Chest
Volume 128, Issue 4, October 2005, Pages 2393-2399
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Clinical Investigations
Pulmonary Hypertension in Patients With Idiopathic Pulmonary Fibrosis

https://doi.org/10.1378/chest.128.4.2393Get rights and content

Study objectives

To determine the impact on survival and clinical correlates of pulmonary hypertension (PH) occurring in patients with idiopathic pulmonary fibrosis (IPF).

Design

Retrospective study.

Setting

Tertiary care, referral medical center.

Patients

Among 487 consecutive patients with IPF, we identified 136 patients who underwent transthoracic echocardiography within 3 months of their initial evaluation at our institution. Patients with left ventricular dysfunction, valvular heart disease, incomplete follow-up, and those in whom pulmonary artery pressures could not be assessed were excluded; the remaining 88 patients were included in this study. Correlations were performed between echocardiographic measures of PH and clinical variables including survival.

Measurements and results

The mean (± SD) estimated systolic pulmonary artery pressure (SPAP) for the 88 patients was 48 ± 16 mm Hg (range, 28 to 116 mm Hg). Among pulmonary function parameters, SPAP correlated best with diffusing capacity of the lung for carbon monoxide (Dlco), to which it was inversely related. For survival analysis, patients were stratified into three groups: ≤ 35 mm Hg (14 patients), 36 to 50 mm Hg (47 patients), and > 50 mm Hg (27 patients). Using the Kaplan-Meier method, the median survival rates for these three groups were 4.8 years, 4.1 years, and 0.7 years, respectively. Those patients with SPAP > 50 mm Hg had significantly worse survival compared to other subgroups (p = 0.009).

Conclusion

In patients with IPF, PH correlates inversely with Dlco and has a significant adverse impact on survival, particularly when SPAP is > 50 mm Hg.

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.

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