Portal de Periódicos da CAPES

THE PATHOLOGY OF PULMONARY ARTERY HYPERTENSION

2001; Elsevier BV; Volume: 22; Issue: 3 Linguagem: Inglês

10.1016/s0272-5231(05)70279-3

1557-8216

Barbara Meyrick,

Liver Disease and Transplantation

Pulmonary hypertension encompasses a number of disease entities that, clinically, are often difficult to distinguish from each other—that is, primary pulmonary hypertension (PPH), thromboembolic pulmonary hypertension, pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease and congenital heart disease. Such diseases are distinguished easily on histologic examination. This article focuses on the pathology and pathogenesis of PPH. The article falls into three main sections: the first concentrates on the structural changes found in the pulmonary arteries (PAs) of patients with PPH; the second highlights animal studies in models of chronic pulmonary hypertension, concentrating mainly on the hypoxic rat model and rats administered the alkaloid pyrrolizidine, monocrotaline; and the third examines the various cell types that contribute to the intimal and medial thickening in the larger PA with the onset of hypertension, with particular emphasis on the synthesis and metabolism of the potent vasoconstrictor, endothelin-1. For this latter section, the author focuses on a large animal model of chronic pulmonary hypertension developed at Vanderbilt, the sheep receiving continuous air embolization into the PA.