Pulmonary Hypertension

Particular Histological Pattern Linked to Severe Pulmonary Hypertension in COPD - Pulmonology Advisor
Widening the Definition and Use of Palliative Care in PAH - Pulmonology Advisor
Hearing 'It Could Be Worse' Makes Me Feel Invalidated - Pulmonary Hypertension News
Transition from PDE5i to Adempas Without Washout is Viable for PH - Pulmonary Hypertension News

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

Hearing 'It Could Be Worse' Makes Me Feel Invalidated - Pulmonary Hypertension News

Posted: 29 Mar 2019 02:00 AM PDT

"It could be worse."

It's amazing how quickly these four words make my head spin. People living with chronic illness are well aware of the fact that "it could be worse." I know that I could be in a hospital bed right now. Instead, I'm at home typing on my computer, living to see another day. I know that there are people who have died of terrible illnesses and ones who have died unexpectedly. I know other parts of the world don't have the healthcare that we do, and I am thankful to have the quality care that I do. I am well aware that it "could be worse."

The first illness that comes to mind for many people who don't know of other serious conditions is cancer. The amount of times my condition has been dismissed or belittled because it wasn't cancer is ignorant. Cancer is often a devastating condition and I have witnessed the effects of it firsthand, but it should not be used to throw in the face of someone with another debilitating condition. It should not be used to justify someone's thinking that it "could be worse."

I want others to know that, yes, my condition could be worse. I am lucky every single day to have two feet on the ground. That fact doesn't diminish my pain and suffering, and it certainly doesn't erase my medical conditions. My "worst" shouldn't be brushed aside. When my suffering is dismissed by others, I start dismissing my health and ignoring how I actually feel.

Hearing "it could be worse" not only makes me feel invalidated, but it also makes me feel remorse for those who do have it worse. My focus drifts away from myself and I am left feeling selfish. I begin to think that my "problems" don't matter. This way of thinking not only is a punishment to my physical health, but it's damaging to my mental health. Putting off my pain and suffering because others have it "worse" is not an act of selflessness. It's an act of self-destruction and can be extremely dangerous.

There are so many people out there who misjudge and misread my suffering because I don't fit the picture of what "sick" looks like. When I was diagnosed with pulmonary hypertension and other rare diseases, I tried to convince myself that it "wasn't that bad." I thought, "At least I didn't have oxygen" … and then I was put on oxygen. "At least I can still go places and don't need oxygen as much" … until I passed out after not wearing it while watching a football game. "At least I'm not in the hospital." … Flash forward to November when I was hospitalized for three months. Any condition can be worse. It makes me anxious to even think about the endless possibilities.

Thinking about "who has it worse" or "how it could be worse" has not done me any good. It has made me neglect my own health. It has made me think that I didn't deserve the care I was receiving. Those with chronic illness know it could be worse. But, what others need to understand is, it could also be a lot better for us too.

I realized that I have control over defining when I've reached my breaking point. My "worst" is solely my own. It doesn't need comparing, it doesn't need judging, it doesn't need belittling. It needs recognition. I feel fortunate to be alive, to be able to stand on my own two feet, to be able to physically type this out and create these words in front of me. I can be fortunate and grateful and still have a right to feel my suffering and pain. I am allowed to say "this is my worst."

With any chronic illness, instead of saying "it could be worse," stop and acknowledge someone's suffering even if it seems "small." Each person's pain is their own. It's real. It's valid. Every person, no matter their struggle, deserves to feel sympathy, compassion, and deserves to feel heard even though someone out there "has it worse."

***

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 Services, and are intended to spark discussion about issues pertaining to 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.

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