Can primary pulmonary hypertension be treated effectively?

Until recently, primary pulmonary hypertension had been untreatable, progressing to death shortly after diagnosis. Investigators have defined a subset of patients who respond to large doses of calcium-channel blockers with a sustained decrease in mean pulmonary artery pressure, pulmonary vascular resistance and increase in cardiac output. Along with improvement in hemodynamic parameters, survival is prolonged markedly, right ventricular hypertrophy is reduced and functional status is improved. Patients who did not respond to high-dose calcium-channel blockers appeared to derive survival benefit from anticoagulation therapy. Determining response to calcium-channel blockers requires careful hemodynamic monitoring to avoid potentially adverse responses.