total anomalous pulmonary venous connection
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Synopsis
total anomalous pulmonary venous connection with obstruction
- +/- obstruction of the pulmonary veins or pulmonary hypertensive vascular changes on histology (49%)
Types
isolated TAPVD (58%) (7971585)
associated TAPVD (42%).(7971585)
- heterotaxy syndromes (asplenia-heterotaxy syndrome) (50%)
- visceral heterotaxy
- situs inversus and dextrocardia
- visceral situs inversus
- polysplenia
- asplenia/polyspenia syndrome (Ivemark syndrome)
- cardiac malformations
- single ventricle (33%) (17062215)
- pulmonary atresia (11%) (17062215)
- hypoplastic left heart syndrome
Classifiation of points of drainage of the anomalous pulmonary veins
obstructed total anomalous pulmonary venous connection
supracardiac anomalous pulmonary venous connection
cardiac anomalous pulmonary venous connection
infracardiac anomalous pulmonary venous connection
mixed anomalous pulmonary venous connection
Points of drainage of the anomalous pulmonary veins (pulmonary-systemic venous anastomoses) (7971585)
infradiaphragmatic veins (40%)
left innominate vein (25%)
coronary sinus (13%)
right superior vena cava (8%)
inferior vena cava above the diaphragm (4%)
right innominate vein (4%)
mixed left innominate vein and coronary sinus (2%)
azygos vein (2%)
mixed right superior vena cava and left hemiazygos vein (2%)
See also
development of the pulmonary vasculature
- development of the pulmonary veins
pulmonary venous obstruction
anomalous venous connections
References
James CL, Keeling JW, Smith NM, Byard RW. Total anomalous pulmonary venous drainage associated with fatal outcome in infancy and early childhood: an autopsy study of 52 cases. Pediatr Pathol. 1994 Jul-Aug;14(4):665-78. PMID: 7971585
Seo JW, Lee HJ, Choi JY, Choi YH, Lee JR. Pulmonary veins in total anomalous pulmonary venous connection with obstruction: demonstration using silicone rubber casts. Pediatr Pathol. 1991 Sep-Oct;11(5):711-20. PMID: 1745640