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Case #10300 - Heterotaxy (isomerisms and situs ambigus) with total anomalous pulmonary veins connection

Wednesday 22 November 2006

Synopsis

- female fetus, 22 weeks, 443g

- cardiac malformation

  • unique atrium
  • persistent right superior vena cava

- total anomalous pulmonary venous connection (TAPVC)

  • infraccardiac TAPVC: subdiaphragmatic venous confluent, propbably in portal system

- heterotaxy

  • visceral situs ambiguus
    • right spleen
    • left liver
    • right stomach (inverse rotation of the stomach)
  • visceral isomerism
    • pulmonary right isomerism

- medial liver
- right spleen
- right stomach (gastric inverse rotation)
- normal intestinal rotation
- normal mesentere
- right appendix
- normal colon with left sigmoid colon

See also

- situs anomalies
- heterotaxy
- visceral isomerism

  • pulmonary right isomerism (bilateral trilobated lungs)

- atrial appendage isomerism

References

- Peeters H, Devriendt K. Human laterality disorders. Eur J Med Genet. 2006 Sep-Oct;49(5):349-62. PMID: #16461029#

Portfolio

  • Medial liver, bilateral trilobar lungs (right pulmonary isomerism)
  • Right-sided stomach, right-sided spleen, medial liver
  • Normally situated heart
  • Persistent rignt superior vena cava
  • Total anomalous pulmonary venous connection (TAPVC)
  • Infracardiac total anomalous pulmonary venous connection (TAPVC)
  • Infracardiac total anomalous pulmonary venous connection (TAPVC)
  • Hypertelorism