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Clinical Investigations: SARCOIDOSIS |

Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension*

Ioana R. Preston, MD; James R. Klinger, MD, FCCP; Michael J. Landzberg, MD; Jeanne Houtchens, BSN; David Nelson, CRT; Nicholas S. Hill, MD, FCCP
Author and Funding Information

*From the Division of Pulmonary, Sleep, and Critical Care Medicine (Drs. Preston, Klinger, and Hill, Ms. Houtchens, and Mr. Nelson), Rhode Island Hospital and Brown University School of Medicine, Providence, RI; and Division of Pediatric Cardiology (Dr. Landzberg), Children’s Hospital and Harvard School of Medicine, Boston, MA.



Chest. 2001;120(3):866-872. doi:10.1378/chest.120.3.866
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Objective: To assess short-term and long-term responses to treatment with pulmonary vasodilators in patients with sarcoidosis-related pulmonary hypertension.

Methods: A prospective, observational study was performed on eight patients with moderate-to-severe sarcoidosis-related pulmonary hypertension. Patients underwent a short-term vasodilator trial, using inhaled nitric oxide (iNO), IV epoprostenol, and/or oral calcium-channel blockers. A favorable short-term response was considered a ≥ 20% decrease in pulmonary vascular resistance (PVR). Five patients received long-term treatment with iNO (with one patient receiving epoprostenol in addition) and underwent follow-up hemodynamic and/or 6-min walk testing. Two patients received long-term treatment with calcium-channel blockers.

Results: Baseline (± SE) mean pulmonary artery pressure (mPAP) was 55 ± 4 mm Hg and PVR was 896 ± 200 dyne·s·cm5. A favorable short-term response was seen in seven of eight patients receiving iNO, four of six patients receiving epoprostenol, and two of five patients receiving calcium-channel blockers. With iNO, PVR decreased 31 ± 5% (p = 0.006) and mPAP decreased 18 ± 4% (p = 0.003); with epoprostenol, PVR decreased 25 ± 6% (p = 0.016) and mPAP decreased 6 ± 2% (p = not significant). Decreased systemic vascular resistance was the only significant response to treatment with calcium-channel blockers. Follow-up 6-min walk test results improved in all five patients receiving long-term treatment with iNO. Follow-up hemodynamic responses in three patients showed preserved vasoresponsiveness. These three patients subsequently died, as did the two patients receiving calcium-channel blockers. The two remaining patients continue to receive iNO.

Conclusion: In the short term, pulmonary hypertension in patients with sarcoidosis is responsive to treatment with pulmonary vasodilators; these patients may benefit from long-term iNO therapy.

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