PubMed · 15804486
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Byung-Boong Lee. 2005. Vascular malformations.. https://doi.org/10.1016/j.jamcollsurg.2004.12.008
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A 45-month-old boy who had complex cardiac anomalies with interrupted inferior vena cava and polysplenia underwent extracardiac Fontan operation. He redeveloped deep cyanosis postoperatively. Cardiac catheterization showed a preferential flow of the hepatic venous blood to the right lung and arteriovenous malformations (PAVMs) in the left lung. He underwent revision of the conduit to attain balanced hepatic venous drainage with subsequent regression of the PAVMs and disappearance of cyanosis. Our experience shows that deficiency of hepatic venous flow played a crucial role in the development of PAVMs and that redistribution of the flow retained potential to ameliorate the pathological condition.
Epithelioid hemangioma is an uncommon benign vascular lesion with distinct clinicopathological characteristics. Histologically, this entity mainly composed of well-formed but often immature vessels lined by epithelioid endothelial cells with prominent chronic inflammatory component. There is considerable controversy whether epithelioid hemangioma is a reactive lesion or a true neoplasm. We postulated that the local hypoxia may play a role in the pathogenesis of this vascular tumor. This local hypoxic condition may caused by many incidences such as congenital vascular malformation or trauma. The hypoxia will lead to the proliferation of endothelial cells and the formation of this vascular tumor, which may lead by putative stimulators VEGF and HIF-1. Hypoxia may also promote endothelial cell proliferation through the renin-angiotensin-aldosterone system. Additionally, the inflammatory cells including eosinophils and mast cells may contribute to the endothelial cell proliferation in EH. Further study investigating the associated factors of hypoxia may lead to new, potentially important insights into epithelioid hemangioma, and might also contribute to novel strategies for the management of this entity.
The development of unilateral pulmonary arteriovenous malformations in patients after total cavopulmonary connection with an extracardiac conduit has been reported. Unequal distribution of hepatic venous flow to the lung is theorized to be the causative factor. We report the surgical management of pulmonary arteriovenous malformations in a patient with heterotaxy syndrome, single ventricle, and interrupted inferior vena cava. The patient had previously undergone a total cavopulmonary connection with an extracardiac conduit draining hepatic venous flow to the right branch pulmonary artery. In the subsequent operation, we redirected the extracardiac conduit to the innominate vein. This operation provided the affected lung with hepatic venous blood without exposing the patient to the morbidity associated with cardiopulmonary bypass.