by Phyllis Hanlon, Contributing Writer

Much research has been done on symptoms that affect quality of life in patients with chronic medical conditions like cancer and diabetes. But until 2016 no studies on this issue had been done in patients with pulmonary arterial hypertension (PAH). Reducing symptom burden is an important part of treatment for patients with WHO Group 1 PH (PAH, pulmonary arterial hypertension). Understanding the severity of these symptoms and how they affect patients’ quality of life is vital in order to understand how patients cope and live with this chronic illness. Recently, researchers from the University of Pennsylvania, an accredited Pulmonary Hypertension Care Center (PHCC), the University of Massachusetts Lowell and Massachusetts General Hospital collaborated on a study looking at the effects of PH symptoms on patients’ health-related quality of life.

Lea Ann Matura, PhD, RN, Annette McDonough, PhD, RN, and Diane L. Carroll, PhD, RN, FAAN, reported that, while patients who have PAH have many different symptoms due to their disease, not much is known about how severely these symptoms affect everyday living. The goal of their study ( was to identify how many symptoms patients experienced that negatively impacted their daily activities, how severe those symptoms were and how age, gender and other demographic factors might interact with PH symptoms to influence health-related quality of life (HRQOL). Matura and her colleagues stated that finding answers to these questions would make it possible for health care professionals to understand the coping methods patients currently use and to help them develop good management strategies.

The study involved 191 patients who self-identified as having PAH, although there was no confirmation from a clinician or medical record as to official diagnosis. Most of the subjects were female, married with a college education and on average 53 years old. Most were New York Heart Association functional class III-IV.

The researchers used a measurement tool called the Pulmonary Arterial Hypertension Symptom Interference Scale (PAHSIS) to look at how much the symptoms disrupted the patients’ lives. The scale measures 17 symptoms using a score from zero to ten, with ten being the most severe. Some of those symptoms include shortness of breath on exertion (i.e., with exercise), lying down, at rest and when waking at night; fatigue; sleep difficulty; chest pain, swelling in the abdomen, ankles or feet; fainting; dizziness; cough; nausea; loss of appetite; hoarseness; and Raynaud’s phenomenon (sensation of cold or numbness in the extremities).

Matura and colleagues also used the Medical Outcomes Study Short Form-36 (SF-36®) survey, which measures the general physical (e.g., functioning, body pain, general health and vitality) and mental (e.g., social functioning and emotional state) health aspects related to quality of life. This survey was not developed specifically for PAH, but has been used previously in PAH studies.

The authors reported that some of the symptoms which impacted physical HRQOL most were fatigue, dizziness and Raynaud’s phenomenon. The 69 percent decrease in physical HRQOL scores was attributed to fatigue, oxygen use, dizziness, age, gender and Raynaud’s. The 45 percent of the decrease in mental HRQOL scores was attributed to shortness of breath while lying down and fatigue.

The authors noted several important limitations of this study. Most notably, PAH diagnosis was self-reported. The study population was not considered diverse enough to be representative of the general population of PAH patients, and specific daily activities that were affected by PAH symptoms were not described. Also, the study was performed at a single point in time, and therefore future studies should look more closely at symptom trajectories and change over time.

Matura, McDonough and Carroll indicated that the next phase in this study should look more closely at which daily activities are affected by which symptoms, and also include the development and evaluation of ways to improve the most severe symptoms and reduce their interference in quality of life.

Each PH patient is different. It is essential that you talk to your own doctor about what treatment options are best for you. For more information on finding a doctor or an accredited care center, visit


A study of PAH patients looked at different symptoms of the disease and how these symptoms affect quality of life.

Some commonly reported symptoms included trouble sleeping, swelling in abdomen, ankles or feet.

Authors reported some of the symptoms which impacted health related quality of life most were fatigue, Raynaud’s phenomenon and dizziness.

Noted limitations of this study include self-reported diagnosis, lack of diversity in study population and lack of future studies to look at trajectory of symptoms and quality of life.