Over the past 40 years, treatment for pulmonary arterial hypertension (PAH) has changed dramatically. What was once considered a disease with very few treatment options is now managed with a growing range of therapies that help people live longer and enjoy a better quality of life. A review recently published in Circulation looks back at the main milestones in pulmonary arterial hypertension care and explores what the future may hold.
Researchers explain that pulmonary arterial hypertension develops when the blood vessels in the lungs become narrowed and damaged. This increases the pressure in the lungs and forces the right side of the heart to work much harder, eventually leading to heart failure if left untreated. Today, doctors understand much more about the biological processes that drive pulmonary arterial hypertension. This has led to the development of treatments that target several important pathways involved in the disease. More recently, a new type of medicine called sotatercept became the first approved treatment designed to directly target blood vessel remodelling—the underlying
disease process in pulmonary arterial hypertension—rather than only helping the blood vessels relax. The review also highlights how the profile of pulmonary arterial hypertension has changed over time. Patients are now often diagnosed at an older age and may have other heart or lung conditions. Unfortunately, diagnosis is still frequently delayed, meaning many people have advanced symptoms before receiving the correct diagnosis.
Looking ahead, researchers are optimistic that artificial intelligence (AI) could help identify people with pulmonary arterial hypertension earlier by analysing information from ECGs, heart scans, and electronic health records. Earlier diagnosis could allow treatment to start sooner, when it may be most effective.
Treatment strategies have also evolved. Most people now start with combination therapy, using two medications from the beginning, while those at highest risk often receive three therapies, including intravenous or subcutaneous prostacyclin treatment. For people who remain at intermediate or high risk despite treatment, adding sotatercept has been shown to improve heart and lung function and reduce the risk of disease worsening. For patients whose disease continues to progress despite all available therapies, lung transplantation remains an important life-saving option.
Why is this important for people with pulmonary arterial hypertension?
This review highlights just how much progress has been made in pulmonary arterial hypertension care. Survival has
improved, new treatments are targeting the disease itself, and researchers are working toward more personalised approaches to treatment.
However, important challenges remain:
- Diagnosing pulmonary arterial hypertension earlier.
- Finding better treatments for people with complex health conditions.
- Developing therapies that protect the right side of the heart, which is crucial for long-term survival.
- Personalising treatment so that each patient receives the therapy most likely to benefit them.
Take-home message
Pulmonary arterial hypertension treatment has entered a new era. Thanks to major scientific advances—including disease-modifying therapies such as sotatercept—the outlook for people living with pulmonary arterial hypertension continues to improve. Researchers are now aiming for an even more ambitious goal: enabling people with pulmonary arterial hypertension to live not only longer, but with a normal life expectancy and a better quality of life.
Read more at this link on Circulation
Citation
Marc Humbert, MD, PhD, Katarina Zeder, MD, PhD, Gabor Kovacs, MD and Jason
Weatherald, MD – Circulation, Volume 154, Number 2
https://doi.org/10.1161/CIRCULATIONAHA.126.079274