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The role of angiogenesis in the development of pulmonary arteriovenous malformations in children after cavopulmonary anastomosis

Published online by Cambridge University Press:  19 August 2008

Blair Marshall ,
Brian W. Duncan  and
Richard A. Jonas
Blair Marshall
Affiliation:
Department of Surgical Research, Children's Hospital, 300 Longwood Avenue, Boston MA, USA
Brian W. Duncan
Affiliation:
Department of Cardiac Surgery, Children's Hospital, 300 Longwood Avenue, Boston MA, USA
Richard A. Jonas*
Affiliation:
Department of Cardiac Surgery, Children's Hospital, 300 Longwood Avenue, Boston MA, USA
*
Richard A. Jonas, MD, Department of Cardiac Surgery, Children's Hospital, Boston, MA 02115. Tel. (617) 355-7930, Fax. (617) 355-6742
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Abstract

Pulmonary arteriovenous malformations are a frequent cause of progressive cyanosis after construction of a cavopulmonary anastomosis. Their formation complicates the management of children with single ventricle physiology after a bidirectional Glenn shunt or the Kawashima procedure. The key role of the liver in this phenomenon is suggested by the observation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circulation limit further development of the malformations. In addition, it is known that patients with end-stage hepatic failure develop pulmonary arteriovenous malformations that diminish after liver transplantation. We have begun purification of a factor derived from hepatocyte-conditioned media that is inhibitory for the proliferation of cultured endothelial cells. This substance is heat sensitive, and binds avidly to a copper-containing chromatography column. These clinical observations, and this preliminary experimental work, support the concept that hepatic-derived angiogenic factors may play a role in the development of pulmonary arteriovenous malformations after construction of cavopulmonary anastomoses.

Keywords

Angiogenesispulmonary arteriovenous malformationscavopulmonary anastomosisbidirectional Glenn shuntFontan procedure
Type
Young Investigators Award
Information
Cardiology in the Young , Volume 7 , Issue 4 , October 1997 , pp. 370 - 374
Copyright
Copyright © Cambridge University Press 1997

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