Although the management of pulmonary arterial hypertension (PAH) has changed dramatically thanks to advances in therapy, the disease continues to be progressive and often fatal. In 2004, the American College of Chest Physicians (ACCP) published its most recent guidelines for diagnosing and managing PAH. After these guidelines were released, the Quality Enhancement Research Initiative (QuERI) was established to help clinicians implement ACCP guideline-based approaches to the diagnosis and management of PAH. While updated guidelines have emerged, the recommended tests for diagnostic assessments of PAH have not differed significantly since 2004.

“There’s a need for better understanding of the management, treatment, and progression of PAH,” says Vallerie V. McLaughlin, MD. “People with PAH are a diverse patient group, leading to considerable variation in how physicians evaluate and treat the condition. It’s important to identify areas of care where improvement is required. The PAH-QuERI initiative is intended to help physicians optimize use of the ACCP guidelines and deliver evidence-based care in real-life healthcare settings.”

Diagnostic Tests for PAH Largely Underutilized

In an issue of Chest, Dr. McLaughlin and colleagues evaluated clinician adherence to the ACCP guidelines when managing patients enrolled in PAH-QuERI. Participating physicians recorded data on diagnostic work-up, disease management, and outcomes of PAH patients. Queries were generated automatically following pre-scheduled follow-up visits if the tests recommended by ACCP were not performed at least once.

“Certain essential and recommended diagnostic tests appear to be underutilized despite the availability of detailed guidelines and reminders.”

Vallerie V. McLaughlin, MD

At the beginning of the study period, all of the ACCP-recommended tests had been performed in only 6% of PAH patients. The automated program generated 1,530 reminders for 642 patients in the study who had validated enrollment data. Despite the availability of guidelines from the ACCP and at least one automatic reminder to most providers, the use of recommended diagnostic tests for PAH was inadequate, Dr. McLaughlin says (Table 1). “Our findings show that certain essential and recommended diagnostic tests appear to be underutilized despite the availability of detailed guidelines and reminders.”

ore specifically, the study found that missing diagnostic tests ranged from right heart catheterization, which is required for a diagnosis of PAH, to relatively simple tests, such as HIV and connective tissue disease screening. Ventilation/perfusion scans are recommended to exclude chronic thromboembolic pulmonary hypertension, but data demonstrated that these scans were not conducted in 43% of patients. Furthermore, several risk factors were shown to increase the likelihood of death within 3 years (Table 2).

A Treatment Disconnect of PAH Patients

The study by Dr. McLaughlin and colleagues also found that 78% of PAH patients were on disease-specific therapy, and use of these therapies tended to increase among patients with greater functional disability. Use of diuretics and warfarin was lower than anticipated, and calcium channel blockers (CCBs) were used in many patients. “What’s troubling is that only a fraction of patients who received CCBs had a positive response to acute vasodilator challenge,” says Dr. McLaughlin. “Only 7% of patients who received CCBs specifically for PAH had met the current guideline for acute vasoreactivity. The low rate of vasoreactivity testing in these patients is concerning, and inappropriate use of CCB therapy can worsen outcomes.”

When CCBs are inappropriately used in patients with PAH, clinicians may delay initiation of other, more effective PAH-specific therapies. Practice guidelines recommend aggressive treatment for PAH patients with more severe disease, including the use of prostacyclin analogues. In the study by Dr. McLaughlin and colleagues, prostacyclin analogues were not used in some patients who were candidates for them. “There may be several reasons for this,” Dr. McLaughlin says, “including issues relating to the complicated delivery of these therapies. Regardless, these findings raise concerns that there are substantial gaps in how patients with more advanced PAH are managed.”

Changing Physician Behaviors

To change the observed trends in the management of patients with PAH, Dr. McLaughlin says it will require modifying physicians’ behavior, which is no easy task. “Our study demonstrates how difficult it is to change current practices despite having evidence-based guidelines,” she says. “Programs that are stricter about using diagnostic tool sets may optimize our ability to diagnose this complex disease earlier. Using tools like standing orders has improved compliance with guideline-recommended therapy and patient outcomes in other areas of cardiovascular disease. Increasing physician education efforts and implementing similar tools for PAH may improve compliance with guidelines and outcomes in this patient population.”

Click here to view or download (pdf) the full guidelines, Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines.


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