Researcher creates first national registry for pulmonary hypertension

Pulmonary hypertension (PH) is a serious condition that causes blocked arteries in the lungs, often leading to heart failure. Patients with PH often suffer with shortness of breath, leg swelling and fainting. Despite treatment options, including lung transplants, many PH patients become sicker and ultimately die from their disease.

Kathy Downey, 52, was diagnosed with PH in March 2020, five years after she started seeking answers for her symptoms.

“It started with shortness of breath and exhaustion,” says Downey. “Over time it became harder to walk even short distances. Even doing the laundry became a difficult task.”

When the mom of young adult children started developing numbness in her arms in February 2020, she went to the emergency room (ER). The ER physician ordered several tests on her heart and lungs that prompted her respirologist to look even further into the heart-lung connection.

“On March 13, I learned I had PH and it was quite severe,” says Downey, explaining the vessels in her lungs are narrowed, so blood has difficulty getting through the lungs for reoxygenation.

Downey is now being treated with several medications and is on supplemental oxygen 24 hours a day, but says she is “relieved” to have received her diagnosis and she is optimistic about the future.

Although PH is a serious condition, there is little known about its prevalence in Canada. Researchers are also unsure of how Canadian health-care providers are treating the condition, making it difficult to predict how patients, who are generally diagnosed between the ages of 40 and 60, will fare.

Dr. Jason Weatherald, MD, a clinician-researcher with the Libin Cardiovascular Institute who specializes in heart and lung issues, is hoping to change that. He recently received a Seed Catalyst Grant from the Heart and Stroke Foundation of Canada to understand how PH is being diagnosed and treated in Canada and to develop new and better ways of predicting poor health outcomes for patients with this condition.

“Our overarching goal is to help doctors treat these patients better in the future,” says Weatherald, noting PH is relatively uncommon, affecting an estimated 400 people in Southern Alberta, and treatment is complicated.

The funds will help him finish setting up the first Canadian national registry, a project he became involved in several years ago. Currently 10 centres, including Calgary, are participating in the registry, which will be used to study risk factors and outcomes of individuals with PH.

Researchers will use the information to study how treatments and health interventions impact the quality of life in patients with PH. Next, they will use computer models to predict health outcomes in Canadian patients with the disease.

“A lot of other countries have national registries, but we don’t really have any idea of what is going on in Canada,” he says, noting the database will include information from several thousand PH patients. “We don’t know if there are unique Canadian factors that might affect outcomes.”

Researchers will assess several variables, such as ethnicity and distance from care centres. A unique aspect to Weatherald’s project is its sex and gender considerations. These questions are critical because in other countries, PH has been found to be more prevalent in women, but men with the condition often fare worse. However, researchers don’t fully understand why. Weatherald is hoping that the project will help solve those mysteries and allow physicians to identify and treat patients most at risk.   

“Ultimately, we want fewer patients to progress in their disease to the point of needing a lung transplant or dying of heart failure,” says Weatherald. “This database and risk prediction model are keys to better outcomes for patients.”