PHA Canada on Pulmonary Hypertension Diagnosis, Care and Research Gaps

Pulmonary hypertension (PH) can be difficult to recognize, difficult to classify and, for many patients, difficult to access care for in time.

Rather than a single disease, PH refers to a complex group of conditions marked by high blood pressure in the blood vessels of the lungs. It can occur on its own, as in pulmonary arterial hypertension (PAH), or develop as a complication of underlying heart or lung disease.

While PH is considered rare, its clinical impact is significant. In Canada, more than 2,000 people have been diagnosed with PAH, while as many as 4,000 may be living with the condition. Although treatment options have improved over the past two decades, PAH remains a serious and progressive disease. Data on PAH in Canada have shown five-year survival of about 56% for PAH, underscoring the ongoing need for earlier diagnosis, better access to care and continued research.

For the Pulmonary Hypertension Association of Canada (PHA Canada), these gaps are central to its mission. Xtalks spoke with Jamie Myrah, Executive Director of PHA Canada, about the evolving PH treatment landscape, persistent diagnostic delays, the role of patient-oriented research and how the organization is expanding its reach to support people living with all types of PH.

Pulmonary Hypertension Is a Collection of Conditions

One of the first challenges in discussing PH is recognizing how broad the term is.

“Pulmonary hypertension is an interesting area because it’s really a collection of conditions,” Myrah said. “So it’s really more like a syndrome than it is a single disease area.”

The rarest types of PH include PAH and chronic thromboembolic pulmonary hypertension (CTEPH). In these areas, therapeutic advances have changed the outlook for many patients over the past two decades. In PAH, several treatments are now available, while in CTEPH, pulmonary endarterectomy surgery can be lifesaving for eligible patients by removing scar tissue and obstructive material from the pulmonary arteries.

Still, Myrah emphasized that rare forms represent only a minority of PH cases. Most people with PH develop it as a result of an underlying heart or lung condition.

This distinction matters because the challenges differ across PH groups. For people with group 2 PH, which is associated with left heart disease, and group 3 PH, which is associated with lung diseases and/or hypoxia, one of the most fundamental barriers is the lack of therapies specifically available to treat their PH.

For group 3 PH, Myrah said research is beginning to translate into new treatment possibilities, including therapies approved in the US and others still under investigation. This could bring treatment options to a population that has historically had few or no PH-specific therapeutic choices.

For patients with PAH and CTEPH, where treatments may exist, the challenge often shifts toward diagnosis, referral and access to expert care. The goal is ensuring that patients who could benefit from established standards of care are identified and connected to specialty teams quickly enough.

PHA Canada’s Role as a Full-Service Health Charity

PHA Canada has been operating for nearly 20 years and has grown into what Myrah described as a “full-service health charity.”

That means PHA Canada’s work extends well beyond general awareness campaigns.

“We are a full-service health charity running a range of programs and services from peer support and disease and treatment education right through to high-level health policy engagement, patient-oriented research and engaging with healthcare professionals and researchers to advance better outcomes for patients,” she said.

On the patient and caregiver support side, PHA Canada offers several peer support programs. These include a peer mentorship program that can directly match patients or caregivers with someone who has lived experience. The program evolved from an informal peer-support initiative established during the COVID-19 pandemic to help patients and caregivers connect with others who understood their experience.

PHA Canada also supports local, community-based support groups that operate more informally as mutual aid groups. In addition, the organization hosts annual support events in local communities wherever possible.

Education is another major part of its work. PHA Canada’s website provides resources on disease awareness, treatment education, psychosocial support and supportive therapies. One of its core print resources is a Navigating PH Guide for newly diagnosed patients, offering a comprehensive overview of the diagnosis and management of different types of PH.

PHA Canada also publishes a biannual magazine and produces regular blogs and daily communications to keep the PH community informed.

Raising Awareness Beyond the General Public

PHA Canada conducts awareness campaigns aimed at improving recognition of PH, including campaigns that target healthcare professionals to support earlier diagnosis and referral.

However, Myrah said some of the organization’s most impactful awareness work has come through its burden-of-illness surveys, impact reports and advocacy submissions. These have been used to communicate the needs of patients to healthcare decision-makers and advocate for improved access to medications, innovative therapies, oxygen therapy, referral pathways and broader supports such as disability benefits.

This type of awareness is about more than general disease recognition. It is also about making sure the healthcare system understands the real burden of PH and the realities of living with the disease.

“It can be very easy, I think, in PAH especially to say, ‘Oh, it’s this rare disease. It’s got 11 approved therapies. How bad can it be?’ Well, when only 50% of people are going to still be here after five years, it can be a lot better,” Myrah said.

For PHA Canada, raising awareness means ensuring that policymakers, payers, clinicians and health system leaders understand both disease burden and treatment burden.

Diagnosis Remains a System-Level Challenge

Canada has an important advantage in PH care: a national network of PH expert centers. Myrah said all provinces except Prince Edward Island have an expert center, and Ontario has five. Some centers have pediatric-specific expertise, and some also specialize in CTEPH treatment in addition to PAH and other forms of PH.

PHA Canada helps bring this expert community together through annual medical meetings, expert advisory committees and a national research network.

Despite this specialized infrastructure, Myrah said the main diagnostic barrier is not necessarily access to PH specialty centers. Instead, the issue often begins earlier in the pathway.

“The answer for me is not in the access to the specialty centers. It’s in access to primary care and the testing that is required to definitively diagnose pulmonary hypertension,” she explained.

PH symptoms can be nonspecific, especially in rare forms like PAH. Shortness of breath, fatigue, reduced exercise tolerance and other symptoms may be attributed to more common conditions, aging, deconditioning or other health concerns. This makes early suspicion difficult.

Patients may also delay seeking medical attention because they do not recognize unexplained shortness of breath as a symptom that requires evaluation. Once they enter the healthcare system, additional delays can occur through referrals, testing and specialist assessment.

Myrah said it can take anywhere from six months to many years from symptom onset to accurate diagnosis. On average, she said, it likely takes two years or more for about half of patients.

Access to echocardiography and other tests can contribute to delays. So can the need for specialized expertise in interpreting right heart findings. Diagnosing PAH requires sonographers who understand what they are looking for on the right side of the heart, as well as cardiologists and respirologists who are aware of PAH and know the referral pathways to PH clinics.

For Myrah, these are largely system issues, including access to primary care, access to diagnostic testing and awareness across the broader healthcare system.

Building Capacity for Patient-Oriented Research

One of PHA Canada’s major priorities in recent years has been strengthening patient-oriented research in PH.

The organization has worked to ensure that research priorities reflect the needs of the PH community. Several years ago, PHA Canada partnered with researchers, patients, caregivers, clinicians and scientists to identify national PH research priorities in Canada.

Since then, the organization has focused on building the capacity of patient partners so they can contribute throughout the research process. The goal is to move beyond involving patients only as study participants and instead recognize them as decision-makers and research partners.

This is particularly important in a complex disease area where traditional clinical research may not capture the full patient experience.

Why Registries Matter in Pulmonary Hypertension

PHA Canada is also investing in its registry as a way to make research participation more inclusive.

Clinical trial participation is not always possible for patients with PH. Some may not be sick enough to qualify, while others may be too sick. Patients with complex comorbidities are often excluded because they can complicate trial interpretation.

“Exclusion criteria mean that lots of folks with complex health conditions are excluded from participating in clinical trials,” Myrah said.

Registries can help capture real-world data from broader and more diverse patient populations, including those who may not fit narrowly defined clinical trial criteria. PHA Canada is working to enroll as many patients as possible across the country and to expand the types of data collected through the registry, potentially including more PH types, additional clinical and patient-reported data and increased biobanking.

Myrah said real-world evidence will be increasingly important as the field asks more complex questions about precision medicine, treatment sequencing and public reimbursement.

PH treatment is becoming more complicated, particularly in PAH, where multiple therapies and combinations may be used. For public payers, the question may increasingly become which patients should receive which therapies, when and in what sequence.

Patients want the same thing, Myrah said: to receive the right therapy and avoid treatments that are unlikely to benefit them or may cause harm.

“I think we all want the same thing and registries and real-world evidence are going to be a big part of that,” she said.

Collaboration Across Patients, Clinicians, Researchers and Industry

Collaboration is central to PHA Canada’s approach. The organization works with clinicians, researchers, patient partners and industry to advance disease awareness, research and access to care.

In practice, that collaboration can include identifying patient-centered research priorities, expanding registry data collection, supporting disease awareness campaigns and ensuring lived experience informs how studies are designed and communicated.

In disease awareness, PHA Canada partners with industry while keeping the patient voice at the center. Myrah said the organization is committed to ensuring that patients speak for themselves about their lived experiences, rather than relying primarily on healthcare providers to act as surrogates for the patient’s perspective.

This reflects a broader shift in health research and advocacy: patients are not only recipients of care or sources of data, but contributors to scientific priorities, study design, policy discussions and knowledge translation.

For PHA Canada, this is especially important as the field moves toward more personalized approaches to treatment and as real-world evidence becomes more important to clinical and reimbursement decision-making.

Expanding Support to All Types of PH

Looking ahead, one of PHA Canada’s strategic priorities is expanding its reach.

Historically, many of the organization’s programs, services and educational resources have focused on group 1 PAH and, to some extent, group 4 CTEPH. PHA Canada is now working to become more inclusive of all types of PH, including group 2 and group 3.

This also means reaching populations that have traditionally been underserved, including people living in rural communities and Indigenous patients and families.

To do this, PHA Canada is deepening relationships with healthcare providers beyond PH expert centers. Myrah pointed to a recent example: the organization’s first interstitial lung disease education day, held in partnership with the Firestone Clinic at St. Joseph’s Healthcare Hamilton.

The event represented a step outside the traditional PH expert center network and was designed to reach people at risk of PH, as well as the healthcare providers who care for them.

Becoming the Natural Partner for PH Research in Canada

Research investment is another major priority for PHA Canada. The organization has a longstanding research scholarship program and continues to build its role in Canada’s PH research ecosystem.

Myrah said PHA Canada’s vision is to be seen as the logical partner for all PH research projects in the country.

“Nobody should be doing PH research in Canada without thinking, how am I going to engage patients in this process? And if I’m going to engage patients in this process, then PHA Canada needs to be at the table,” she said.

That includes investigator-initiated research as well as industry-initiated projects. PHA Canada wants to be involved not only at the end of a project as a knowledge translation partner, but from the earliest stages of project conception.

For patients and families, this could help ensure that research addresses questions that matter in real life: how long diagnosis takes, how therapies affect daily functioning, how treatment burdens shape quality of life, which patients benefit from which approaches and how access can be improved across geography, disease type and community.

What Comes Next for PH Care in Canada

Pulmonary hypertension care has advanced considerably, particularly for PAH and CTEPH. But major gaps remain across diagnosis, access, treatment options, evidence generation and support for underserved populations.

PHA Canada’s work sits at the intersection of these challenges. By combining patient support, education, policy advocacy, registry development and research partnerships, the organization is helping shape a more inclusive and evidence-driven future for PH care in Canada.

As new therapies emerge and the field becomes more complex, Myrah’s message is clear: patients must be part of the process from the beginning.

For PH research, care and policy to move forward, lived experience cannot be an afterthought. It must help define the questions, guide the evidence and shape how advances reach the people who need them.