Faculty Collaboration Database

A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease. J Thorac Cardiovasc Surg 1999 May;117(5):931-8

Date

04/30/1999

Pubmed ID

10220688

DOI

10.1016/S0022-5223(99)70374-0

Scopus ID

2-s2.0-0032924143 (requires institutional sign-in at Scopus site)   45 Citations

Abstract

INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent.

METHODS: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system.

RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). Immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium.

CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. Immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.

Author List

Duncan BW, Kneebone JM, Chi EY, Hraska V, Isik FF, Rosenthal GL, Jones TK, Starnes SL, Lupinetti FM

Author

Viktor Hraska MD Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Anastomosis, Surgical
Angiography
Arteriovenous Malformations
Biopsy
Capillaries
Child
Child, Preschool
Cyanosis
Female
Follow-Up Studies
Heart Atria
Heart Defects, Congenital
Hepatic Veins
Humans
Lung
Male
Pulmonary Artery
Pulmonary Veins
Vena Cava, Superior