2 to 5 yr after detection. Women are affected five times more often than men. Intimal hyperplasia and consequent narrowing of the vessel lumen are always present. Precordial pain and syncope on exertion are less common. Raynaud's phenomenon and arthralgias occur in many patients, often years before the apparent onset of primary pulmonary hypertension.
Physical examination shows, to a variable extent, the manifestations of cor pulmonale.
This scan pattern is not seen in primary pulmonary hypertension but suggests chronic thrombotic occlusion of pulmonary arteries due to unresolved pulmonary embolism, which can be helped in some cases by thromboendarterectomy. Pulmonary angioscopy may reveal chronic mural thrombi, even when arteriography is negative. The need for open lung biopsies is controversial. However, vasodilators should first be found effective in the cardiac catheterization laboratory. Injudicious use of these drugs has resulted in marked deterioration or fatality.