Guest Editor's Memo

The story is familiar. A young woman presents to clinic years after she noted breathlessness and fatigue. She has seen other physicians, and the inhalers, exercise, and diet have not helped. In fact she is worse, much worse.

Delays in diagnosis of PAH are not a new phenomenon. The median time from symptom onset to diagnosis of primary pulmonary hypertension was 1.3 years in the early 1980s. That was an era without effective treatments. Now, 9 medications are approved for the treatment of Group 1 PAH; and yet the delay from symptom onset to diagnosis has not changed.

Earlier accurate diagnosis of PAH is imperative in an era of effective treatments. The Pulmonary Hypertension Association (PHA) launched a public awareness campaign “Sometimes it's PH” (www.SometimesItsPH.org); and this issue of Advances addresses key aspects of earlier accurate diagnosis of PAH. Lynn Brown, MD, describes what is known about delays in diagnosis of Group 1 PAH, emphasizing the importance of considering PAH as a possible diagnosis. Many PAH patients have co-morbidities such as obesity or asthma that mask PAH. Health professionals must consider “zebras” like PAH, especially when symptoms are out of proportion to those seen with more common disorders or when patients are not responding to treatment. Dr Brown also stresses the importance of patient contributions to delayed diagnoses. Like physicians, patients often attribute their breathlessness to growing older or being overweight. Laura D'Anna, DrPH, explores the difficult subject of health disparities on early diagnosis of PH. Social risk factors including low-wage jobs and low educational attainment often lead to delays in diagnoses of serious illnesses like PAH.

Screening protocols achieve early accurate diagnoses and improve outcomes for many disorders such as hypertensive vascular disease, cervical cancer, and breast cancer. Peter Leary, MD, and Jeffrey Edelman, MD, examine the subject of screening for PAH before the arrival of symptoms. Their article identifies at-risk populations, available screening modalities including genetic testing, and the limited evidence for therapeutic intervention early in the disease.

In addition to the 3 key articles by Drs Brown, D'Anna, and Leary and Edelman, 3 leading physician – scientists share their perspectives in the Pulmonary Hypertension Roundtable. Drs Marc Humbert, John New-man, and Julio Sandoval remind us that delayed diagnosis of PAH is truly a global problem. They identify many challenges and opportunities for the pulmonary hypertension community to achieve the goal of earlier accurate diagnosis of PAH.

Matt Hegewald, MD, provides the perspective of a busy practicing pulmonary and critical care specialist in “Ask the Expert.” As in every issue, the regular sections supplement the publication's theme for practitioners.

In my view, the present state of affairs is unacceptable. Change will require the effort of the entire PH community to raise awareness and to devise, test, and implement new strategies to reduce the time from symptom onset to diagnosis. This issue of Advances and the PHA campaign “Sometimes it's PH” offer an important start.