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An immunohistochemical study of arterial lesions due to pulmonary hypertension in patients with congenital heart defects

Published online by Cambridge University Press:  19 August 2008

Vera Demarchi Aiello*
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
Maria de Lourdes Higuchi
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
Edgard Augusto Lopes
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
Antonio Augusto Barbosa Lopes
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
Miguel Barbero-Marcial
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
Munir Ebaid
Affiliation:
From the Instituto do Coração do Hospital das Clinicas da FMUSP, São Paulo
*
Dr. Vera D.Aiello, INCOR HCFMUSP, Servico de Patologia, Avenida Dr. Enéas C. Aguiar, 44, 05403-000 São Paulo SP, Brazil. Tel. (011) 282-7766, Ext. 252; Fax. (011) 282-2354.

Abstract

In order to understand some of the cellular mechanisms of interaction in secondary pulmonary vaso-occlusive disease, we studied 21 lung biopsies from patients with different types of congenital cardiac defects. Their ages ranged from four to 248 months (mean 71.5 months; median 41 months). Changes in the cytoskeleton and extracellular matrix were assessed in the arterial wall. Immunostaining was applied to formalin-fixed, paraffin- embedded tissue, using antibodies to muscle-specific actin, vimentin and fibronectin in supra-optimal dilution. The staining for muscle-specific actin in the medial layer revealed a heterogenous pattern, with areas exhibiting low or absent labelling, reflecting a process of dedifferentiation of the smooth muscle cells in those segments. Within intimal proliferative lesions, the expression of muscle-specific actin was variable, being weak in some lesions and strong in those showing concentrically arranged intimal smooth muscle cells, suggesting a reversion of the migrated cells to the contractile phenotype. The endothelial cells of arteries from cases presenting severe qualitative lesions exhibited strong expression of vimentin, reflecting their heightened regenerative activity and/or their necessity to maintain their shape. The expression of fibronectin was greater in the predominantly cellular lesions of the intima when compared to the fibrotic lesions, indicating the role of that matrix glycoprotein in cellular migration and in replicative processes.

Type
World Forum for Pediatric Cardiology Young Investigator Prize
Copyright
Copyright © Cambridge University Press 1994

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