ABSTRACT: The treatment of pulmonary arterial hypertension (PAH) is directed at the underlying cause, such as diastolic heart failure or chronic thromboembolic disease. Patients with idiopathic PAH or PAH associated with connective-tissue disease who have World Health Organization (WHO) functional class II or III PAH should receive a trial of oral bosentan, ambrisentan, and/or sildenafil; inhaled iloprost is an alternative or an additive agent. If patients fail to respond to these interventions or if they have WHO functional class IV PAH, consider subcutaneous or intravenous treprostinil or epoprostenol. The use of these latter agents is much more complicated and may be difficult to initiate in elderly patients. (J Respir Dis. 2008;29(12):468-474)
Pulmonary arterial hypertension (PAH) is an increasingly recognized cause of shortness of breath in elderly persons. In the November 2008 issue of The Journal of Respiratory Diseases, we reviewed the clinical presentation and diagnostic evaluation of PAH. In this article, we will focus on treatment.
The traditional therapies for idiopathic PAH (IPAH) have included calcium channel blockers (CCBs), anticoagulants, diuretics, digoxin, and supplemental oxygen, but in the past several years, multiple treatments have emerged (Table). These therapies range from continuous intravenous medications, such as epoprostenol (an intravenous prostacyclin) and treprostinil (a subcutaneous or intravenous prostaglandin analogue), to oral or inhaled medications, including endothelin receptor antagonists (bosentan, ambrisentan), sildenafil (a phosphodiesterase type 5 inhibitor), and iloprost (an inhaled prostaglandin vasodilator). However, the approach to treatment is different in patients with diastolic heart failure and pulmonary hypertension (PH) (see below "Diastolic heart failure and pulmonary hypertension").
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