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. Genetic testing and professional genetic counseling should be offered to relatives of patients with familial pulmonary arterial hypertension (FPAH). Level of evidence: expert opinion; benefit: intermediate; grade of recommendation: E/A.
2. Patients with idiopathic pulmonary arterial hypertension (IPAH) should be advised about the availability of genetic testing and counseling for their relatives. Level of evidence: expert opinion; benefit: intermediate; grade of recommendation: E/A.
3. In patients with a suspicion of pulmonary arterial hypertension (PAH), electrocardiogram (ECG) should be performed to screen for a spectrum of cardiac anatomic and arrhythmic problems; it lacks sufficient sensitivity to serve as an effective screening tool for PAH, but contributes prognostic information in patients with known PAH. Quality of evidence: low; benefit: small/weak; strength of recommendation: C.
4. In patients with a suspicion of PAH, a chest radiograph (CXR) should be obtained to reveal features supportive of a diagnosis of PAH and to lead to diagnoses of underlying diseases. Quality of evidence: low; benefit: intermediate; strength of recommendation: C.
5. In patients with a clinical suspicion of PAH, Doppler echocardiography should be performed as a noninvasive screening test that can detect pulmonary hypertension (PH), though it may be imprecise in determining actual pressures compared to invasive evaluation in a portion of patients. Quality of evidence: fair; benefit: substantial; strength of recommendation: A.
6. In patients with a clinical suspicion of PAH, Doppler echocardiography should be performed to evaluate the level of right ventricular systolic pressure and to assess the presence of associated anatomic abnormalities such as right atrial enlargement, right ventricular enlargement, and pericardial effusion. Quality of evidence: expert opinion; benefit: intermediate; strength of recommendation: E/B.
7. In asymptomatic patients at high risk, Doppler echocardiography should be performed to detect elevated pulmonary arterial pressure. Quality of evidence: expert opinion; benefit: intermediate; strength of recommendation: E/B.
8. In patients with suspected or documented pulmonary hypertension (PH), Doppler echocardiography should be performed to look for left ventricular systolic and diastolic dysfunction, left-sided chamber enlargement, or valvular heart disease. Quality of evidence: good; benefit: substantial; strength of recommendation: A.
9. In patients with suspected or documented PH, Doppler echocardiography with contrast should be obtained to look for evidence of intracardiac shunting. Quality of evidence: fair; benefit: intermediate; strength of recommendation: B.
10. In patients with unexplained PAH, testing for connective tissue disease and human immunodeficiency virus (HIV) infection should be performed. Quality of evidence: expert opinion; benefit: intermediate; strength of recommendation: E/A.
11. In patients with PAH, ventilation/perfusion (V/Q) scanning should be performed to rule out chronic thromboembolic pulmonary hypertension (CTEPH); a normal scan result effectively excludes a diagnosis of CTEPH. Quality of evidence: low; benefit: substantial; strength of recommendation: B.
12. In patients with PAH, contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) should not be used to exclude the diagnosis of CTEPH. Quality of evidence: low; benefit: negative; strength of recommendation: D.
13. In patients with PAH and a ventilation/perfusion (V/Q) scan suggestive of CTEPH, pulmonary angiography is required for accurate diagnosis and best anatomic definition to assess operability. Quality of evidence: expert opinion; benefit: substantial; strength of recommendation: E/A.
14. In patients with PAH, testing of pulmonary function and arterial blood oxygenation should be performed to evaluate for the presence of lung disease. Quality of evidence: low; benefit: substantial; strength of recommendation: B.
15. In patients with systemic sclerosis, pulmonary function testing with diffusing capacity of the lung for carbon monoxide (DLCO) should be performed periodically (every 6 to 12 months) to improve detection of pulmonary vascular or interstitial disease. Quality of evidence: fair; benefit: intermediate; strength of recommendation: B.
16. In patients with PAH, lung biopsy is not routinely recommended because of the risk, except under circumstances in which a specific question can only be answered by tissue examination. Quality of evidence: expert opinion; benefit: substantial; strength of recommendation: E/A.
17. In patients with suspected PH, right-heart catheterization is required to confirm the presence of PH, establish the specific diagnosis, and determine the severity of PH. Quality of evidence: good; benefit: substantial; strength of recommendation: A.
18. In patients with suspected PH, right-heart catheterization is required to guide therapy. Quality of evidence: low; benefit: substantial; strength of recommendation: B.
19. In patients with PAH, serial determinations of functional class and exercise capacity assessed by the 6-minute walk test provide benchmarks for disease severity, response to therapy, and progression. Quality of evidence: good; benefit: intermediate; strength of recommendation: A.

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

A clinical algorithm is provided in the original guideline document for patient evaluation.

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: Michael McGoon, MD, May Clinic, Rochester, MN; David Gutterman, MD, FCCP, Medical College of Wisconsin, Madison, WI; Virginia Steen, MD, Georgetown University, Washington, DC; Robin Barst, MD, Columbia University College of Physicians and Surgeons, New York, NY; Douglas C. McCrory, MD, MHS, Center for Clinical Health Policy Research, Department of Medicine, Duke University Medical Center, Center for Health Services Research in Primary Care, Durham, NC; Terry A. Fortin, MD, Department of Medicine, Duke University Medical Center, Durham, NC; James E. Loyd, MD, FCCP, Division of Allergy, Pulmonary and Critical Care, Vanderbilt University School of Medicine, Nashville, TN

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 27, 2004.

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