Drug treatment of primary pulmonary hypertension

Primary pulmonary hypertension is a rare but difficult-to-manage condition. Adequate clinical trials of agents used in its treatment have not been performed. A variety of drugs have been reported to provide benefit in patients with primary pulmonary hypertension, including beta-adrenoceptor agonists, alpha-adrenoceptor antagonists, and vasodilators such as isosorbide dinitrate, diazoxide, hydralazine, angiotensin converting enzyme inhibitors, prostaglandins and calcium antagonists. Calcium antagonists appear to offer the most promise, although treatment failures have occurred with them as well as with all other drugs used in this condition. In the absence of a specific treatment for primary pulmonary hypertension, several of the agents listed above should be tried before accepting therapeutic failure. Invasive investigation is necessary to adequately monitor the acute response to therapy. Whether combination therapy with 2 or more drugs might improve the response to treatment is an area worthy of further research.