Symbolic illustration of healthy lungs with cherry blossoms, representing hope and renewed health in an Asian setting.

Breathing Easier: Understanding and Managing Pulmonary Hypertension in Asia

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Mira Elwood in Health & Wellness May 2025 • 5 min read.

"Despite modern advancements, pulmonary hypertension remains a critical health challenge. Learn about the latest research and treatment options available in Asia to improve outcomes and enhance quality of life."


Pulmonary arterial hypertension (PAH) has significantly risen in prominence over the past two decades. This surge in attention is largely fueled by the development of innovative treatments and deeper insights into the genetic and pathological underpinnings of the disease. Once considered a rare and often overlooked condition, PAH now commands major sessions at international conferences, driving increased frequency of discussions at both national and global levels. This heightened focus has spurred the formation of new national societies dedicated to advancing research and treatment.

Alongside this growing interest, numerous national and international registries have emerged, aiming to evaluate patient outcomes in real-world settings. These include the REVEAL registry in the USA, the PHSANZ registry in Australia and New Zealand, and several European registries. These registries are vital in tracking and understanding the effectiveness of current treatments and identifying areas for improvement in patient care.

The recent publication in 'Respirology' by Lim et al. marks an important contribution to understanding the epidemiology and outcomes of PAH. As the first report from a registry in the Asia-Pacific region (Singapore), it offers over a decade of follow-up data (2003-2016). This study enriches our understanding of how PAH is managed and the challenges faced in this specific geographic context.

Key Insights from the Singapore PAH Registry

Symbolic illustration of healthy lungs with cherry blossoms, representing hope and renewed health in an Asian setting.

The study by Lim et al. provides valuable insights into the long-term management of PAH in Singapore. While the study involves a smaller number of participants compared to other registries, its extended follow-up period offers a unique perspective on disease progression and treatment outcomes. At three years, the survival rate was 70.9%, showing the persistent challenges in managing PAH despite available treatments. This figure aligns with findings from the larger REVEAL registry, highlighting the global complexities of treating this condition.

Other contemporary registries report slightly improved survival rates, with the Australian/NZ registry noting a 77% three-year survival rate in its most recent incident cohort (2012–2016). However, it’s important to recognize that significant mortality remains a concern across all regions. The Lim et al. study also confirmed the prognostic utility of the REVEAL risk score, although the distinction between risk groups was simplified due to the smaller sample size.

Key observations include:
  • Survival rates at 3 years are around 70.9%, indicating ongoing management challenges.
  • REVEAL risk score is useful but simplified due to smaller numbers.
  • Outcomes vary across registries due to differences in sample sourcing and local practices.
  • Extended follow-up provides unique insights into long-term disease management.
Beyond the initial three-year period, outcomes vary more significantly among different registries. This variability likely reflects differences in sample sourcing, enrollment timeframes, and local clinical practices. Understanding these nuances is crucial for interpreting registry data and applying findings to diverse patient populations. The increased interest in PAH has also facilitated the development of modern international guidelines for management, such as the 2016 guidelines prepared jointly by the European Society of Cardiology and the European Respiratory Society. These guidelines emphasize a 'treatment-to-target' approach, which aims to reduce risk based on a comprehensive risk identification profile.

Addressing the Challenges and Future Directions

Despite advancements, the high cost of available drugs and funding limitations in national health systems remain significant barriers. In Singapore, for example, no PAH-specific agents are funded through the national system, creating a substantial negative impact on patients. This lack of funding is a major problem in many Asian-Pacific countries. Even in more fortunate regions, such as Australia, only monotherapy is typically supported by federal health systems, making combination therapies contingent on pharmaceutical industry programs or local health network support. Moving forward, ongoing research is essential to improve PAH therapy. While many efforts are focused on identifying new treatment pathways, it’s crucial to ensure that existing, proven drugs are more readily available and optimally used. The paper by Lim et al. underscores that there is still progress to be made in this area, particularly in ensuring equitable access to care and effective treatment strategies.

Everything You Need To Know

1

What is pulmonary arterial hypertension (PAH), and why has it become such a prominent health concern?

Pulmonary arterial hypertension (PAH) is a condition that has gained significant attention due to advancements in treatments and a better understanding of its genetic and pathological causes. It's important because, once overlooked, PAH is now a focal point in medical discussions, leading to the formation of societies and registries dedicated to improving research and care. The implications of this increased focus are better treatment strategies, improved patient outcomes, and a global effort to combat this critical health challenge.

2

What role do national and international registries play in understanding and managing pulmonary arterial hypertension (PAH)?

National and international registries, such as the REVEAL registry in the USA, the PHSANZ registry in Australia and New Zealand, and several European registries, are essential for understanding pulmonary arterial hypertension (PAH). These registries track patient outcomes in real-world settings, helping evaluate the effectiveness of current treatments and pinpoint areas for improvement. Their importance lies in providing data-driven insights that guide clinical practices and treatment strategies, ultimately leading to better patient care. Without these registries, understanding the true impact of treatments and the disease's progression would be severely limited.

3

Why is the study by Lim et al. from the Singapore PAH Registry an important contribution to our understanding of pulmonary arterial hypertension (PAH)?

The study by Lim et al., which is the first report from a registry in the Asia-Pacific region (Singapore), provides a unique and crucial perspective on pulmonary arterial hypertension (PAH) due to its extended follow-up period (2003-2016). This study enriches our understanding of how PAH is managed and the challenges faced in this specific geographic context. While involving fewer participants, its long-term data offers insights into disease progression and treatment outcomes that are invaluable for shaping strategies in similar settings. The implication is that it highlights the need for tailored approaches in different regions, acknowledging variations in healthcare systems and patient populations.

4

What does the 'treatment-to-target' approach mean in the context of managing pulmonary arterial hypertension (PAH), and why is it important?

The 'treatment-to-target' approach, emphasized in the 2016 guidelines prepared jointly by the European Society of Cardiology and the European Respiratory Society, aims to reduce risk based on a comprehensive risk identification profile. It's significant because it represents a proactive and personalized strategy in managing pulmonary arterial hypertension (PAH). By tailoring treatment to specific risk factors, healthcare providers can optimize outcomes and improve patients' quality of life. However, implementing this approach effectively requires access to advanced diagnostic tools and a thorough understanding of individual patient profiles.

5

What are the major challenges hindering progress in pulmonary arterial hypertension (PAH) therapy, particularly concerning access to treatments?

The high cost of available drugs and funding limitations in national health systems significantly impede progress in pulmonary arterial hypertension (PAH) therapy. In Singapore, the lack of funding for PAH-specific agents through the national system has a major negative impact on patients. This problem extends to many Asian-Pacific countries. The implication is that despite the availability of effective treatments, unequal access due to economic constraints prevents many patients from receiving optimal care. Addressing this challenge requires advocating for increased funding, exploring cost-effective treatment options, and ensuring equitable access to healthcare resources.

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