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