THE ROLE OF ANGIOGENESIS IN THE DEVELOPMENT OF PULMONARY ARTERIOVENOUS-MALFORMATIONS IN CHILDREN AFTER CAVOPULMONARY ANASTOMOSIS

Pulmonary arteriovenous malformations are a frequent cause of progress ive 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 procedu re. The key role of the liver in this phenomenon is suggested by the o bservation that providing modifications of the Fontan procedure which permit hepatic venous effluent to reach the pulmonary arterial circula tion limit further development of the malformations. In addition, it i s 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-condi tioned media that is inhibitory for the proliferation of cultured endo thelial 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 hepa tic-derived angiogenic factors may play a role in the development of p ulmonary arteriovenous malformations after construction of cavopulmona ry anastomoses.