Primary pulmonary vascular sclerosis in the restricted sense of the term defined by Brenner1 is a rare condition. Up to 1935 Brenner collected from the literature only 15 cases, to which he added 1 of his own. This number, however, is probably incomplete; at least 1 other case should be included, the one reported by MacCallum.2
Since 1935 2 additional instances have been reported; including the case herein presented, there are now on record some 20 cases fulfilling the requirements for inclusion within this restricted group.
These requirements are (1) the presence of significant hypertrophy of the right ventricle but not of the left and (2) the absence of all factors commonly believed to cause secondary pulmonary vascular sclerosis, pulmonary hypertension or isolated strain of the right side of the heart.
REPORT OF A CASE
A married woman aged 21 entered the Multnomah County Hospital Feb. 2, 1940
BRILL IC, KRYGIER JJ. PRIMARY PULMONARY VASCULAR SCLEROSIS. Arch Intern Med (Chic). 1941;68(3):560-577. doi:10.1001/archinte.1941.00200090187008