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Can Pulmonary Arterial Hypertension Be Diagnosed by an Elevated Pulmonary Capillary Wedge Pressure Outside of the Guideline Criteria?Pulmonary Arterial Hypertension Diagnosis FREE TO VIEW

Christopher F. Barnett, MD, MPH; Teresa De Marco, MD
Author and Funding Information

From the University of California San Francisco (Drs Barnett and De Marco); the Division of Cardiology, San Francisco General Hospital (Dr Barnett); and the Heart Failure and Pulmonary Hypertension Program, Division of Cardiology, University of California San Francisco Medical Center (Dr De Marco).

Correspondence to: Christopher F. Barnett, MD, MPH, San Francisco Hospital, Division of Cardiology, 1001 Potrero Ave, Room 5G1, San Francisco, CA 94110; e-mail: barnettc@medsfgh.ucsf.edu


Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr De Marco has received research support from Novartis Pharmaceuticals. She is a consultant for Gilead Sciences, Inc, and Actelion and has served on speakers bureaus and received honoraria from Gilead, Actelion, and United Therapeutics. Dr Barnett has reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;140(3):828-829. doi:10.1378/chest.11-0616
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To the Editor:

We read with interest the report by Frost and colleagues1 in a recent issue of CHEST (July 2011) comparing the results of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) with the other registries of pulmonary arterial hypertension (PAH). We are troubled by the inclusion of patients with a pulmonary capillary wedge pressure (PCWP) > 15 mm Hg, a value > 2 SD higher than normal, in this most recent and other publications from the REVEAL Registry.2

Since the 1979 National Institutes of Health registry, a PCWP ≤ 15 mm Hg has been accepted as a requirement for diagnosing PAH by professional societies and for entrance into clinical trials of PAH.3 Of 2,967 subjects in the REVEAL Registry, 239 (8%) had a PCWP > 15 mm Hg, suggesting either a proper diagnosis of pulmonary venous hypertension from unrecognized left-sided heart disease (LHD) or coexistent PAH and LHD. Obesity, female sex, hypertension, sleep apnea, and renal insufficiency, characteristics associated with left-sided heart disease, particularly heart failure with preserved ejection fraction, were more common among subjects with a PCWP > 15 mm Hg.4 The primary disease process in these patients may be quite different from that in patients with PAH. Including this heterogeneous population in REVEAL Registry analyses may confound the true picture of PAH.

Risks of inaccurate diagnosis include psychologic stress and adverse effects of therapy. Risks may be higher in patients treated with PAH-specific therapy who have unrecognized LHD. In patients with heart failure with reduced ejection fraction, a trial of epoprostenol was stopped early because of a trend toward increased mortality. Studies of bosentan, enrasentan, tezosentan, and darusentan in heart failure with reduced ejection fraction showed increased transaminases, fluid retention, and hospitalization for heart failure in the treatment arms.5 Adequate trials to assess the safety of sildenafil in LHD have not yet been performed. Unrecognized and untreated LHD could result in further preventable morbidity and mortality. Well-designed and executed studies of patients with characteristics of PAH but PCWP > 15 mm Hg are mandatory before the accepted definition of PAH is modified and patients who do not meet these criteria are treated with PAH-specific therapy.

Frost AE, Badesch DB, Barst RJ, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US contemporary registries. Chest. 2011;1391:128-137 [CrossRef] [PubMed]
 
McGoon MD, Krichman A, Farber HW, et al. Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc. 2008;838:923-931 [PubMed]
 
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54suppl 1:S43-S54 [CrossRef] [PubMed]
 
Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;1372:376-387 [CrossRef] [PubMed]
 
Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med. 2007;281:233-241 [CrossRef] [PubMed]
 

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References

Frost AE, Badesch DB, Barst RJ, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US contemporary registries. Chest. 2011;1391:128-137 [CrossRef] [PubMed]
 
McGoon MD, Krichman A, Farber HW, et al. Design of the REVEAL registry for US patients with pulmonary arterial hypertension. Mayo Clin Proc. 2008;838:923-931 [PubMed]
 
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54suppl 1:S43-S54 [CrossRef] [PubMed]
 
Badesch DB, Raskob GE, Elliott CG, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;1372:376-387 [CrossRef] [PubMed]
 
Oudiz RJ. Pulmonary hypertension associated with left-sided heart disease. Clin Chest Med. 2007;281:233-241 [CrossRef] [PubMed]
 
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