pulmonary hypertension causes

Particular Histological Pattern Linked to Severe Pulmonary Hypertension in COPD - Pulmonology Advisor
Widening the Definition and Use of Palliative Care in PAH - Pulmonology Advisor
Transition from PDE5i to Adempas Without Washout is Viable for PH - Pulmonary Hypertension News
Patient credits Greeley pulmonary hypertension program with saving his life - Greeley Tribune

Particular Histological Pattern Linked to Severe Pulmonary Hypertension in COPD - Pulmonology Advisor

Posted: 29 Mar 2019 01:00 AM PDT

Compared with individuals with chronic obstructive pulmonary disease (COPD) with moderate pulmonary hypertension (PH) or without PH, those with acute PH-COPD have lower capillary density and significantly greater pulmonary microvessel muscularization, according to a study recently published in CHEST.

This study included 30 participants with COPD who underwent lung transplant, 10 of whom had acute PH-COPD, 10 of whom had moderate PH-COPD (mean pulmonary pressure, 25-34 mm Hg without low cardiac index), and 10 with no PH (mean pulmonary pressure <25 mm Hg). The 3 groups were compared retrospectively in terms of muscular pulmonary arterial wall thickness, microvascular muscularization, and pulmonary capillary density. Dunn's post-test, chi-squared test, and the Kruskal-Wallis test were used to perform univariate analyses, while a Spearman test was used to estimate correlation coefficients.

Compared with individuals with moderate pulmonary hypertension, the acute PH-COPD group had significantly greater microvessel remodeling scores (1.284 vs 0.867; P =.0045) and lower capillary density (0.00235 per μm² vs 0.00526 per μm²; P =.0049). Medium-size arterial alterations were less discriminating. Low levels of ERG staining in participants with acute PH appears to be associated with capillary network loss.

Limitations to this study included a retrospective design, a low number of participants with PH-COPD, a slightly older definition of acute PH-COPD, and the exclusive examination of lungs with end-stage lung disease.

The researchers of the study concluded that "the morphological correlate or hallmark of severe PH-COPD, when compared with moderate PH-COPD, lies in the substantially increased muscularization of pulmonary microvessels (arterioles and/or venules), as well as in the lower capillary density. Importantly, no significant differences were observed in the muscular-type pulmonary arteries, which are relevant in PAH (group 1) and no typical PAH lesions, such as plexiform lesions or onion-skin lesions (concentric laminar intimal fibrosis) were detected, stressing at least the different morphological phenotype of severe PH-COPD and [pulmonary arterial hypertension]."

Reference

Bunel V, Guyard A, Dauriat G, et al. Pulmonary arterial histological lesions in COPD patients with severe pulmonary hypertension [published online March 11, 2019]. CHEST. doi:10.1016/j.chest.2019.02.333

Topics:

Chronic Obstructive Pulmonary Disease Pulmonary Hypertension

Widening the Definition and Use of Palliative Care in PAH - Pulmonology Advisor

Posted: 28 Mar 2019 05:00 PM PDT

While the prognosis for patients with pulmonary arterial hypertension (PAH) has improved since the introduction of PAH-specific therapies, mortality and morbidity associated with the disease remain high. The 7-year survival rate for PAH is 50%, according to findings from a 2012 study of 2635 individuals, and many patients have numerous intolerable symptoms despite treatment.^1,2

In addition to physical limitations due to symptoms such as fatigue and shortness of breath, mental health disorders commonly affect patients with PAH. According to one study, there was a 35% prevalence of psychological disorders in patients with PAH, and other studies have shown elevated rates of depression, anxiety, and panic attacks in this population.^2,3 The caregivers of these patients are also vulnerable to stress, exhaustion, depression, and social impairment.

In many of individuals with PAH, this range of factors often contribute to a poor health-related quality of life (HRQoL), which is influenced by indicators such as functional status and physical, social, and emotional well-being. Various studies have observed that HRQoL in patients with PAH may be similar to that in patients with other chronic illnesses including cancer, spinal cord injury, and interstitial lung disease.

Although palliative care can improve HRQoL in PAH, such interventions are underused in this group because of misconceptions about its role. For example, a study published in 2014 found that many physicians view palliative care as an approach to be implemented in the context of end-of-life care.⁴ However, palliative care is "focused on providing relief from symptoms and stress caused by any chronic illness… [and] improving the HRQoL of the patient and family," according to a paper published in December 2018 in European Respiratory Review.² "It is appropriate at any age or stage of disease and importantly, it must be provided in concert with curative treatment." [Read about one cardiologist's shift in perspective on this topic.]

The most recent CHEST guidelines on PAH treatment contain a consensus statement supporting the integration of palliative care into PAH management, noting significant improvements in outcomes observed in other pulmonary diseases, including lung cancer.⁵ Although the expert panel did not identify any studies that examined the effects of palliative interventions combined with standard PAH care, this "lack of evidence… does not negate the potential benefits palliative care offers to all patients and families for assisting in management of disease burden, pain, and symptoms of chronic or acute needs," they stated. "The addition of palliative care interventions to assist in management of disease burden and symptoms can often be beneficial to improving patient quality of life."

The 2018 review described invasive palliative interventions including atrial septostomy, pulmonary artery denervation (PADN), and right ventricular assist devices, as well as noninvasive interventions including treatment for depression and anxiety, pain control, patient education and support groups, management of symptoms such as nausea and dyspnea, financial assistance, and pastoral counseling. Although research regarding palliative care in PAH is scant, studies have reported improvements in 6-minute walking distance, World Health Organization functional status, and mean pulmonary artery pressure in patients with PAH who underwent atrial septostomy or PADN.^6,7 Further research is needed to explore the effect of various invasive and noninvasive palliative approaches.

"It is important that physicians come to understand that palliative care is not the same as end-of-life care, and that palliative care is best when offered alongside life-prolonging therapies," the review authors concluded. "As there is a better understanding of what palliative care can offer, patients should know that a referral to palliative care is not 'giving up' but instead is an effort to thoroughly investigate all possible avenues for making quality of life the best it can be."

Pulmonology Advisor spoke with the following experts to learn more about palliative care in PAH: Lauren Goodman, MD, a pulmonary, critical care, and palliative medicine physician at the Ohio State University Wexner Medical Center in Columbus; and Roham Zamanian, MD, FCCP, associate professor of pulmonary and critical care medicine at the Stanford University Medical Center and director of the Stanford Adult Pulmonary Hypertension Program in California.

Pulmonology Advisor: What are some potential benefits of palliative care in patients with PAH and what are your thoughts on why it is underutilized in this population?

Dr Goodman:Although some PAH symptoms can be alleviated by treating the condition, others tend to persist even with maximal treatment. These symptoms often include at least dyspnea, fatigue, and anorexia. There can also be significant side effects and complications of PAH treatments, some of which require creativity and patience to manage.

Patients with PAH often think of their PAH physicians as their lifesavers. They may feel that if they mention ongoing symptoms, they will sound unappreciative, ungrateful, or perhaps even unworthy of the care and sometimes aggressive treatments they are receiving. Patients may also worry that continued symptoms or complaints would reduce their chances of being listed for or receiving a transplant, if transplant is an option.

While physicians both need and want to know about their patients' symptoms, time available to explore the severity and effect of symptoms can be limited. Palliative care physicians are often allotted more time for visits and fully expect to explore symptoms in depth with patients and with their families, if the patient desires.

Topics:

Pulmonary Arterial Hypertension Pulmonary Hypertension

Transition from PDE5i to Adempas Without Washout is Viable for PH - Pulmonary Hypertension News

Posted: 06 Mar 2019 12:00 AM PST

Transition from phosphodiesterase-5 inhibitors (PDE5i) — such as United Therapeutics' Adcirca (taladafil) and Pfizer's Revatio (sildenafil) — to Bayer's Adempas (riociguat), a soluble guanylate cyclase (sGC) stimulator, without a washout period is a viable option for some patients with pulmonary hypertension, a small study suggests.

The study, "Successful Transition from Phosphodiesterase-5 Inhibitors to Riociguat Without a Washout Period in Patients With Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: A Pilot Cohort Study," was published in the journal Heart, Lung and Circulation.

Pulmonary arterial hypertension (PAH) is a rare, life-threatening disorder caused by a narrowing of the arteries in the lungs, leading to high blood pressure. Over the last decade, therapies have substantially improved, with the generation of PAH-specific drugs with different mechanisms of action that can be combined to provide the best possible outcome for each patient.

PDE5i and sGCs are both vasodilator compounds — agents that induce blood vessels' widening and relaxation. They do so in different ways, but cannot be used as a therapeutic combination.

"Because sGC stimulator cannot be co-administered with a PDE5i because of the risk of systemic [low blood pressure], drug transition is considered in patients with an intolerance or inadequate clinical response to PDE5i," the researchers stated.

Patients who transition from one medication to another, immediately or with a short washout period, may develop hemodynamic instability — a medical condition in which the body's circulatory system fails to maintain a constant blood circulation because of lack of blood pressure.

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"A previous study [RESPITE trial, NCT02007629] suggested that selected patients with PAH may benefit from switching from PDE5i to sGC stimulator with a washout period. However, the feasibility of transitioning from a PDE5i to an sGC stimulator and the washout period required for transition have not been clearly established," the researchers said.

Therefore, a team of Japanese investigators set out to test whether transitioning from Adcirca and Revatio (PDE5i) to Adempas (an sGC stimulator) would be safe without a washout period in patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH).

The pilot cohort study enrolled six patients with PAH and one with CTEPH, who had already received dual- or triple-combination therapy. All patients were monitored for signs of hemodynamic instability by heart catheterization, a procedure in which a thin catheter is inserted into blood vessels and threaded into the heart to diagnose and treat heart problems.

Three patients switched from PDE5i treatment to Adempas because of side effects (severe headaches) associated with PDE5i treatment; four switched because of inadequate therapy response.

Results showed that all patients transitioned successfully without a washout period, with no signs of hemodynamic instability.

Immediately after the transition, pulmonary vascular resistance (PVR, a measure of heart strain induced by high blood pressure in the lungs) dropped (from 797 to 518 dyne/s/cm–5), as well as systemic blood pressure (a significant drop from 121 to 100 mmHg) in all patients.

The most common side effects associated with PDE5i disappeared after transition to Adempas.

"Transition from a PDE5i to [Adempas] without washout periods is safe. This transition may be a viable option for patients with PAH and side effects, such as headache, caused by PDE5i or who experience an inadequate response to combination therapy including PDE5i," the team concluded.

Patient credits Greeley pulmonary hypertension program with saving his life - Greeley Tribune

Posted: 16 Mar 2019 12:00 AM PDT

About two years ago, John Kamprath was planning for something very different from the spring break vacation he planned this year.

The now-58-year-old Estes Park resident had been diagnosed with high blood pressure of the lungs, something known as "pulmonary hypertension." The online literature Kamprath had read said pulmonary hypertension patients faced an average of two years before the complications become fatal.

With two girls to take care of, Kamprath sat down with his wife at the time to confront what seemed like a very real possibility: in two years, it would just be her and the girls.

"Are you guys going to be okay when I pass away?" Kamprath remembers thinking. "Where are we finances-wise? Let's make sure we don't drain funds. My life insurance policy's good, so the family's going to be just fine."

Fortunately for Kamprath, things started to turn around after a referral to Dr. Lin-Wang Dong, director of the CardioVascular Institute's Pulmonary Hypertension program, 1800 15th St., No. 310, in Greeley. Where other doctors in Denver and Arizona kept referring Kamprath from the cardiologist — the heart doctor — to the pulmonologist — the lungs and respiratory tract doctor — and back again, Dong took a step back and looked at Kamprath as a total patient. Dong started to help Kamprath recover in ways other doctors couldn't.

Pulmonary hypertension can be difficult to diagnose and treat because of the complex interplay between the heart and the lungs. The pulmonary arteries take de-oxygenated blood in the heart to the lungs, where the blood picks up oxygen. The pulmonary veins then return oxygenated blood to the heart, where it sends the blood out to the rest of the body. Issues at various points in this system can cause pulmonary hypertension, which is a problem for cardiologists, who specialize only in the heart, and pulmonologists, who specialize only in the lungs and respiratory tract. Focusing only on the tests of the systems they've specialized in, the two specialists can end up passing patients with pulmonary hypertension back and forth, like Kamprath experienced.

High blood pressure in the lungs can deprive the body of the right amount of oxygen, causing shortness of breath and a range of other problems. For Kamprath, it didn't seem like much more than a cold about five years ago, but as his condition worsened, he started seeing specialists.

Though doctors thought pulmonary hypertension was a possibility, they contradicted themselves and weren't able to bring Kamprath lasting relief or treatment. Eventually he decided he at least needed a cardiologist near his work at the University of Colorado Boulder, where he's the deferred maintenance program manager for facilities. That cardiologist recommended he start seeing Dong.

Just as Kamprath started seeing Dong, about a year and a half ago, he ended up in the hospital with pneumonia. He caught an illness because pulmonary hypertension weakened his immune system. Kamprath said he was having a terrible time breathing and gained more than a dozen pounds of fluid from around his lungs. In about 48 hours, Kamprath said he had nearly 16 pounds of fluid drained. With visits around morning, midday and night, Dong kept a watchful eye on Kamprath.

"I think if I hadn't been here, I doubt I would have made it," he said. "I credit him with saving my life."

Dong, thanks to an extensive background including 16 years in a research lab as a cardiovascular pathophysiology major, started the pulmonary hypertension program in 2008. The Pulmonary Hypertension Association designated the program a Regional Clinical Program earlier this year. Only six other programs have earned the accreditation in the U.S. — the nearest one to Greeley is located in Dallas. Dong said they see patients from all over Colorado, Kansas, Nebraska and Wyoming. Margo Karsten, CEO for Banner Health in northern Colorado, said the accreditation helps a high-risk, vulnerable group of patients find the care they need by meeting the standards of a third party.

"Because of the way that Dr. Dong has coordinated their care, their quality of life is drastically impacted in a positive way," Karsten said.

For the past year and a half, Kamprath has primarily seen Dong, and he still can't say enough about Dong's expertise. When he first met Dong, Kamprath was on four liters of oxygen and expecting his life to end soon. These days, he's comfortable with a one-liter oxygen tank and is talking about visiting colleges with his soon-to-graduate daughter.

"He opened a door that, two years ago, was closed. … His ability to take where I was at and transform it into where I am today — I'm speechless," Kamprath said. "I'm more active than I've ever been. I go to my kids' basketball games."

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