Our Patient's Therapy and Additional Therapies

Our patient was placed on chronic intravenous prostacyclin, because she did not respond to short-acting vasodilators during catheterization. In addition, she was treated with an oral anticoagulant to prevent in situ thrombus in the pulmonary artery and to prolong survival. Diuretics were given for symptoms of right heart failure.

Other patients who are hypoxemic at rest or develop significant hypoxemia during limited activity, should be treated with oxygen. Exercise may improve symptoms.

Additional therapies for pulmonary hypertension are available. Endothelin receptor antagonists, such as bosentan, are oral therapies that improve exercise tolerance. They work by blocking endothelin, a growth factor that is increased in pulmonary hypertension. Prostacyclin analogues administered by other routes can be affected. These include treprostinil and iloprost. Phosphodiesterase-5 is an enzyme that reduced nitric oxide in the lung. Inhibitors, such as sildenafil, raise nitric oxide. Their long-term benefits are to be determined. Additional combinations of these drugs may be used.

Atrial septostomy has been used in patients with severe pulmonary arterial hypertension and intractable right heart failure who are on optimal medical therapy. A right-to-left shunt is created at the atrial level, unloading the right ventricle and improving the cardiac output.