Abstract
Pulmonary veno-occlusive disease is refractory to medical treatment and is generally associated with a poor prognosis. Treatment with vasodilators, such as prostacyclin, of patients with PVOD is controversial because of concerns regarding hemodynamic deterioration. Although a preferential pulmonary vasodilatory effect of a specific phosphodiesterase-5 inhibitor, sildenafil, has recently been reported in patients with primary pulmonary hypertension, little information is available regarding the effect of sildenafil on patients with pulmonary veno-occlusive disease. In the present case, remarkable improvement of hemodynamics and of clinical course was produced by adjunctive use of oral sildenafil in association with intravenous high-dose epoprostenol. These findings suggest that sildenafil may be a therapeutic option in the medical treatment of pulmonary veno-occlusive disease.
MeSH terms
-
Adult
-
Antihypertensive Agents / pharmacology
-
Antihypertensive Agents / therapeutic use*
-
Drug Therapy, Combination
-
Dyspnea / drug therapy
-
Dyspnea / physiopathology
-
Epoprostenol / pharmacology
-
Epoprostenol / therapeutic use*
-
Humans
-
Lung / pathology
-
Male
-
Phosphodiesterase Inhibitors / pharmacology
-
Phosphodiesterase Inhibitors / therapeutic use*
-
Piperazines / pharmacology
-
Piperazines / therapeutic use*
-
Pulmonary Veno-Occlusive Disease / diagnosis
-
Pulmonary Veno-Occlusive Disease / drug therapy*
-
Pulmonary Veno-Occlusive Disease / physiopathology
-
Purines / pharmacology
-
Purines / therapeutic use
-
Sildenafil Citrate
-
Sulfones / pharmacology
-
Sulfones / therapeutic use*
-
Treatment Outcome
-
Vasodilator Agents / pharmacology
-
Vasodilator Agents / therapeutic use*
Substances
-
Antihypertensive Agents
-
Phosphodiesterase Inhibitors
-
Piperazines
-
Purines
-
Sulfones
-
Vasodilator Agents
-
Sildenafil Citrate
-
Epoprostenol