pulmonary artery hypertension

Widening the Definition and Use of Palliative Care in PAH - Pulmonology Advisor
Pulmonary Arterial Hypertension (PAH) Market Professional Inspection Report 2018-2026 - Business Herald
Balloon Pulmonary Angioplasty Improves Hemodynamics in Inoperable CTEPH - Pulmonology Advisor

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.

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Pulmonary Arterial Hypertension Pulmonary Hypertension

Pulmonary Arterial Hypertension (PAH) Market Professional Inspection Report 2018-2026 - Business Herald

Posted: 29 Mar 2019 03:44 AM PDT

Pulmonary hypertension or PH is a condition in which the blood pressure rises in the pulmonary artery, vein, or capillaries (collectively known as lung vasculature), which might lead to shortness of breath, fainting, leg swelling, and dizziness, among others. Pulmonary hypertension may be a relentless disease with a noticeable reduction in exercise tolerance. Pulmonary arterial hypertension or PAH indicates that the patient has high blood pressure in arteries that carry blood from the heart to the lungs. With PAH, the arteries in the lungs become thin or get blocked resulting into irregular blood flow. Since the flow of blood is hindered, it becomes hard for the blood to flow evenly thereby, increasing the blood pressure in the patient. In layman terms, it means that the heart works harder to pump blood. It may lead to weakening of the heart muscles after a certain period of time. Ultimately, pulmonary arterial hypertension may cause heart failure.

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Based on the reports gathered from a variety of sources, including CDC (Centers for Disease Control and Prevention) and WHO (World Health Organization), the global prevalence of pulmonary arterial hypertension is projected to be 100,000 – 200,000, that is about 15 – 50 patients per million of the population. Even though, the treatment available for pulmonary arterial hypertension is rather limited, various treatment options are available to decrease symptoms, slow down disease progression, and improve the quality of life. Endothelin receptor antagonists or ERAs, phosphodiesterase-5 (PDE-5) inhibitors and Prostacyclin and prostacyclin analogs are some of the widely prescribed drug classes used for treatment of the disease. Lately, a new drug class known as the soluble guanylate cyclase or sGC stimulators has been recognized to be effective in treating people with PAH.

On the contrary, the expiry of patents of major pulmonary arterial hypertension drugs such as Letairis, Tracleer, Tyvaso, and Adcirca is projected to pose a challenge for the growth of the market, as the generic versions of these drugs will be sold at 70% to 80% lower prices than that of the present branded drugs. Various breakthrough therapies for treatment of pulmonary arterial hypertension is projected to be a growth factor for the PAH treatment drug manufacturers, since the available drugs for the same are not sufficient to meet the patient's needs. These factors will fuel growth of the PAH market and increase the demand for PAH treatment drugs.

Growing prevalence of cardiac diseases to drive growth of the overall pulmonary arterial hypertension (PAH) market

Owing to the sedentary lifestyles, growing consumption of fast food, and lack of exercise, the global numbers for the patients with cardiac diseases is expected to rise substantially over the forecast period thereby, increasing the susceptibility of pulmonary arterial hypertension among the global population. This would propel the overall growth of the PAH market in the near future.

North America accounted for the largest market share of the global PAH treatment market, followed by Europe. These regional market are projected to witness considerable growth in the forecast period, owing to the freshly approved PAH medicines in the market and high prevalence of heart diseases.

Some of the major companies operating in the pulmonary arterial hypertension (PAH) market in the current scenario are Actelion Pharmaceuticals, Ltd., Gilead Sciences, Inc., United Therapeutics Corporation, Bayer HealthCare, Pfizer, Inc., Novartis International AG and GlaxoSmithKline plc.

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Balloon Pulmonary Angioplasty Improves Hemodynamics in Inoperable CTEPH - Pulmonology Advisor

Posted: 28 Mar 2019 11:03 PM PDT

Mildly improved hemodynamics and overall low mortality rates have been reported following the use of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). A systematic review and meta-analysis on the subject were conducted, with the results published in the International Journal of Cardiology.

A total of 17 prospective, noncomparative, observational studies that included 670 patients with CTEPH were included in the analysis. All of the studies had small sample sizes (ie, <100 patients). Studies with <20 patients were excluded from the analysis. The mean age of the participants ranged from 57.0 to 70.0 years, with a median age of 62.5 years. There were a median of 4 BPA sessions conducted per patient across the studies. Overall, the studies included a majority (68.4%) of women. Methodologic quality evaluation of the included studies suggested a high risk for bias, based mainly on the lack of a comparison group.

Five studies that included 167 patients reported the development of procedure-related complications. Of these pooled periprocedural complications, the most common was reperfusion lung injury in 25% of individuals, which was followed by reperfusion edema in 16%. In addition, the pooled incidence of perforation by guidewire, right ventricular failure, and acute pulmonary embolism was 3.6%, 1.8%, and 1.2%, respectively.

The meta-analysis demonstrated significantly decreased mean pulmonary artery pressure (–14.2 mm Hg; 95% CI, –18.9 to –9.5 mm Hg), pulmonary vascular resistance (–303.5 dyn · s/cm⁵; 95% CI, –377.6 to –229.4 dyn · s/cm⁵), and mean right arterial pressure (–2.7 mm Hg; 95% CI, –4.1 to –1.3 mm Hg) following the use of BPA. Moreover, 6-minute walk distance (67.3 m; 95% CI, 53.8-80.8 m) and cardiac output (0.21/min; 95% CI, 0.0-0.3/min) were also significantly increased following BPA.

In 12 studies that reported mortality with a median follow-up of 9 months after BPA (range, 1-51 months), the pooled incidence of short-term (ie, ≤1 month) and long-term (ie, >1 month) mortality was 1.9% and 5.7%, respectively.

The investigators concluded that the noncomparative evidence derived from this analysis can be used to facilitate clinical decision making until the results of larger, international multicenter randomized controlled studies become available.

Reference

Khan MS, Amin E, Memon MM, et al. Meta-analysis of use of balloon pulmonary angioplasty in patients with inoperable chronic thromboembolic pulmonary hypertension [published online February 23, 2019]. Int J Cardiol. doi:10.1016/j.ijcard.2019.02.051

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