Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension.
ABSTRACT To describe the long-term clinical efficacy of inhaled iloprost as first-line vasodilator mono-therapy in patients with idiopathic pulmonary arterial hypertension (IPAH).
Seventy-six IPAH patients were prospectively identified and treated with inhaled iloprost. Clinical, haemodynamic, and exercise parameters were obtained at baseline, after 3 and 12 months of therapy and yearly thereafter. Four endpoints were prospectively defined as follows: (i) death, (ii) transplantation, (iii) switch to intravenous (i.v.) therapy, or (iv) addition of or switch to other active oral therapy. During follow-up (535+/-61 days), 11 patients died, six were transplanted, 25 were switched to i.v. prostanoids, 16 received additional or other oral therapy, and 12 patients discontinued iloprost inhalation for other reasons. Event-free survival at 3, 12, 24, 36, 48, and 60 months was 81, 53, 29, 20, 17 and 13%, respectively. Among haemodynamic and exercise parameters, mixed venous oxygen saturation (P<0.001), right atrial pressure (P<0.001), and peak oxygen uptake (P=0.002) were associated with event-free survival.
In this study, only a minority of patients could be stabilized with inhaled iloprost mono-therapy during a follow-up period of up to 5 years. In the presence of multiple treatment options, chronic iloprost inhalation as mono-therapy appears to have a limited role.
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ABSTRACT: Pulmonary artery hypertension (PAH) is a pathological condition of small pulmonary arteries, characterised by vascular proliferation and remodelling. The pulmonary artery pressure and pulmonary vascular resistance progressively rise, leading to right heart failure and death. Pulmonary artery hypertension may be secondary to various conditions, or it may be idiopathic where no underlying cause is identifiable. Earlier, only symptomatic treatment was available for such patients which did not change the natural history of the disease. However, over the years, improvement in understanding the pathogenesis has resulted in the development of targeted approaches to the treatment of PAH. Survival advantage has also been shown with some of the pharmacologic agents. This review article discusses the current management strategy for PAH with special emphasis on an idiopathic variety, in an Indian context.Indian Heart Journal 01/2012; 64(1):60-73. DOI:10.1016/S0019-4832(12)60013-3 · 0.17 Impact Factor
Article: [Prostaglandins and prostacyclin].[Show abstract] [Hide abstract]
ABSTRACT: Prostacyclin was the first effective therapy for patients with pulmonary arterial hypertension. The first experiences were reported in 1980 and the first patient with severe idiopathic pulmonary arterial hypertension [IPAH; formerly primary pulmonary hypertension (PPH)] to receive long-term therapy was reported in 1984. After 25 years, prostanoids remain a mainstay in the treatment of these patients. Prostacyclin and its analogs (prostanoids) are potent vasodilators and possess antithrombotic, antiproliferative, and anti-inflammatory properties. Pulmonary hypertension is associated with vasoconstriction, thrombosis, and proliferation, and this may be partly due to a lack of endogenous prostacyclin secondary to prostacyclin synthase downregulation. This supports a strong rationale for prostanoid use as therapy for this disease.Der Gynäkologe 09/1992; 25(4):205-11. DOI:10.1007/978-0-387-87429-6_104