pulmonary hypertension symptoms

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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PH, Other Disorders May Develop from Congenital Heart Defects, Expert Says - Pulmonary Hypertension News

Posted: 25 Mar 2019 01:00 AM PDT

Heart defects that develop before birth may lead to a variety of complications later in life, including pulmonary hypertension (PH), according to cardiologist Sanjay Gandhi, MD.

However, he says, it is still not possible to predict if or when these complications will arise.

Congenital heart defects (CHDs) occur when the heart or the blood vessels around it do not develop normally in the fetus. They are the most common type of birth defect, affecting approximately 1 percent of all newborns — which accounts for about 40,000 babies in the U.S. each year.

There are several types of CHDs: holes in different areas of the heart, blood vessels narrower than normal, leaky valves, or in more severe cases, parts of the heart that are incorrectly formed, misplaced, or even nonexistent. These can significantly undermine blood flow and cause several disorders including arrhythmia (abnormal heart rhythms), stroke, congestive heart failure, atherosclerosis (hardening or narrowing of the arteries), and PH.

Owing to better methods of detection, diagnosis and treatment, living with CHDs has become easier, and today these defects are not as life-threatening as they were a few years ago. Data from the Centers for Disease Control and Prevention indicate that current survival rates up to the age of 18 are about 95 percent for babies with mild CHDs and 70 percent for those with severe CHDs.

However, some people with a CHD won't discover their problem until later in life. Heart defects may only present signs or symptoms at older ages, and because many variables can affect the onset of complications, there is no way to predict if or when these problems will appear.

"The heart is an amazing organ with any moving parts and there are a lot of possibilities as the heart is developing for something to not connect right, so there are a lot of different congenital defects, probably more than 30," Gandhi, an interventional cardiologist at Wake Forest Baptist Medical Center, said in a press release.

"Some of these are identifiable right away, or they manifest in early childhood or maybe in adolescence. But otherwise there are too many variables to predict when someone with CHD might experience symptoms or effects," he added.

These variables include the type of defect, severity, location, the function it affects, and if it is simple or complex. Age, general health, and lifestyle habits, especially related to diet and exercise, can also have an impact.

Even CHDs that are identified and treated in childhood can reappear and lead to complications in adulthood.

Fortunately, most of these cases are preceded by warning signs, such as shortness of breath, fatigue, dizziness or fainting, and heart palpitations. According to Gandhi, if a person has any of these symptoms, and they are significantly affecting his or her normal daily life, "you should definitely inform your primary health care provider, and explore whether you need to see a heart specialist and have some cardiac testing."

Although patients are often surprised when they receive a CHD diagnosis, some realize they had already been experiencing signs of the problem for a while.

"Sometimes they realize that things weren't always right with their breathing or stamina, that they couldn't keep up with their friends on the soccer field or in the swimming pool when they were younger, that maybe this problem with their heart did manifest earlier than they had thought," Gandhi said.

Doctors can use different methods to detect a heart defect, the most common being an electrocardiogram (ECG or EKG) and an echocardiogram (ultrasound), which can be complemented with blood tests, chest X-rays, and computed tomography (CT) or magnetic resonance imaging (MRI) scans.

Treatments vary based on the type and severity of the defect. These can include medications to relieve symptoms and reduce the risk of complications, or one or more surgeries that can be either minimally invasive, such as percutaneous catheterization, or more complex, such as open-heart procedures.

As opposed to pediatric cardiologists, cardiologists and heart surgeons of adult patients are usually more familiar with acquired heart disorders than CHD, so it is important that both experts work together to offer the best possible treatment to a person with a CHD.

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