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

"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.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

This article is based on research published under:

DOI-LINK: 10.1080/01443410.2017.1381288, Alternate LINK

Title: Editorial

Subject: Developmental and Educational Psychology

Journal: Educational Psychology

Publisher: Informa UK Limited

Authors: Detlef Urhahne

Published: 2017-09-28

Everything You Need To Know

1

What factors have contributed to the increased prominence of pulmonary arterial hypertension (PAH) in recent years?

Pulmonary arterial hypertension (PAH) has gained considerable attention due to the development of innovative treatments and a deeper understanding of its genetic and pathological basis. This increased focus has led to more discussions, the formation of national societies, and the establishment of registries to track patient outcomes in real-world settings. However, there is still a large way to go.

2

Why are national and international registries important in the context of 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 European registries are vital for evaluating patient outcomes. These registries help in tracking the effectiveness of current treatments, identifying areas for improvement, and understanding the nuances of PAH management across diverse patient populations and geographic locations. The Singapore PAH registry adds to this body of knowledge with its unique long-term follow-up data.

3

What are the key insights from the Singapore PAH Registry highlighted in the study by Lim et al.?

The Singapore PAH Registry, reported by Lim et al. in 'Respirology', offers over a decade of follow-up data on PAH management in Singapore. Key findings include a three-year survival rate of 70.9%, indicating persistent management challenges, and confirmation of the REVEAL risk score's utility. The study highlights the importance of long-term data in understanding disease progression and treatment outcomes in the Asia-Pacific region.

4

How do modern international guidelines, such as those from the European Society of Cardiology and the European Respiratory Society, approach the management of pulmonary arterial hypertension (PAH)?

Modern international guidelines, such as the 2016 guidelines from the European Society of Cardiology and the European Respiratory Society, emphasize a 'treatment-to-target' approach for PAH. This approach aims to reduce risk by using a comprehensive risk identification profile to tailor treatment strategies. However, the implementation of these guidelines can be hindered by factors like the high cost of drugs and funding limitations.

5

What are the major challenges in ensuring equitable access to effective treatment for pulmonary arterial hypertension (PAH), especially in the Asia-Pacific region?

The high cost of drugs and funding limitations in national health systems, particularly in the Asia-Pacific region, pose significant barriers to effective PAH treatment. For instance, in Singapore, PAH-specific agents are not funded through the national system, and in Australia, only monotherapy is typically supported. Addressing these challenges requires ongoing research, ensuring the availability of existing drugs, and promoting equitable access to care and effective treatment strategies.

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