Pulmonary hypertension
Pulmonary Hypertension in End-stage Pulmonary Sarcoidosis: Therapeutic Effect of Sildenafil?

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Background

The objectives of this study were to assess the frequency and severity of pulmonary hypertension (PH) and the effect of sildenafil treatment in patients with recalcitrant pulmonary sarcoidosis.

Methods

This investigation was a single-center, retrospective study of all patients (n = 25) with end-stage pulmonary sarcoidosis referred for lung transplantation. Hemodynamic measurements were evaluated by right-side cardiac catheterization in 24 of 25 patients. Sildenafil treatment for patients with sarcoidosis-associated PH was introduced in April 2004.

Results

The study group of 24 patients (16 men, 8 women) had a median age of 45 (range 35 to 58) years, and duration of sarcoidosis of 11 (range 2 to 38) years. Mean pulmonary arterial pressure (MPAP) was median 36 (range 18 to 73) mm Hg. PH (MPAP >25 mm Hg) was present in 19 of 24 patients (79%). Sildenafil was administered to 12 of 13 patients at a dose of 150 (range 75 to 225) mg/day for 4 (range 1 to 12) months. Sildenafil treatment was associated with reductions in MPAP of −8 mm Hg (CI −1 to −15 mm Hg), and PVR −4.9 Wood units (CI −7.2 to −2.6 Wood units). Cardiac output and cardiac index also increased during treatment (p = 0.01, respectively). There were no consistent changes in 6-minute walk distance.

Conclusions

Patients with severe pulmonary sarcoidosis have a high prevalence of PH. Sildenafil treatment was associated with significant improvements in hemodynamic parameters.

Section snippets

Methods

From January 1992 to December 2006, 25 patients with biopsy-verified advanced pulmonary sarcoidosis were evaluated at our National Centre for Lung Transplantation. All patients had been treated with repeated courses of corticosteroids, and 4 patients were receiving additional cytostatic therapy with azathioprine at the time of referral. Hemodynamic measurements obtained by right-side heart catheterization were available for 24 patients. No medications were withheld prior to the procedure.

All

Demographics

The data from the initial examinations of all 24 patients are shown in Table 1. The median time from diagnosis of pulmonary sarcoidosis to referral was 11 years, with a large variation from 2 to 38 years. All patients had a normal left ventricular ejection fraction as evaluated by transthoracic echocardiography. None of the patients had evidence of extrathoracic sarcoidosis. All but 3 patients were smokers, who, in addition to active smoking, were also exposed to passive smoking. Most patients

Discussion

The principle findings in this retrospective analysis of a relatively large series of patients with end-stage pulmonary sarcoidosis referred for lung transplantation are as follows: (1) PH was present in a high percentage (19 of 25) of these patients; (2) sildenafil treatment was associated with a reduction in pulmonary arterial pressures, a reduction in pulmonary vascular resistance, and a corresponding increase in cardiac output and cardiac index, and was well tolerated with no serious

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