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Adaani E. Frost, MD, FCCP
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From the Department of Medicine, Section of Pulmonary and Critical Care, Baylor College of Medicine.

Correspondence to: Adaani E. Frost, MD, FCCP, Baylor College of Medicine, 6620 Main S, Ste 1225, Houston TX 77030; e-mail: frost@bcm.tmc.edu


Financial/nonfinancial disclosures: The author has reported to CHEST the following conflicts of interest: Dr Frost has participated in the steering committee of the REVEAL Registry since its inception and has been reimbursed for her time in the development of the database, modifications in the database, and direction of the scientific questions and data analysis. This registry is supported by a grant from Actelion Pharmaceuticals US, Inc. She is on the speakers’ bureau and has received honoraria for speaking for several pulmonary hypertension-related pharmaceutical companies including Gilead, United Therapeutics, and Pfizer. She has also participated in and received honoraria for advisory boards for Bayer and Gilead. Baylor College of Medicine and Dr Frost as principal investigator have received funds for the conduct of institutional review board-approved multicenter national and international clinical research trials in pulmonary hypertension from Gilead, Pfizer, Lilly, United Therapeutics, Actelion, Novartis, and Bayer. She has also participated in advisory boards.

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):829-830. doi:10.1378/chest.11-0873
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To The Editor:

We appreciate the letter from Drs Barnett and De Marco regarding our recent article in CHEST,1 because it highlights several important points from this and other Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL Registry) articles. Drs Barnett and De Marco emphasize that patients with pulmonary arterial hypertension (PAH) should not be confused with patients with pulmonary venous hypertension related to unrecognized left-sided heart disease (LHD) and that such an erroneous diagnosis can have a major impact on the patients as well as on the database. We completely concur with these important points and agree with the comments that proven therapies for patients with LHD-associated pulmonary hypertension are lacking and that there is no proven role for PAH-specific therapies in treating these patients.

It is important to emphasize that those patients with a higher than traditional pulmonary capillary wedge pressure (PCWP) included in the REVEAL Registry were considered to have group 1 pulmonary hypertension (ie, PAH) by the enrolling physician. Although not mandated by the study protocol, it is assumed that the enrolling physician performed the necessary evaluations to ensure the diagnosis of PAH.

The design of the REVEAL Registry2 permitted the inclusion of such patients because:

  • 1. The level of PCWP allowed for definition of PAH has increased over time (PCWP was ≤12 mm Hg in the National Institutes of Health idiopathic PAH registry, not the more recent 15 mm Hg). This change has been driven by expert opinion and enrollment criteria for clinical studies, both of which strive to exclude patients with unrecognized LHD while maximizing enrollment of appropriate patients.

  • 2. Having a risk factor for LHD does not confer protection from development of true intrinsic pulmonary vascular disease (ie, PAH).

  • 3. Ventricular interdependence can result in elevated left-sided pressures (PCWP or left ventricular end-diastolic pressure) in the absence of LHD in a patient with severe PAH.

  • 4. Characterization of these patients and their treatment in clinical practice needs further exploration.

The emphasis of this article was on the comparison of historical and contemporary registries of PAH with the REVEAL Registry database. The size of the REVEAL Registry database permitted comparison of subgroups with enrollment characteristics identical to traditionally defined patients with PAH; the 1,072 patients in the REVEAL Registry National Institutes of Health Comparison Cohort subgroup had idiopathic PAH, familial PAH, and PCWP ≤12 mm Hg, whereas the 2,355 patients in the REVEAL Registry French Comparison Cohort had PCWP <15 mm Hg. The conclusions drawn in the article about increasing age, obesity, and increasing female preponderance in patients with PAH in the United States remain true in these subgroups.

Finally, the comments from Drs Barnett and De Marco provide a welcome introduction to our analysis of the characteristics, changes in hemodynamics over time, and outcomes of the patients with expanded hemodynamic criteria included in the REVEAL Registry. These data will be forthcoming shortly.

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]
 

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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]
 
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