This is the current release of the guideline.

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

1. In select patients with pulmonary arterial hypertension (PAH) unresponsive to medical management, atrial septostomy (AS) should be considered. Quality of evidence: low; net benefit: intermediate; strength of recommendation: C.
2. In patients with PAH, AS should be performed only at institutions with significant procedural and clinical experience. Quality of evidence: expert opinion; net benefit: substantial; strength of recommendation: E/A.
3. Patients with suspected chronic thromboembolic pulmonary hypertension (CTEPH) should be referred to centers experienced in the procedure for consideration of pulmonary thromboendarterectomy (PTE). Level of evidence: expert opinion; benefit: substantial; grade of recommendation: E/A.
4. In patients with operable CTEPH, PTE is the treatment of choice for improved hemodynamics, functional status, and survival. Level of evidence: low; benefit: substantial; grade of recommendation: B.
5. In patients with CTEPH deemed inoperable or with significant residual postoperative pulmonary hypertension (PH), balloon dilation, PAH medical therapy, or lung transplantation (LT) may be considered. Level of evidence: low; benefit: small/weak; grade of recommendation: C.
6. PAH patients with New York Heart Association (NYHA) functional class III and IV symptoms should be referred to a transplant center for evaluation and listing for LT or heart-lung transplantation (HLT). Level of evidence: low; benefit: substantial; grade of recommendation: B.
7. Listed patients with PAH whose prognosis remains poor despite medical therapy should undergo LT or HLT. Level of evidence: fair; benefit: substantial; grade of recommendation: A.
8. In patients with PAH who are undergoing transplantation, the procedure of choice is bilateral lung transplantation (BLT). Level of evidence: low; benefit: intermediate; grade of recommendation: C.
9. In children with PAH who are undergoing transplantation, the procedure of choice is BLT. Level of evidence: low; benefit: substantial; grade of recommendation: B.
10. In adult patients with PAH and simple congenital heart lesions, BLT with repair of the cardiac defect is the procedure of choice. Level of evidence: low; benefit: intermediate; grade of recommendation: C.
11. In adult patients with PAH and complex congenital heart disease who are undergoing transplantation, HLT is the procedure of choice. Level of evidence: low; benefit: substantial; grade of recommendation: B.

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: Ramona L. Doyle, MD, FCCP, Stanford University, Palo Alto, CA; Douglas McCrory, MD, MHSc, Duke University Medical Center, Durham, NC; Richard N. Channick, MD, FCCP, University of California-San Diego; Gerald Simonneau, MD, Hospital Antoine Beclere, Clamart, France; John Conte, MD, FCCP, Johns Hopkins University, Baltimore, MD

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.

None available

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.