PRIMARY PULMONARY VASCULAR SCLEROSIS
In this rare condition there is sclerosis of the pulmonary arteries for no obvious reason (such as cardiac or pulmonary disease) as well as hypertrophy of the right but not the left side of the heart. Most of the patients die of heart failure. Hypertrophy of the right side of the heart, though not stressed in the definitions given in the literature, is particularly important. In the present series, 23 of 31 patients without ecardiac or pulmonary disease showed pulmonary vascular sclerosis, but circulatory symptoms and hypertrophy of the right heart were present in only 1 patient, the only one in whom a diagnosis of primary sclerosis was justified. In many cases reported in the literature chronic pulmonary or cardiac disease was present or hypertrophy of the right heart was absent, so that those cases must be considered as spurious. The question may be clarified
BRENNER O. PATHOLOGY OF THE VESSELS OF THE PULMONARY CIRCULATIONPART IV. Arch Intern Med (Chic). 1935;56(5):976–1014. doi:10.1001/archinte.1935.00170030144013
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