plexogenic pulmonary arteriopathy

A·yer·za syn·drome

(ah-yār'shah),
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.
 
Aetiology
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.

Ayerza,

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

plexogenic

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).
Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome.
56) The hallmark histopathologic lesion is plexogenic pulmonary arteriopathy.
11] In a prospective study [12] of 507 patients with portal hypertension, prevalence of pulmonary hypertension was 2%, with the majority of patients having plexogenic pulmonary arteriopathy on biopsy or autopsy.
Of the reported cases of HIV-associated pulmonary hypertension with available pathology 78% (36 of 46) had plexogenic pulmonary arteriopathy.
Kim KK, Factor SM: Membranoproliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homo-sexual man with acquired immunodeficiency syndrome.
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.