PRIMARY PULMONARY HYPERTENSIONl

Alfred P. Fishman, Giuseppe G. Pietra
1980 · W2181921368
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Abstract

The designation pulmonary is generally used as a synonym for unexplained pulmonary (1, 2). Both clinicians and pathologists have adopted this term. Unfortunately, despite this appar­ ent concurrence, not all who use the term have in mind the same disorders, etiologies, pathogenetic mechanisms, or anatomic lesions. For the clinician, the syndrome of primary pulmonary hypertension has three essential features: (a) clinical, x-ray, and electrocardiographic evi­ dence of pulmonary hypertension, (b) abnormally high pulmonary arterial pressures and pulmonary vascular resistance in the face of a normal pulmo­ nary wedge pressure and a nearly normal cardiac output, and (c) insufficient cause for the pulmonary hypertension either in the lungs or heart (3). Since the advent of cardiac catheterization (4), the pathologist has partic­ ipated in the search for etiology by seeking to establish the extent and nature of the widespread pulmonary vascular disease, and by excluding cardiac or pulmonary causes of diffuse pulmonary vascular involvement. Indeed, re­ cently a few pathologists assumed the lead in attempting to sort out primary pulmonary hypertension when they proposed that a distinctive vascular lesion, i.e. plexogenic arteriopathy, is prerequisite for the diagnosis of primary pulmonary hypertension (1). But, as indicated below, this proposi­ tion has not been universally accepted (5-7).

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