Original clinical scienceCarbon monoxide diffusing capacity and mortality in pulmonary arterial hypertension
Section snippets
Methods
We studied patients in the Pulmonary Hypertension Connection (PHC) registry, which was initiated in March 2004, as described elsewhere.19 All patients evaluated at a single practice over time at three different university hospitals (University of Illinois at Chicago, Rush University Medical Center, and University of Chicago) between 1982 and 2006 were entered into the registry. Patients were entered retrospectively from 1982 to February 2004 and prospectively from March 2004 through 2006.
Results
The mean age of patients upon entry into the study was 48 ± 14 years and 77% were female. The cohort was divided into tertiles: Tertile I—DLCO <43% (range 11% to 42.5%) of predicted; Tertile II—DLCO 43% to 64% of predicted; and Tertile III—DLCO >64% (range 65% to 120%) of predicted. Table 1 lists differences in demographic, clinical, laboratory, exercise testing, and hemodynamic data by tertile of DLCO.
Reduced DLCO was associated with increased age, CTD etiology, poor functional class, oxygen
Discussion
In our study, the lowest tertile of DLCO was independently associated with death. Advantages of our study include the large number of patients with PAH and the comprehensive clinical characterization of the study subjects. To our knowledge, ours is the first study to associate reduction in DLCO with mortality in patients with PAH. Our finding of an independent association between the lowest tertile of DLCO (<43% of predicted) and death is consistent with studies of idiopathic pulmonary fibrosis
Disclosure Statement
The authors thank Tobias Perrino for help with data collection and validation of clinical data. We also thank Virginia Steen, MD, for advice and critical review during the early stages of this manuscript.
Dr. Gomberg-Maitland has received research grant support from Actelion, Gilead, Lilly ICOS, Pfizer, and United Therapeutics. She has served as a consultant for Biomarin, Gilead, Medtronic, and Pfizer.
Dr Chandra, Dr Archer, Dr Thenappan, and Dr Rich have no conflicts of interest to disclose.
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Cited by (0)
Supported by the American Heart Association, Actelion Pharmaceuticals and the Doris Duke Charitable Foundation.
S.C. and S.J.S. contributed equally to this study.