This is the current release of the guideline.

Rating schemes for level of evidence, strength of recommendation, and net benefit follow the "Major Recommendations."

As the majority of the evidence reviewed in the original guideline document is applicable to patients with idiopathic pulmonary arterial hypertension (IPAH), the following recommendations pertain to patients with IPAH. In most instances, data are insufficient to make recommendations for patients with pulmonary arterial hypertension (PAH) due to diagnosis other than IPAH. In patients with IPAH, the following parameters, as assessed at baseline, may be used to predict a worse prognosis:

1. Advanced New York Heart Association functional class (NYHA-FC). Quality of evidence: good; net benefit: substantial; strength of recommendation: A.
2. Low 6 -minute walk test (6MWT) distance. Quality of evidence: good; net benefit: substantial; strength of recommendation: A.
3. Presence of a pericardial effusion. Quality of evidence: good; net benefit: substantial; strength of recommendation: A.
4. Elevated mean right atrial pressure (mRAP). Quality of evidence: fair; net benefit: substantial; strength of recommendation: A.
5. Reduced cardiac index (CI). Quality of evidence: fair; net benefit: substantial; strength of recommendation: A.
6. Elevated mean pulmonary arterial pressure (mPAP). Quality of evidence: fair; net benefit: intermediate; strength of recommendation: B.
7. Elevated Doppler Echocardiography right ventricular (RV) (Tei) index. Quality of evidence: low; net benefit: intermediate; strength of recommendation: C.
8. Low VO₂max (maximum oxygen consumption) and low peak exercise systolic blood pressure (SBP) and diastolic blood pressure (DBP) as determined by cardiopulmonary exercise test (CPET). Quality of evidence: low; net benefit: intermediate; strength of recommendation: C.
9. Electrocardiogram (ECG) findings of increased P-wave amplitude in lead II, qR pattern in lead V₁, and World Health Organization criteria for RV hypertrophy. Quality of evidence: low; net benefit: intermediate; strength of recommendation: C.
10. Elevated brain natriuretic peptide (BNP) (>180 pg/mL). Quality of evidence: low; net benefit: intermediate; strength of recommendation: C.
11. In patients with IPAH treated with epoprostenol, persistence of NYHA-FC III or IV status after at least 3 months of therapy may be used to predict a worse prognosis. Quality of evidence: fair; net benefit: substantial; strength of recommendation: A.
12. In patients with scleroderma-associated PAH, reduced diffusing capacity of the lung for carbon monoxide (DLCO) (<45% of predicted) may be used to predict a worse prognosis. Quality of evidence: low; net benefit: small/weak; strength of recommendation: C.
13. In pediatric patients with IPAH, younger age at diagnosis may be used to predict a worse prognosis. Quality of evidence: low; net benefit: small/weak; strength of recommendation: C.

Definitions

Quality of the Evidence

Good = evidence based on good randomized controlled trials or meta-analyses

Fair = evidence based on other controlled trials or randomized controlled trials with minor flaws

Low = evidence based on nonrandomized, case-control, or other observational studies

Expert opinion = evidence based on the consensus of the carefully selected panel of experts in the topic field. There are no studies that meet the criteria for inclusion in the literature review.

Strength of Recommendations

A = strong recommendation
B = moderate recommendation
C = weak recommendation
D = negative recommendation
I = no recommendation possible (inconclusive)
E/A = strong recommendation based on expert opinion only
E/B = moderate recommendation based on expert opinion only
E/C = weak recommendation based on expert opinion only
E/D = negative recommendation based on expert opinion only

Net Benefit

Substantial
Intermediate
Small/weak
None
Conflicting
Negative

None provided

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").

Not applicable: The guideline was not adapted from another source.

2004 Jul

American College of Chest Physicians - Medical Specialty Society

Funding for both the evidence reviews and guideline development was provided through an unrestricted educational grant from GlaxoSmithKline, Texas Biotechnology Corporation, and Actelion Pharmaceuticals US. Representatives from these companies were not granted right of review, nor were they allowed participation in any portion of the guideline development.

American College of Chest Physicians (ACCP) Expert Panel on Pulmonary Artery Hypertension

Primary Authors: Vallerie V. McLaughlin, MD, FCCP, Rush Presbyterian St. Luke's Hospital, Chicago, IL; Kenneth W. Presberg, MD, FCCP, Medical College of Wisconsin, Milwaukee, WI; Ramona L. Doyle, MD, FCCP, Stanford University, Palo Alto, CA; Steven H. Abman, MD, Children's Hospital, Denver, CO; Douglas C. McCrory, MD, MHSc, Duke University Medical Center, Durham, NC; Terry Fortin, MD, Duke University Medical Center, Durham, NC; Gregory Ahearn, MD, Duke University Medical Center, Durham, NC

The following participants have disclosed information regarding potential or real conflicts of interest and commitment:

Steven H. Abman, MD: scientific advisory board for INO Therapeutics; consultant for Pfizer.

Charles W. Atwood, Jr., MD, FCCP: research support from Respironics, Inc.

David B. Badesch, MD, FCCP: consultant or Speaker's Bureau for Glaxo Wellcome/GlaxoSmithKline, Actelion, InterMune, Encysive, Myogen, Astra-Merck, Astra-Zeneca, Exhale Therapeutics/CoTherix, Forrest Labs, INO Therapeutics, Berlex; research support from Glaxo Wellcome/GlaxoSmithKline, United Therapeutics, Boehringer Ingelheim, Actelion, Encysive, ICOS/Texas Biotechnologies/Encysive, Myogen, INO Therapeutics, Scleroderma Foundation, National Institutes of Health, National Heart, Lung, and Blood Institute, United Therapeutics, Pfizer, American Lung Association.

Robyn J. Barst, MD: consultant and research support from Actelion, Encysive, Exhale Therapeutics, INO, Myogen, United Therapeutics, Pfizer GlaxoSmithKline; unrestricted education grants from GlaxoSmithKline, Encysive, Actelion.

Richard N. Channick, MD, FCCP: research support from Actelion, Pfizer, Myogen, United Therapeutics; consultant and Speaker’s Bureau for Actelion.

Ramona L. Doyle, MD, FCCP: Speaker's Bureau for Actelion; clinical research for Actelion, Myogen, United Therapeutics.

David D. Gutterman, MD, FCCP: stock options with Johnson & Johnson; relative who is a Vice-President at GlaxoSmithKline.

James E. Loyd, MD, FCCP: relationships with GlaxoSmithKline, United Therapeutics, Actelion, ICOS/Texas Biotechnology, Westat, PRA International, Pfizer, Exhale Therapeutics.

Michael D. McGoon, MD: past research support from Glaxo Wellcome, United Therapeutics, Actelion; research support from Texas Biotech/Encysive, Myogen, Pfizer, Medtronic.

Vallerie V. McLaughlin, MD, FCCP: consultant for Actelion, United Therapeutics, Exhale Therapeutics; Speaker's Bureau for Actelion; research funding from Actelion, United Therapeutics, Pfizer, Encysive/Texas Biotechnologies, Glaxo Wellcome, Exhale Therapeutics, Myogen.

Stuart Rich, MD: research funding from Actelion, Pfizer, United Therapeutics, Encysive, Myogen; consultant for Actelion, Pfizer, United Therapeutics, GlaxoSmithKline.

Lewis J. Rubin, MD, FCCP: consultant for Actelion, Myogen, Schering, Exhale Therapeutics, United Therapeutics, Pfizer, Celgene; investigator for Actelion, Myogen, Exhale, Pfizer, Celgene; no stock holdings or other ownerships or positions.

Gerald Simonneau, MD: consultant and investigator for Glaxo Wellcome, Pfizer, Actelion, Schering, Myogen, United Therapeutics.

Virginia D. Steen, MD: relationships with Arthritis Foundation, Scleroderma Foundation, Actelion.

Fredrick M. Wigley, MD: research funding from Biogen, Pfizer, Actelion; consultant to Genzyme.

This is the current release of the guideline.

This NGC summary was completed by ECRI on August 30, 2004.

This NGC summary is based on the original guideline, which is subject to the guideline developer's copyright restrictions.