Chest
Selected ReportsEffects of Continuous IV Prostacyclin in a Patient With Pulmonary Veno-occlusive Disease
Section snippets
Case Report
A 30-year-old man with New York Heart Association functional class IV was hospitalized with chief complaints of syncope and dyspnea, which had begun in November 1998. He had no medical or family history of note. He had smoked one pack of cigarettes per day for 10 years. He was noted to have jugular vein dilatation, a loud pulmonary component of S2, an S3 gallop, right ventricular lift, and hepatomegaly. Arterial blood gas analysis (room air) revealed a pH of 7.45, Po2 of 66.0 mm Hg, and Pco2 of
Discussion
Although PGI2 therapy has been established as a treatment for PPH, the use of this treatment for PVOD, a subtype of PPH, is controversial. There are theoretical reasons about why PGI2 may not be efficacious in patients with PVOD and why it may, in fact, worsen the cardiopulmonary status. If the pulmonary arterioles dilate but the resistance of the pulmonary veins remains fixed, an increase in transcapillary hydrostatic pressure may ensue and produce florid pulmonary edema.56 Thus, lung
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Cited by (51)
Use of vasodilators for the treatment of pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: A systematic review
2019, Respiratory InvestigationCitation Excerpt :In a relatively larger case series, the one-year survival was reported to be 83% and the two-year survival was 50% with intravenous epoprostenol therapy [19]. Improvement in the 6MWD was reported in seven reports (Table 1) [11,13,19,21,25,29,31]. Because all of studies were single case reports or case series, the 6MWD was evaluated at various times after the initiation of several different drugs with varying doses.
Pulmonary veno-occlusive disease
2018, Revue des Maladies RespiratoiresEarly Onset Noninfectious Pulmonary Syndromes after Hematopoietic Cell Transplantation
2017, Clinics in Chest MedicinePulmonary Capillary Hemangiomatosis and Pulmonary Veno-occlusive Disease
2016, Clinics in Chest MedicineCitation Excerpt :Initiation of these therapies frequently leads to increased transcapillary pressures and pulmonary edema following pulmonary arterial vasodilation and can result in rapid clinical deterioration or death. Nonetheless, there are case reports of clinical improvement with intravenous and inhaled prostacyclins,10,38–41 phosphodiesterase type-5 inhibitors (PDE5-I),42–44 and endothelin receptor antagonists.45 For this reason, experts have recommended cautious consideration of these therapies in patients with PVOD.38
Alveolar Hemorrhage and Rare Infiltrative Diseases
2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth EditionEpidemiology of pulmonary arterial hypertension
2013, Clinics in Chest MedicineCitation Excerpt :Differentiation from other forms of PAH is important because treatment with prostanoids or other agents that are effective for IPAH may produce pulmonary edema by increasing cardiac output to postcapillary vessels with obstructing lesions and death in a subset of patients with PVOD.158 In part because of these difficulties in treatment, the prognosis of PVOD is poor, with most patients dying within 2 years of diagnosis unless long-term treatment with prostanoids is tolerated.154,156,159 Immediate consideration of lung transplantation is thus encouraged when the diagnosis of PVOD is established, although recurrence of the condition after transplantation has been reported.160