Medical therapy of pulmonary hypertension. Conventional therapies

The 1-, 3-, and 5-year survival rates of the patients included in the National Institute of Health Registry on Primary Pulmonary Hypertension 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 calcium-channel blockers, prostacyclin, or even transplantation. To date, by far the most favorable results are reported with high-dose calcium-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 patients 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 patients with PAH. 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. Patients now are counseled more adequately regarding how to remain physically active while avoiding exercise-induced anginal pain or syncope. 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 hypoxia are now better appreciated. The indications of supplemental oxygen, inotropic agents, and diuretics have been refined based on improved pathophysiologic understanding. Most patients now benefit from anticoagulant therapy with coumarin 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 patients who benefit from long-term high-dose calcium-channel blocker therapy.