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total anomalous pulmonary venous connection

Thursday 22 March 2007

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

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

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