Chest
Volume 138, Issue 5, November 2010, Pages 1078-1085
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Original Research
Pulmonary Hypertension
Survival in Sarcoidosis-Associated Pulmonary Hypertension: The Importance of Hemodynamic Evaluation

https://doi.org/10.1378/chest.09-2002Get rights and content

Objective

Pulmonary hypertension (PH) has been associated with increased mortality in patients with advanced pulmonary sarcoidosis. Sarcoidosis-associated PH may be the result of left ventricular dysfunction (LVD) or isolated pulmonary vasculature abnormality. Our objective was to determine if the cause of PH affects survival in patients with sarcoidosis with persistent dyspnea.

Methods

Patients with sarcoidosis with persistent dyspnea despite immunosuppressive therapy underwent right-sided heart catheterization. Patients with a pulmonary artery occluding pressure (PAO) ≥ 15 mm Hg were defined as having LVD. Patients were classified based on hemodynamics as no PH (pulmonary artery mean pressure [PAmean] < 25 mm Hg), PH without LVD (PAmean ≥ 25 mm Hg and PAO < 15 mm Hg), and PH with LVD (PH/LVD) (PAmean ≥ 25 mm Hg and PAO ≥ 15 mm Hg).

Results

One hundred thirty patients were studied at one institution: 50 (38.5%) patients had PH without LVD, whereas 20 (15.4%) had PH/LVD. All patients had their diagnostic procedure at least 18 months prior to analysis. The hazard ratio (HR) for death in PH without LVD vs no PH was 10.39 (95% CI, 2.99–13.78; P < .0001). The HR for dying for PH without LVD vs PH/LVD was 3.14 (95% CI, 1.01–5.62; P < .05). The presence of stage 4 chest roentgenograms and the need for supplemental oxygen were different between the groups. In a Cox proportional hazards model, independent predictors for survival were stage 4 chest roentgenogram (P < .005) and hemodynamic group (P < .02).

Conclusion

PH without LVD was associated with increased mortality. Proper characterization of patients required hemodynamic evaluation, as 29% of sarcoidosis-associated PH was due to LVD.

Section snippets

Materials and Methods

Patients at the University of Cincinnati Sarcoidosis and Interstitial Lung Disease Clinic were eligible for this study. All patients in this clinic are entered into a database and information regarding right-sided heart catheterization has been collected prospectively for the past 7 years. All patients eligible for this study were diagnosed with sarcoidosis based on standard criteria,17 and they underwent right-sided heart catheterization for persistent moderate-to-severe dyspnea and at least 6

Results

One hundred thirty patients with sarcoidosis underwent right-sided heart catheterization, revealing 50 (38.5%) patients with PH without LVD and 20 (15.4%) patients with PH/LVD. Figure 1 summarizes the outcome of the catheterizations, including the survival of patients. At the end of the study, all patients were either accounted for by clinic visits or had died.

Table 1 summarizes the characteristics of the three groups studied. No race, age, or gender differences were identified among the three

Discussion

For patients with sarcoidosis, the rate of PH has been reported to be between 5% and 15%.3, 5 In studies of patients with dyspnea, the percentage of patients with PH has been reported as > 50%.2, 4 The current study evaluated right-sided heart catheterization findings in 130 patients with sarcoidosis with persistent moderate to severe dyspnea despite antiinflammatory therapy for sarcoidosis. We did not have a standard protocol for referring patients for right-sided heart catheterization. We did

Acknowledgments

Author contributions: Dr Baughman: contributed to the design of the study, the care of patients, obtaining data on patients, and writing, reading, and approving the manuscript.

Dr Engel: contributed to the design of the study, the care of patients, obtaining data on patients, and writing, reading, and approving the manuscript.

Ms Taylor: contributed to the care of patients, obtaining data on patients, and reading and approving the manuscript.

Dr Lower: contributed to the design of the study, the

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