total anomalous pulmonary venous connection

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)
    • asplenia

• 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

• anomalous systemic venous connections
  • bilateral superior vena cava

• anomalous pulmonary 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