pulmonary hypertension treatment

SAPH Patients May Benefit from PH-specific Therapy, Study Suggests - Pulmonary Hypertension News

Posted: 29 Mar 2019 01:30 AM PDT

Patients with sarcoidosis-associated pulmonary hypertension (SAPH) can improve their levels of a key biomarker after receiving specific treatment for PH, which suggests that PH-specific treatment may benefit this patient population, according to a real-world study.

The study, "Clinical Features and Outcomes of Patients with Sarcoidosis-associated Pulmonary Hypertension," was published in the journal Nature Scientific Reports.

Although reports have shown a high frequency of PH in people with sarcoidosis, no specific PH therapies are available for these patients. Thorough understanding how PH affects blood flow and heart function in people with SAPH is limited, particularly in real-world practice.

Therefore, a team at Duke University Medical Center decided to study a large group of patients with newly diagnosed SAPH, and their treatment, clinical outcomes, and disease management.

A total of 95 patients (76% women, 86% African-American) were identified from 1990–2010 using the Duke University Health System database. Diagnosed with SAPH at a mean age of 52, all patients had their diagnosis confirmed by right heart catheterization, and were followed up for a median of three years.

The study evaluated post-therapy changes in 6-minute walk distance (6MWD), a measure of exercise capacity, and in N-terminal pro-brain natriuretic peptide (NT-proBNP), which is a PH biomarker. This biomarker was assessed because of the questionable validity of 6MWD as a marker for PH severity in lung sarcoidosis, the scientists noted.

Pre- and post-therapy 6MWD and NT-proBNP values were measured three to nine months before and after initiating the first treatment.

At baseline, 70% of patients had stage IV or advanced sarcoidosis, and almost all (99%) were symptomatic. Also, 59% had moderate or severe right ventricular (RV) enlargement, while 55% had moderate/severe RV dysfunction. The median NT-proBNP value was elevated (910 pg/mL).

Mean pulmonary arterial pressure, or mPAP, (at 49 mmHg) and pulmonary vascular resistance (8.5 Woods units) indicated severe PH. Most patients were being treated with medications for pulmonary sarcoidosis, the most common being steroids (61%).

PH-specific therapies for SAPH were used by 74 patients. Among this group, 36 (37.9%) patients initially received oral monotherapy, 23 (24.2%) had parenteral monotherapy, four (4.2%) had inhaled monotherapy, 11 (11.6%) received combined therapy, and 21 (22.1%) had no therapy. Parenteral delivery refers to a route other than the digestive tract, such as under-the-skin, intramuscular, or intravenous injection.

The percentage of women was higher in the group on PH-specific therapy (81%) than in the no therapy group (57%). Receiving treatment for PH was not associated with RV dysfunction, sarcoidosis stage, or mPAP. The prevalence of moderate or severe RV dysfunction at baseline was highest (83%) in people on parenteral stand-alone therapy, and lowest (48%) in patients on no therapy.

The median time to hospitalization or death was six months, and the mortality rate over a median three-year follow-up was 32%.

Among the 64 patients (67%) with a clinical event (hospitalization or death), 73% had advanced sarcoidosis. Imaging and blood flow data of RV function and PH were not associated with these outcomes. However, the follow-up NT-proBNP value was significantly higher in patients who died (1258.0 pg/mL) or were hospitalized (262.0 pg/mL).

Patients on intravenous, inhaled, or oral combination treatment for SAPH were more likely to experience clinical symptoms, which the researchers attributed to having more advanced disease.

Analysis of the 37 patients on PH-specific therapy, and with pre- and post-treatment 6MWD and NT-proBNP values, revealed that 33 continued on their initial therapy for the duration of their follow-up. While 6MWD results did not change over time, NT-proBNP levels significantly decreased (improved), with a median change of 51.2%.

Although cautioning that more trials are needed, the researchers concluded that the "use of PH-specific therapy may be helpful in selected patients with SAPH," and that NT-proBNP may be an important measure of treatment efficacy in these patients.

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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