Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension

doi:10.1378/chest.120.3.866

Chest

Volume 120, Issue 3, September 2001, Pages 866-872

https://doi.org/10.1378/chest.120.3.866 Get rights and content

Abstract

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·cm⁻⁵. 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.

Section snippets

Materials and Methods

We included all patients with a history of pulmonary sarcoidosis who were referred between 1996 and 2000 to the Pulmonary Hypertension Center at Rhode Island Hospital in Providence, RI, or the Adult Congenital Heart Clinic at Children’s Hospital in Boston, MA, and who underwent right-heart catheterization. Patients underwent catheterization if they had progressive symptoms of dyspnea on exertion and an estimated PA systolic pressure ≥ 40 mm Hg by echocardiography. The study protocol was

Baseline Characteristics

Characteristics of the eight enrolled patients are listed in Table 1. Mean age was 53 ± 4 years. All patients had a tissue diagnosis consistent with sarcoidosis, obtained an average of 19 ± 4 years before the right-heart catheterization, and all but one patient had radiologic stage IV sarcoidosis (Table 1). Results of pulmonary function studies (Table 2) showed at least mild restriction in all patients tested and significant airway obstruction in two patients (patients 4 and 5). Patients in

Discussion

Our findings support the hypothesis that patients with sarcoidosis-associated pulmonary hypertension often have substantial acute reversibility, even in the face of severe parenchymal disease and pulmonary dysfunction, reversibility that is preserved during long-term treatment with iNO. These patients also manifested functional improvement, albeit temporary, after initiation of long-term treatment with iNO.

Previous authors¹⁶ have surmised that the pulmonary hypertension that occurs with stage

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: This study was supported by National Heart, Lung, and Blood Institute grants HL-02613 (Dr. Klinger) and HL-45050 (Dr. Hill).

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Chest

Clinical InvestigationsSarcoidosisVasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension

Abstract

Objective

Methods

Results

Conclusion

Section snippets

Materials and Methods

Baseline Characteristics

Discussion

Chest

Am J Med Sci

Chest

Chest

J Biol Chem

Manifestations of sarcoidosis

Am J Med

Chronic cor pulmonale in pulmonary sarcoidosis

Thorax

Effects of corticosteroid treatment on pulmonary hemodynamics in patients with sarcoidosis

Eur Respir J

Pulmonary hemodynamics at rest and during exercise in patients with sarcoidosis

Respiration

Sarcoidosis

Am Rev Respir Dis

Clinical Investigations
Sarcoidosis
Vasoresponsiveness of Sarcoidosis-Associated Pulmonary Hypertension