A gene for familial total anomalous pulmonary venous return maps to chromosome 4p13-q12. Development of the pulmonary vein and the systemic venous sinus: an interactive 3D overview. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult (Wolters Kluwer Health/Lippincott Williams & Wilkins, Philadelphia, 2008). Nadas' Pediatric Cardiology (Saunders Elsevier, Philadelphia, 2006). Normal and abnormal development of pulmonary veins: state of the art and correlation with clinical entities. In normal development pulmonary veins are connected to the sinus venosus segment in the left atrium. Three-dimensional and molecular analysis of the arterial pole of the developing human heart. The development of the human pulmonary vein and its major variations. Total anomalous pulmonary venous connection. Partial anomalous pulmonary venous connections. Kalke, B.R., Carlson, R.G., Ferlic, R.M., Sellers, R.D. An anatomic survey of anomalous pulmonary veins: their clinical significance. Das Venensystem der Japaner 1st edn (Kenkyusha, Tokyo, 1933). Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Gross and histologic anatomy of total anomalous pulmonary venous connections. Congenital causes of pulmonary venous obstruction.
Drainage of the pulmonary veins into the right side of the heart. These results identify Sema3d as a crucial pulmonary venous patterning cue and provide experimental evidence for an alternate developmental model to explain abnormal pulmonary venous connections.īrody, H.
Sequencing of SEMA3D in individuals with anomalous pulmonary veins identified a phenylalanine-to-leucine substitution that adversely affects SEMA3D function. Normally, Sema3d provides a repulsive cue to endothelial cells in this area, establishing a boundary. In the absence of Sema3d, endothelial tubes form in a region that is normally avascular, resulting in aberrant connections. In these embryos, the maturing pulmonary venous plexus does not anastomose uniquely with the properly formed MES.
However, we found that TAPVC occurs in Sema3d mutant mice despite normal formation of the MES. Prevailing models suggest that TAPVC occurs when the midpharyngeal endothelial strand (MES), the precursor of the common pulmonary vein, does not form at the proper location on the dorsal surface of the embryonic common atrium 2, 3. Here we show that the secreted guidance molecule semaphorin 3d (Sema3d) is crucial for the normal patterning of pulmonary veins. In contrast to the extensive knowledge of arterial vascular patterning, little is known about the patterning of veins. Total anomalous pulmonary venous connection (TAPVC) is a potentially lethal congenital disorder that occurs when the pulmonary veins do not connect normally to the left atrium, allowing mixing of pulmonary and systemic blood 1.
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