plexogenic pulmonary arteriopathy

A·yer·za syn·drome

sclerosis of the pulmonary arteries in chronic cor pulmonale; associated with severe cyanosis, it is a condition resembling polycythemia vera but resulting from primary pulmonary arteriosclerosis or primary pulmonary hypertension and characterized by plexiform lesions of arterioles.

plexogenic pulmonary arteriopathy

A vascular change seen in advanced pulmonary hypertension which is characterised by medial hypertrophy of muscular pulmonary arteries, intimal fibrosis, replacement of normal endothelium and fibrinoid necrosis.
Idiopathic; secondary to left-sided heart disease, lung disease, thrombosis, vasoconstriction of arteries (e.g., primary pulmonary hypertension), extravascular compression (e.g., due to disease of the chest wall), anorexiants (e.g., fenfluramine, possibly also phentermine).

plexogenic pulmonary arteriopathy

Pulmonary disease A vasculopathy seen in pulmonary HTN, characterized by medial hypertrophy of muscular pulmonary arteries, fibrinoid necrosis Etiology Anorexiants–eg, fenfluramine; possibly phentermine. See Fen-phen.


Abel, Argentinian physician, 1861-1918.
Ayerza-Arrillaga disease - Synonym(s): Ayerza syndrome
Ayerza disease - Synonym(s): Ayerza syndrome
Ayerza syndrome - sclerosis of the pulmonary arteries in chronic cor pulmonale. Synonym(s): Ayerza-Arrillaga disease; Ayerza disease; cardiopathia nigra; plexogenic pulmonary arteriopathy


giving rise to a plexus or plexiform growth.

plexogenic pulmonary arteriopathy
pulmonary vascular lesions caused by pulmonary hypertension and hyperfusion in congenital cardiac defects such as patent ductus arteriosus.
References in periodicals archive ?
However, from the pathology standpoint, 3 distinct pathologic patterns might be found in those patients: plexogenic pulmonary arteriopathy, recurrent pulmonary thromboembolic disease, and pulmonary veno-occlusive disease (PVOD).
56) The hallmark histopathologic lesion is plexogenic pulmonary arteriopathy.
3] Histopathologic examination of 36 patients undergoing heart-lung transplantation with plexogenic pulmonary arteriopathy revealed cholesterol granulomas in 9 cases.
9] In another series of 36 patients with plexogenic pulmonary arteriopathy who were undergoing combined heart-lung transplantation, cholesterol granulomas were found in 9 patients.