Experts create new tool to speed pulmonary hypertension diagnosis
New tool aimed at helping doctors navigate latest clinical guidelines
An expert panel convened by the American Lung Association and the Pulmonary Hypertension Association has developed practical flowcharts to help U.S. healthcare providers diagnose and treat pulmonary hypertension (PH).
The meeting aimed to guide providers through the most recent European guidelines for PH diagnosis and treatment. The results were compiled in the “Guidance to the Guidelines” tool provided by the American Lung Association, and a paper titled “American Lung Association Pulmonary Hypertension Roundtable: Executive Summary,” which was published in Pulmonary Circulation.
“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,” Deb Brown, chief mission officer of the American Lung Association, said in a press release.
PH patients can endure symptoms for long periods without proper treatment
PH is characterized by abnormally high blood pressure (hypertension) in the pulmonary arteries, the blood vessels that carry blood through the lungs. This makes the heart work harder to pump blood and leads to PH symptoms such as shortness of breath, fatigue, and chest pain.
“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,” Brown said.
The European Society of Cardiology and the European Respiratory Society published new guidelines for the diagnosis and management of PH in 2022. These guidelines defined PH as mean pulmonary arterial pressure (a measure of blood pressure in the lungs) higher than 20 mmHg at rest, and aimed to help providers identify patients and begin treatment earlier.
They also provide specific information for all PH types, particularly pulmonary arterial hypertension, characterized by narrowing of the pulmonary arteries, and chronic thromboembolic pulmonary hypertension, in which PH develops due to blood clots in the lungs.
Roundtable developed educational resources for U.S. healthcare providers
To discuss the latest recommendations from the European guidelines, the American Lung Association and the Pulmonary Hypertension Association convened a scientific roundtable of leading PH experts in April 2024. The roundtable aimed to develop educational resources for U.S. healthcare providers by summarizing the most recent guidelines, outlining their strengths and limitations, and resolving differences in areas where guidelines disagree.
It also sought to guide the incorporation of Winrevair (sotatercept-csrk) into clinical practice. In the U.S., the treatment is approved for adults with PAH to improve exercise capacity and World Health Organization (WHO) functional class, as well as to reduce the risk of clinical worsening events, including hospitalization for PAH, lung transplant, and death.
“Significant advances in medical therapies for PAH provide new hope for many patients,” said James Klinger, MD, medical director of the Pulmonary Hypertension Center at Rhode Island Hospital and professor at Brown University. “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.”
The panel of experts generally agreed on the newly established blood flow parameters for PH diagnosis and for defining PH types, as well as the symptoms, clinical characteristics, and diagnostic signs described in the 2022 European guidelines. They also concurred with recommendations for screening certain high-risk groups, such as patients with connective tissue diseases or heritable PAH. Still, the panel noted that patients with connective tissue diseases who do not have symptoms or those with interstitial lung disease, which is marked by inflammation and scarring, should also be screened for PH.
The experts noted that there is still an unacceptable delay in PH diagnosis and made several recommendations regarding diagnostic approaches. These included referrals to expert care centers when echocardiogram findings are inconclusive.
The panel also reviewed the guidelines and their model for patients’ risk stratification, which is based on WHO functional class, the 6-minute walk test of exercise endurance, and levels of a biomarker of heart failure called brain natriuretic peptide/N-terminal pro-brain natriuretic peptide. Regarding Winrevair, they agreed there is no evidence supporting its use as first-line therapy, but it should be considered as part of combination therapy in high-risk patients or those who do not reach or maintain low-risk status after initial treatments.
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