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Heterotaxy
- The heart is in the right side of chest.
- Stomach is on the left.
- There is functional single ventricle.
- There is a common AV valve with mild AV regurgitation.
- There is a large anterior vessel which comes off the heart. This continues as descending aorta. A ductus like vessel comes off the arch region and fills retrograde the posterior vessel, which appears to be pulmonary artery.
- There appears to be anomalous pulmonary venous return in a confluence behind the atrium.
- These findings are suggestive of functional single ventricle with common AV valve, possible pulmonary atresia with anterior aorta, and anomalous pulmonary venous return, consistent with right atrial isomerism.
Fetal echocardiographic evaluation of atrial morphology and the prediction of laterality in cases of heterotaxy syndromes. Berg C., Geipel A., Kohl T., Smrcek J., Germer U., Baschat A. A., Hansmann M. and Gembruch U. Ultrasound Obstet Gynecol 26(5):538-45 (2005)
Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience. Berg C., Geipel A., Smrcek J., Krapp M., Germer U., Kohl T., Gembruch U. and Baschat A. A. Ultrasound Obstet Gynecol 22(5):451-9 (2003)
Role of four-dimensional ultrasound (spatiotemporal image correlation and sonography-based automated volume count) in prenatal assessment of atrial morphology in cardiosplenic syndromes. Paladini D., Sglavo G., Masucci A., Pastore G. and Nappi C. Ultrasound Obstet Gynecol 38(3):337-43 (2011)
Heterotaxy
Left Isomerism
Right Isomersim
- Bilateral right atrial appendages
- AV septal defect, pulmonary atresia, transposition
- Anomalous pulmonary venous return
- IVC that is anterior or juxtaposed to the aorta
- Bilateral morphological right (trilobed) lungs
- Asplenia
- Bilateral left atrial appendages
- AV septal defect and pulmonary stenosis
- Congenital heart block
- Interrupted IVC with azygos continuation
- Bilateral left (bilobed) lungs
- Polysplenia
- More commonly detected postnatally
- Death in the first year of life is common, especially if there is pulmonary atresia and anomalous pulmonary venous return
- Those who survive cardiac palliation are at great risk of dying from sepsis
- Most commonly detected prenatally
- Cases involving AV canal with complete heart block carry a high risk for intrauterine heart failure and fetal demise.
- Prognosis in the neonatal period may be quite good in the absence of heart block for those babies that survive to birth
Normal
R sided
L sided
R sided
L sided