Improving Pulmonary Hypertension Diagnosis and Care: Lung Association’s Guidance Published in Pulmonary Circulation

Pulmonary hypertension (PH) is a serious condition in which there is abnormally high pressure in the blood vessels between the lungs and the heart. PH has a high mortality rate if untreated. Unfortunately, the disease is not consistently diagnosed or treated across the United States. To improve the diagnosis and care of people in the U.S. living with PH, the American Lung Association, in collaboration with the Pulmonary Hypertension Association, convened an expert panel who published its "Guidance to the Guidelines" in Pulmonary Circulation.

“People with PH often endure symptoms for months or even years without proper treatment because many of the initial signs and symptoms, such as feeling short of breath or fatigued, are similar to symptoms of other chronic lung diseases,” said Deb Brown, Chief Mission Officer of the American Lung Association. “The ‘Guidance to the Guidelines’ is an integral tool to help healthcare providers who might not be familiar with guideline updates to diagnose PH accurately and quickly to help people living with PH get the correct treatment they need to live longer, more full lives.”

“Delayed diagnosis remains one of the greatest challenges facing people living with pulmonary hypertension. By translating complex clinical guidelines into practical tools for U.S. providers, this guidance represents a meaningful step toward earlier diagnosis, appropriate treatment and better outcomes for patients nationwide,” said Matt Granato, President and CEO of the Pulmonary Hypertension Association.

In 2022, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) convened a task force to develop new ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. These new guidelines integrate recent developments in detecting and managing PH. The guidelines can help providers identify patients earlier, and the information can be vital to disease management and health outcomes. However, the intricacy of the guidelines, complexity of the U.S. healthcare system and heterogeneity of the U.S. population created a need for more customized, easily digestible guidance for healthcare providers supporting patients in the United States.

On April 8, 2024, the Lung Association and Pulmonary Hypertension Association convened a scientific roundtable of leading PH experts. The objective of the meeting was to develop educational resources for health care providers summarizing the currently available pulmonary arterial hypertension guidelines; to provide an expert critique of existing guidelines by outlining strengths, limitations, and resolving differences in areas where guidelines do not agree; and to offer guidance on incorporating the recently approved therapy sotatercept into clinical practice. The roundtable resulted in the development of practical flowcharts to support decision making, collectively forming a  “Guidance to the Guidelines” resource for the diagnosis and management of PH.

Dr. James Klinger, Medical Director of the Pulmonary Hypertension Center at Rhode Island Hospital and Professor of Medicine, Division of Pulmonary, Sleep and Critical Care Medicine at Brown University, facilitated the roundtable. He said, "Significant advances in medical therapies for PAH provide new hope for many patients with this devastating disease. The roundtable provided a unique opportunity for experts in this field to share ideas and outline approaches to how these exciting new therapies can best be utilized to help our patients."

See "American Lung Association Pulmonary Hypertension Roundtable: Executive Summary" in Volume 15, Issue 4 of Pulmonary Circulation.

Learn more about pulmonary hypertension at Lung.org/ph.