27 Jun Pulmonary Arterial Hypertension Guidelines Discussed

MedicalResearch.com Interview with:
James R. Klinger, MD, FCCP
Professor of Medicine, Brown University
Darren Taichman, MD, PhD, FCCP
Adjunct Associate Professor of Medicine, University of Pennsylvania
Greg Elliott, MD, MACP, FCCP
Professor of Medicine, University of Utah School of Medicine

Background: The authors discuss the June 17, 2014 CHEST publication:

Pharmacological Therapy for Pulmonary Arterial Hypertension in Adults: CHEST Guideline

MedicalResearch: How widespread is the problem of Pulmonary Arterial Hypertension (PAH)?

Greg Elliott: PAH is an uncommon, but important disorder because untreated it is usually progressive and fatal, AND there are effective treatments. This makes the guidelines important for physicians, patients and insurers.

MedicalResearch: What are the most common causes of PAH?

Greg Elliott: Common causes are connective tissues diseases like scleroderma, toxins like methamphetamine and cases for which the cause is not known, i.e. idiopathic. Some (about 20 %) idiopathic cases are caused by gene mutations.

MedicalResearch: What are the main treatment modalities?

James Klinger: Presently available treatments aim to reverse some of the imbalances in the amounts of natural substance in the body the cause relaxation or constriction of pulmonary blood vessels.

Most PAH patients have decreased levels of prostacyclins and cGMP, two substances that help to decrease pulmonary artery pressure and increase blood flow through the lungs. At the same time, PAH patients have increased levels of endothelin, a small protein that increase pulmonary artery pressure and makes it more difficult for the right heart to pump blood through the lungs.

Presently available treatments include 3 general classes of drugs:

1) Prostacyclin derivatives that increase prostacyclin levels,

2) Phosphodiesterase inhibitors and soluble guanylate cyclase stimulators that increase cGMP levels in the lung, and

3) Endothelin receptor blockers that blunt the pulmonary hypertensive effects of endothelin.

MedicalResearch: How will the new guidelines for PAH treatment influence current medical practice?

James Klinger: The new treatment guidelines summarize the growing body of literature regarding the efficacy of the presently available medications for Pulmonary Arterial Hypertension and provide practitioners with a general guide to determine which patients are best suited for each type of drug.  The guidelines will facilitate the medical management of PAH by providing the health care practitioner with an evidence-based approach to proper drug selection.

MedicalResearch: Are there any special PAH situations or treatments you wish to highlight?

Darren Taichman: PAH (Pulmonary Arterial Hypertension) is not the same as PH (pulmonary hypertension). PAH is a specific form of PH, and requires a cardiac catheterization among other tests to diagnose correctly, and with the only very specific and also “special” situation of CTEPH (Chronic Thromboembolic Pulmonary Hypertension), is the only form of pulmonary hypertension for which the drug therapy addressed in this guideline is appropriate.

Citation:

Pharmacological Therapy for Pulmonary Arterial Hypertension in Adults: CHEST Guideline

Darren B. Taichman, MD, PhD, FCCP (Chair); Joe Ornelas, MS; Lorinda Chung, MD, MS; James Klinger, MD, FCCP; Sandra Lewis, PhD; Jess Mandel, MD; Harold Palevsky, MD, FCCP; Stuart Rich, MD, FCCP; Namita Sood, MD, FCCP; Erika B. Rosenzweig, MD; Terence K. Trow, MD, FCCP; Rex Yung, MD, FCCP (GOC Liaison); C. Gregory Elliott, MD, FCCP; David Badesch, MD, FCCP

Last Updated on June 27, 2014 by Marie Benz MD FAAD