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| 招請講演 |
A new era in medical management of severe pediatric pulmonary arterial hypertension |
| Dunbar Ivy Chief and Selby’s Chair of Pediatric CardiologyProfessor of Pediatrics, University of Colorado Denver Denver, USA |
| Pulmonary hypertension in children previously carried a very poor prognosis. In a 1965 series of 35 patients with idiopathic (primary) pulmonary arterial hypertension (IPAH), 22 patients died within one year of the onset of symptoms and none survived greater than 7 years. Although there is still no “cure” for PAH, recent advances in disease diagnosis and palliative therapy have resulted in significant improvements in clinical and hemodynamic status, as well as in survival. For example, in one 2004 series, children with severe IPAH treated with epoprostenol (n=35) at 1, 5, and 10 years was 94 percent, 81 percent, and 61 percent, respectively. Based on known mechanisms of action, three classes of drugs have been extensively studied for the treatment of PAH:prostanoids (epoprostenol, treprostinil, iloprost, beraprost), endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan), and phosphodiesterase inhibitors (sildenafil, tadalafil). Novel agents are being targeted to reverse remodeling of the pulmonary vasculature. |
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