Presentation
Cyanosis since birth.
Patient Data
Age: 15 years
Gender: Female
From the case:
Heterotaxy syndrome
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- levopositioned heart with the cardiac apex directed towards the left
- large atrioventricular septal defect
- pulmonary atresia with small confluent pulmonary arteries
- all pulmonary veins drain into the left-sided atrium
- aorta arising from the morphologic right ventricle with left-sided arch and descending segment
- major aortopulmonary collateral arteries (MAPCA's)
- interrupted inferior vena cava with hemiazygos continuation
- hepatic veins and suprahepatic inferior vena cava draining into the left-sided atrium
- right-sided superior vena cava draining into the right-sided atrium
- smaller left-sided superior vena cava draining into the left-sided atrium
- bilateral hyparterial bronchi
- midline liver
- right-sided stomach and spleen
Case Discussion
This is a case of heterotaxy syndrome with complex congenital heart disease.
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