CHEST: Severe PAH May Be Undertreated



By Todd Neale, Senior Staff Writer, MedPage Today
Published: October 27, 2011
Reviewed by Robert Jasmer, MD; Associate Clinical Professor of Medicine, University of California, San Francisco and
Dorothy Caputo, MA, RN, BC-ADM, CDE, Nurse Planner

HONOLULU -- Many of the sickest patients with pulmonary arterial hypertension may not be receiving recommended treatment, researchers found.

Of nearly 800 such patients who died, most (57%) were not receiving parenteral prostanoids at the time of death, Harrison Farber, MD, of Boston University, reported at the CHEST meeting here.

Most current guidelines recommend either IV or subcutaneous prostanoids -- including the prostacyclins and prostaglandins -- for patients with the most severe pulmonary hypertension.

Although there are several unknown factors that might help explain why parenteral prostanoids were not being used, Farber said the study highlights the fact that clinicians can do better.
Action Points
  • Note that this study was published as an abstract and presented at a conference. These data and conclusions should be considered to be preliminary until published in a peer-reviewed journal.

  • Note that many of the sickest patients with pulmonary arterial hypertension may not be receiving recommended treatment with parenteral prostanoids.

  • Note that the highest rate parenteral prostanoid use was found in patients with functional class IV disease who had been assessed no longer than six months before death.

"No matter how we try and explain away these data, ultimately there is a percentage or a subset of those patients who would have gone on [parenteral prostanoids] if it had been proposed to them," he said.

"All of us like to think we're doing what we can that's best for our patients ... but I think that these data make you open your eyes and wonder if we actually are doing what we think we're doing."

There was some evidence in the literature, and in Farber's practice, that patients with severe pulmonary arterial hypertension were not receiving parenteral prostanoids, which led Farber and his colleagues to explore the issue using the large Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

The current analysis included 793 adult patients with pulmonary arterial hypertension who died. Most (61%) had a New York Heart Association/World Health Organization (NYHA/WHO) functional class assessment no more than six months before death.

The researchers looked at the medications the patients were taking at the time of death, including phosphodiesterase type-5 inhibitors, endothelin receptor antagonists, prostacyclin (either parenteral or oral/inhaled), or other drugs.

Overall, 8.4% were not taking any medications, 35.3% were on monotherapy, and 56.1% were on combination therapy.

Only 43% of patients were receiving IV or subcutaneous prostacyclin either alone or in combination with other drugs.

That rate was about 40% for patients with NYHA/WHO functional class III disease, regardless of how long before death the assessment was performed. It was 46.3% for class IV patients who were assessed more than six months before dying and 60.1% for class IV patients assessed six months or less before dying.

Farber acknowledged that there were several unanswered questions regarding:

  • The number of patients who stopped taking all medications when they entered hospice care
  • The number who refused parenteral prostanoids
  • Those who were in nursing homes
  • Those who were treated at tertiary care centers versus community settings
  • Those who tried parenteral prostanoids but stopped,
  • Those who died of causes other than the underlying disease


All of these could influence the results.

But, he said, none of those factors -- or the inclusion of the 13.9% of patients who were taking inhaled or oral prostanoids -- would entirely account for the 57% of patients who were not receiving IV or subcutaneous prostanoids at the time of death.

The study "suggests that there's a large number of patients who within six months of their death are not seeing what we consider to be optimal therapy," he said.

Farber reported relationships with Actelion, Gilead, and United Therapeutics.

One of his co-authors reported relationships with Actelion, Gilead, Lung Rx, GlaxoSmithKline, and Medtronic.

One of the authors is employed by ICON Late Phase & Outcomes Research, a company that receives research support from Actelion and other pharmaceutical companies.

Primary source: CHEST 2011
Source reference:
Farber H, et al "Use of parenteral prostanoids at time of death in patients with pulmonary arterial hypertension enrolled in REVEAL" CHEST 2011; Abstract 903A.
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