Empowering Primary Care: New Tool Aims To Improve Pulmonary Hypertension Diagnosis and Care

Pulmonary hypertension is a serious condition in which there is abnormally high pressure in the blood vessels between the lungs and the heart. While this condition is rare, if untreated it has a high mortality rate, and the disease is not consistently diagnosed or treated across the U.S. To help primary care providers better understand pulmonary hypertension, the American Lung Association and Pulmonary Hypertension Association today released the “Guidance to the Guidelines.”

Research and understanding of pulmonary hypertension (PH) is growing, however diagnosis and treatment of the disease vary across geographic areas and among health care providers. Many primary care physicians are not subject matter experts in rare lung disease and may need more guidance for recognizing symptoms, ordering diagnostic tests and knowing when to refer patients to specialty care.

“Diagnosing PH can be difficult because many of the initial signs and symptoms present like other lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD). A person with PH may have other medical conditions, and the disease may be associated with a variety of cardiovascular and respiratory diseases such as congenital heart disease, COPD or lupus,” said Albert Rizzo, Chief Medical Officer for the American Lung Association. “Managing PH often requires a multi-faceted and multidisciplinary approach, with active involvement from patients diagnosed and living with PH.”

In 2022, the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) convened a taskforce to develop new ESC/ERS Guidelines for the diagnosis and treatment of PH. These new guidelines integrate recent developments in detecting and managing PH. The guidelines can help providers to 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 creates a need for more customized, easily digestible guidance for the U.S. audience.

“PHA scientific leaders worked with American Lung Association to break down complex information about PH and diagnostic information for health care professionals who might not be as familiar with pulmonary hypertension,” said Matt Granato, President and CEO of the Pulmonary Hypertension Association. “We hope to see more people diagnosed when their symptoms initially arise instead of suffering for years without appropriate treatment. With earlier, accurate diagnosis, people with PH can find specialized care, targeted therapy, experience higher quality of life, and live longer with PH.”

To improve the diagnosis and care of people in the U.S. living with PH, the American Lung Association and Pulmonary Hypertension Association convened a scientific roundtable of PH experts on April 8, 2024. The experts created flowcharts for decision-making based on current guidelines and practice to develop a “Guidance to the Guidelines” for diagnosis and management of PH and to distribute it to health care providers across the U.S.

The new “Guidance to the Guidelines” document is available here.