The 1-, 3-, and 5-year survival rates of the @entity1 included in the National Institute of Health Registry on @entity120 were 77%, 41%, and 27% respectively. It is unclear to what extent better applied conventional therapy contributes to improved survival rates that now are reported with @entity460 -channel blockers, prostacyclin, or even transplantation. To date, by far the most favorable results are reported with high-dose @entity460 -channel blockers combined with anticoagulant therapy, with survival rates at 3 years approximately 100%. It has to be emphasized, however, that such exceptionally favorable results are to be expected only in a small minority of @entity1 who should not be considered to be cured because, sooner or later, their disease will continue to evolve. Further improvements obviously are needed for most @entity1 with @entity2556 . Interesting developments are likely in the coming years, with new multidrug approaches to control pulmonary vasoreactivity and remodeling and, hopefully, also with progress in lung transplantation. The past 2 decades have witnessed important progress in the treatment of PAH. Although significant improvements in quality of life and survival rate have been obtained with prostacyclin therapy, and better perspectives now are offered with atrial septostomy and lung transplantation, conventional therapy also has evolved. @entity1 now are counseled more adequately regarding how to remain physically active while avoiding exercise-induced @entity158 or @entity1163 . Invasive and potentially risky medical procedures have been restricted in favor of noninvasive and functional evaluations whenever possible. Risk factors such as appetite suppressants, pregnancy, and hypobaric @entity519 are now better appreciated. The indications of supplemental @entity26 , inotropic agents, and diuretics have been refined based on improved pathophysiologic understanding. Most @entity1 now benefit from anticoagulant therapy with @entity1524 derivatives, although some uncertainty remains about the optimal international normalized ratio to be achieved. Safer acute reversibility testing now is performed with fewer and shorter-acting agents that are more specific to the pulmonary circulation to select the small proportion of @entity1 who benefit from long-term high-dose @entity460 -channel blocker therapy.

Medical therapy of XXXX . Conventional therapies.

@entity1 :: ('9606', 'Species') :: ['Patients', 'patients'] @entity1524 :: ('MESH:C030123', 'Chemical') :: ['coumarin'] @entity26 :: ('MESH:D010100', 'Chemical') :: ['oxygen'] @entity120 :: ('MESH:D006976', 'Disease') :: ['Primary Pulmonary Hypertension'] @entity1163 :: ('MESH:D013575', 'Disease') :: ['syncope'] @entity2556 :: ('CHEBI:33848', 'Chemical') :: ['PAH'] @entity519 :: ('MESH:D000860', 'Disease') :: ['hypoxia'] @entity158 :: ('MESH:D010146', 'Disease') :: ['anginal pain'] @entity460 :: ('MESH:D002118', 'Chemical') :: ['calcium']
@entity120 :: (MESH:D006976,Disease) :: ['pulmonary hypertension']
