Volume 11: Issue 2

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

The Clinical Trials Update highlights new and ongoing research trials that are evaluating therapies for PAH. In this issue, Dr Torres presents information on current studies that may result in re-evaluation of historical endpoints for future PH clinical trials.

Substantial delays between the onset of pulmonary arterial hypertension (PAH) symptoms and diagnosis of the disease continue for the majority of PAH patients. The delays persist despite a growing number of effective treatments and the knowledge that early initiation of therapy is clinically beneficial. Patients need to be encouraged not to rationalize their symptoms when they do occur but to seek medical evaluation. Physicians should be alert to the common symptoms found at PAH presentation and recognize that patients may have comorbid conditions that mask the disease. The goal of identifying patients at the earliest stages of functional impairment will not be achieved unless PAH is considered as a possible diagnosis.

“Of all the forms of inequality, injustice in health care is the most shocking and inhumane.” – Dr. Martin Luther King, Jr.

Early recognition, diagnosis, and treatment of pulmonary arterial hypertension (PAH) is a worthwhile endeavor in that it remains underdiagnosed in an era where 9 FDA-approved treatments are available, which have shown to provide clinical improvements and prolonged survival.1 Furthermore, despite the significant advances in understanding the etiology of PAH, the time from onset of symptoms to recognition of the disease has not improved over the past 2 decades, the delay of which results in progression of pathologic changes that are life threatening and irreversible when left untreated. This is particularly concerning in that the potential for optimal clinical outcomes and survival is diminished even after a 2-year delay in diagnosis.2 The aim of this article is to evaluate the current public health framework to highlight social determinants that influence and may result in barriers in achieving optimal health outcomes, with a focus on factors that may contribute to delayed diagnosis of PAH.3

Guest Editor's Memo

PAH: Looking for Zebras

A Change is Coming

Helping Patients Understand the Complex Pulmonary Hypertension Workup

Time to Diagnosis of Pulmonary Arterial Hypertension

Considering the Impacts of Health Disparities, Inequalities, and Inequities on Early Diagnosis of Pulmonary Arterial Hypertension

Screening for Pulmonary Arterial Hypertension

Importance of Early Diagnosis

Diagnosis of PAH From a Pulmonologist Perspective

Review of the Latest Published Research

Conference Abstracts

Guest Editor’s Memo

A Roundtable Discussion Addressing Patient-Centered Care and Quality of Life in Patients With Pulmonary Arterial Hypertension

Editor’s Memo

Pulmonary Hypertension Associated With Parenchymal Lung Disease