Abstract | BACKGROUND: 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.
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Authors | Nils Milman, Christopher M Burton, Martin Iversen, Regitze Videbaek, Claus V Jensen, Jørn Carlsen |
Journal | The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
(J Heart Lung Transplant)
Vol. 27
Issue 3
Pg. 329-34
(Mar 2008)
ISSN: 1557-3117 [Electronic] United States |
PMID | 18342757
(Publication Type: Journal Article)
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Chemical References |
- Piperazines
- Purines
- Sulfones
- Vasodilator Agents
- Sildenafil Citrate
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Topics |
- Adult
- Blood Pressure
(drug effects, physiology)
- Cardiac Output
(drug effects, physiology)
- Dose-Response Relationship, Drug
- Female
- Humans
- Hypertension, Pulmonary
(drug therapy, etiology, physiopathology)
- Male
- Middle Aged
- Piperazines
(therapeutic use)
- Prevalence
- Purines
(therapeutic use)
- Retrospective Studies
- Sarcoidosis, Pulmonary
(complications)
- Severity of Illness Index
- Sildenafil Citrate
- Sulfones
(therapeutic use)
- Treatment Outcome
- Vasodilator Agents
(therapeutic use)
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