Chest
Clinical InvestigationsPulmonary Hypertension in Patients With Idiopathic Pulmonary Fibrosis
Section snippets
Study Population
This study was approved by the Mayo Foundation institutional review board. Using a computer-assisted search, 487 patients with IPF evaluated at Mayo Clinic Rochester during the period of January 1, 1994, to December 31, 1996, were identified.15 The clinical, radiologic, and physiologic features for this population have been published previously.15 Of these 487 patients, 136 patients (28%) underwent a comprehensive echocardiographic evaluation within 3 months of their initial visit at our
Clinical Characteristics of IPF Patients With PH
The clinical characteristics of the 88 patients are summarized in Table 1. The mean estimated SPAP for the entire group was 48 ± 16 mm Hg (range, 28 to 116 mm Hg). Evidence of PH (SPAP > 35 mm Hg at rest) was present in 74 patients (84%). Patients were stratified by SPAP into three subgroups: ≤ 35 mm Hg, 36 to 50 mm Hg, and > 50 mm Hg, and included 14 patients (16%), 47 patients (53%), and 27 patients (31%), respectively. The three groups were similar except for gender, NYHA functional status,
Discussion
This study describes echocardiographic characteristics and survival of patients with PH associated with IPF. Our data suggest that significant PH is not limited to patients with advanced IPF. SPAP correlated inversely with Dlco. The estimated survival from the time of IPF diagnosis of patients with SPAP > 50 mm Hg was substantially worse than that of patients with SPAP ≤ 50 mm Hg. PH is likely more common than currently suspected in patients with IPF and has prognostic and management
Conclusions
Our data demonstrate that SPAP correlates inversely with Dlco and PH has a significant adverse impact on survival, particularly when SPAP is > 50 mm Hg. Further studies are needed to define the prevalence of PH as well as management implications for patients with IPF.
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Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).
Dr. Chaowalit was supported by Siriraj Hospital, Mahidol University, Bangkok, Thailand.