A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease

Abstract

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.

Pediatric Cardiology and Cardiovascular Surgery: 1950-2000

“…Duncan and his colleagues have performed a histological analysis of pulmonary arteriovenous malformations in 2 children with cyanotic congenital heart disease. 139 Their study did not elucidate the role of the liver in the formation of pulmonary arteriovenous malformations. The histological correlate of pulmonary arteriovenous malformations seems to be greatly increased numbers of thin-walled vessels, but application of immunohistochemical techniques suggests that the rate of cellular proliferation is not increased.…”

“…This is not the forum to explore all these issues, but a few words about pulmonary arteriovenous malformations are germane. [131][132][133][134][135][136][137][138][139][140][141][142] The etiology for the development of pulmonary arteriovenous malformations after the Fontan operation remains uncertain. Clinical practice currently suggests that exclusion of the hepatic veins and hepatic venous effluent from the pulmonary circuit may be causal to the development of pulmonary arteriovenous malformations and that inclusion of the hepatic venous blood into the pulmonary circuit may reverse their formation.…”

Right atrium and superior vena cava pressure measurements in a novel animal model to study one and a half ventricle repair as compared to Fontan type procedure

“…28 The role of non-pulsatile blood flow in the development of pulmonary arteriovenous fistulae is more controversial. [29][30][31][32][33] Exclusion of hepatic venous return from the pulmonary circulation may be associated with the formation of such fistulae in patients following a classic Glenn-type cavopulmonary anastomosis. 33,34 We hypothesize that establishing a communication between the IVC and the pulmonary arteries may be important in preventing pulmonary arteriovenous malformations.…”

“…[29][30][31][32][33] Exclusion of hepatic venous return from the pulmonary circulation may be associated with the formation of such fistulae in patients following a classic Glenn-type cavopulmonary anastomosis. 33,34 We hypothesize that establishing a communication between the IVC and the pulmonary arteries may be important in preventing pulmonary arteriovenous malformations. [32][33] There is a potential disadvantage to this operative approach: the increased reverse flow to the SVC, but it has proved to be well tolerated by patients with Fontan procedure at midterm and long term follow-up1.…”

“…33,34 We hypothesize that establishing a communication between the IVC and the pulmonary arteries may be important in preventing pulmonary arteriovenous malformations. [32][33] There is a potential disadvantage to this operative approach: the increased reverse flow to the SVC, but it has proved to be well tolerated by patients with Fontan procedure at midterm and long term follow-up1. Our animal study showed in real time well tolerated hemodynamic changes with increased pressure at SVC level with normal RA pressure after a SVCbidirectional PA anastomosis, even after induced stress test.…”