Heterotaxy syndrome (dextrocardia, atrioventricular discordance, pulmonary atresia)
Diagnosis certain
Presentation
Cyanosis since birth
Patient Data
Age: 10 months
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- dextrocardia (morphologic right atrium on the right, morphologic left atrium on the left, morphologic right ventricle with moderator band on the left and morphologic left ventricle on the right)
- atrioventricular discordance (morphologic right atrium empties into the morphologic left ventricle while the morphologic left atrium empties into the morphologic right ventricle)
- ventricular septal defect
- pulmonary atresia
- small confluent pulmonary arteries supplied by a patent ductus arteriosus and major aortopulmonary collateral arteries (MAPCA's)
- all pulmonary veins drain into the morphologic left atrium
- aorta arising from the morphologic right ventricle with left-sided arch and descending segment
- right-sided superior and inferior venae cavae drain into the morphologic right atrium
- right-sided liver
- left-sided stomach and spleen
Case Discussion
This is a case of heterotaxy syndrome with complex congenital heart disease.