Establishing an underlying cause and treatment plan for patients with pulmonary hypertension presents a significant challenge to practicing physicians. Doppler echocardiography is a simple, cost-effective tool for detecting pulmonary hypertension and evaluating right ventricular function. Nonspecific therapy (use of digoxin and diuretics, anticoagulation) for pulmonary hypertension and right ventricular failure achieves a degree of symptomatic improvement and should be considered in patients with moderate to severe disease. CTEPH should be considered in patients with dyspnea. Because severe forms of pulmonary hypertension usually are not discovered until late in the disease course, a high level of suspicion is required when evaluating symptoms and risk factors consistent with pulmonary vascular disease. Pulmonary hypertension is classified as idiopathic, or primary, when no secondary cause can be identified. Primary pulmonary hypertension is a devastating disease that largely affects young women. Significant advances in treatment have been made and will be discussed in detail in part 2 of this article.
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