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: Pulmonary hypertension[Show abstract] [Hide abstract]
ABSTRACT: According to the last updated Dana Point clinical classification, pulmonary hypertension (PH) is divided into five categories . The categories are as follows. 1) An intrinsic pre-capillary pulmonary arteriopathy called pulmonary arterial hyper-tension (PAH) includes idiopathic PAH (IPAH) and familial/inherited PAH, as well as PH associated with connective tissue disease (CTD), congenital heart disease (CHD) with systemic-to-pulmonary shunts, portal hypertension, HIV and the use of drugs or anorexigens. 2) This category includes PH as a consequence of left heart disease and increased left heart filling pressures. 3) This category relates to primary pulmonary disease leading to capillary destruction and hypoxic pulmonary vasoconstriction. 4) This category comprises chronic thromboembolic pulmonary hypertension (CTEPH) and is a consequence of pulmonary embolism. 5) The last category is PH with unclear multifactorial mechanisms. For categories 2 and 3, treatment consists of minimising the cardiac or pulmonary anomalies by reducing left ventricular filling and afterload or by correcting hypoxaemia. For categories 1 and 4, specific therapeutic approaches have been developed and are the focus of the present chapter. These approaches can be very successful and, in some patients, may lead to an avoidance of the need for transplantation while in other patients they are insufficient to stabilise the disease and used only as a bridge to transplantation. Overall, the proportion of lung transplantation performed for IPAH has fallen fromy12% in the 1990s to 2% in 2006 . A number of patients initially listed for lung transplantation were removed from the lists as they improved following targeted medical therapy. Recently, however, there has been an evolution towards more aggressive therapeutic approaches. Therapeutic goals have been derived from prognostic factors, which have to be achieved over a relatively short period of time after diagnosis using monotherapy or combination drug therapies. If these goals are not achieved, transplantation should again be considered earlier. Pulmonary arterial hypertension Prognostic factors in pulmonary arterial hypertension In the pre-prostacyclin era, the median survival of untreated patients with IPAH was 2.8 yrs. However, some patients with much better survival could already be identified on the basis of favourable clinical indices. A low functional class (New York Heart association (NYHA) I and II) and better haemodynamics (right atrial pressure Eur Respir Mon, 2009, 45, 9–24. Printed in UK -all rights reserved. Copyright ERS Journals Ltd 2009; European Respiratory Monograph; ISSN 1025-448x.