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Figure 1. A 17-year-old male patient presented with severe pulmonary hypertension of uncertain cause (i.e., primary pulmonary hypertension). A chest x-ray film (Panel A) revealed massive dilatation of the central pulmonary arteries (open arrows) and right ventricular enlargement (solid arrows) that had not been present three years earlier (Panel B). There was no evidence of congenital heart disease. Pulmonary angiography showed no sign of chronic thromboembolic disease or veno-occlusive disease. The pulmonary arterial pressure was 106/58 mm Hg (mean, 76), with a mean right atrial pressure of 9 mm Hg, cardiac output of 5.4 liters per minute, and pulmonary . . . [Full Text of this Article] |