP checkups and regular CT scans.
She had to sign a disclosure to say she understood her risk of cancer was increased due to the amount of scans she needed.
Rachael said: "My life had turned upside down. I'd never known anyone to have a blood clot, so I was unaware of everything.
"I used to be someone who did lots of exercise but my life very quickly did a 360.
"It was insane. I would try and walk 100 metres after the first couple of weeks and I would struggle.
"I was literally just walking at a snail's pace and even then I was having to come back because my lungs felt tight, I was breathless or just tired.
"There was a lot of pain in my chest for a while. I was constantly out of breath even just from getting up and walking to the bathroom. I was sleeping about 14 to 16 hours a day.
"I didn't know if my lung would recover or if this would kill me."
Rachael still went to work, but her social life diminished.
Single at the time, she felt as though she couldn't be honest with dates, and wasn't able to drink alcohol due to her medication.
She said: "I would sometimes tell people I was dating that I was on blood thinners and they'd almost run away like it was a red flag. I had to explain that it's not contagious.
"But it seemed like I had baggage, so I ended up never telling people and kept it to myself.
"Otherwise people looked at me in a different way and felt sorry for me. They acted like I was a broken, destroyed person.
"Yes, my life has changed but I'm alive and can deal with it!"
Rachael started chatting to a woman through her Instagram page who was looking for others with pulmonary embolism and Rachael realised she could help and show others what life looks like on the other side.
LIVING HER DREAMShe'd always wanted to do beauty pageants and grew up watching Miss Congeniality.
Her blood clot scare made her realise life could be taken away in a second, so she decided to sign up to her first pageant in August 2022 and came fourth.
Rachael said: "2020 was the year I decided to take the time to learn to be OK with myself and get over what happened.
"I wanted to show people suffering that it can be fine, you can be normal, and show young people like me that life will be OK.
"Then I started thinking about pageants - something I've always wanted to do. I now know that life can be taken away at a moment's notice.
"If I hadn't gone to the doctors when I thought I had a chest infection, I probably wouldn't be here.
"So I thought, I've always loved Miss Congeniality, I'm going to give it a go and pretend I know what I'm doing!
"I went into the Miss United Kingdom finals as Ms North Lanarkshire 2022 then came fourth in the Royal International Miss United Kingdom finals.
"I love every single moment of pageant life and will be signing up to more and continuing to raise awareness."
Now in 2023, Rachael is grateful for the position she's in.
She's in a happy relationship and loves spending her weekends exploring the great outdoors on lengthy walks.
She also works with a personal trainer to continue working on her fitness journey and is learning to love herself.
But life is still somewhat different as she'll likely never have full function in her right lung.
She said: "Yes, I have less lung function, have had an unknown number of CT scans and MRIs, multiple failed biopsies, and a few hospital stays, but I'm the healthiest I've ever been.
"Mentally and physically, I'm proud of myself for going through all of it and not giving up.
"I'm living proof that it does get better eventually."
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After spending a couple of days in hospital for treatment, it took Rachael two years to build up her health and fitness againCredit: Make The Headlines5
Her health will never be the same again, but the experience made her prioritise her dream of becoming a beauty queenCredit: Make The HeadlinesPatients With COPD, Severe Pain At An Increased Risk Of Falling
Patients with both chronic obstructive pulmonary disease (COPD) and severe pain are at an almost 40% predicted probability of falling, indicating the potential interaction between the conditions.
Patients with chronic obstructive pulmonary disease (COPD) and severe pain are at an increased predicted probability of falling, according to a study published in the International Journal of Environmental Research and Public Health.
The researchers explained that frailty is common in patients with COPD, as 23% have been classified as frail and 56% as prefrail. They emphasized that falling is a prominent feature of frailty, resulting in patients with COPD being 4 times more likely to fall than healthy patients. Pain is also common among patients with COPD, as they have a 66% prevalence of pain.
Because of this, the researchers created a study to predict the probability of falling among older adults with COPD and their healthy peers, investigating the impact of pain severity on fall incidents and the association between pain and fall severity. The researchers used data from the English Longitudinal Study of Ageing (ELSA) to create their study population, considering their sex, age, wealth, and education level; ELSA investigates aspects of aging and older people in England, collecting data on their physical and mental health, as well as social and financial circumstances, at 2-year intervals.
The study population consisted of those a part of the ELSA cohort who were at least 58 years old at baseline assessment. A total of 18,572 people matching these criteria were initially included, but this number decreased after the researchers removed those missing necessary data points. As a result, the study population consisted of 806 patients with COPD and 3898 healthy patients.
The researchers noted that both groups were similar in age, sex, and body mass index, but they differed in pain level, wealth, education level, and fall occurrences. They explained that 56.1% of the patients with COPD experienced pain, while only 22.4% of the healthy patients did. Also, patients with COPD had lower levels of both education (median of no qualifications in the COPD group vs median of intermediate in the healthy controls) and wealth status (mean of £28,478 in theCOPD group vs £65,097 in thehealthycontrols). Lastly, 33% of those with COPD reported falls compared with 19.5% of healthy patients.
By calculating odds ratios (ORs), researchers discovered a relationship between incident falls and pain severity categories within both the COPD group and the control group. They explained that those with any severity of pain in both groups had a greater risk of falling, increasing with pain severity (mild pain: OR, 1.47 for COPD and 1.40 for healthy controls; moderate pain: OR, 1.68 for COPD and 1.61 for healthy controls; severe pain: OR, 2.39 for COPD and 1.84 for healthy controls).
Additionally, the researchers found that the predicted probabilities of falling for patients with COPD were greater across all pain categories than for healthy controls. The predicted probability for patients with COPD and no pain was 20% (95% CI, 17%-25%), with mild pain was 28% (95% CI, 18%-38%), with moderate pain was 28% (95% CI, 22%-34%), and with severe pain was 39% (95% CI, 30%-47%). In comparison, the predicted probability of falling for healthy controls with no pain was 17% (95% CI, 16%-18%), with mild pain was 22% (95% CI, 18%-27%), with moderate pain was 25% (95% CI, 20%-29%), and with severe pain was 27% (95% CI, 20%-35%).
Overall, the researchers highlighted that those with COPD and severe pain had an almost 40% predicted probability of falling, indicating the potential interaction between COPD and severe pain. Also, the predicted probability of falling for those with COPD and pain was greater than those for both people with COPD and no pain and healthy controls with pain. For both groups, the predicted probability of falling increased as the severity of pain increased, but this was more prevalent for patients with COPD across all pain severities.
The researchers noted various limitations to their study, one being that the researchers could not identify all comorbidities beyond the common ones identified by the main ELSA set. Because of this, some patients within the healthy group may have comorbidities that affected their risk of falls but were not included within the data set.
The researchers noted that future research will aim at understanding potential shared factors between COPD and pain that increase the risk of falling, including multisite pain, pain interference, and psychological impacts.
"Further prospective research is required to determine if the pain is associated with falls in people with COPD, including the identification of those with pain for intervention to prevent the deleterious effects of falling," the authors concluded.
Reference
Loughran KJ, Tough D, Ryan CG, et al. The association of pain with incident falls in people with chronic obstructive pulmonary disease: evidence from the English Longitudinal Study of Ageing. Int J Environ Res Public Health. 2023;20(13):6236. Doi:10.3390/ijerph20136236
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