Presentation
Newborn with cyanotic congenital heart disease. CT scan was requested to trace the drainage of the pulmonary veins.
Patient Data
Age: Neonate
Gender: Male
From the case:
Heterotaxy syndrome
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- hyperaerated lungs with stringy pulmonary vascular markings
- cardiac apex appears to be on the left
- right-sided gastric bubble
- ambiguous liver shadow
- umbilical artery catheter with its distal tip overlying the L1-L2 left hemivertebral disk space and umbilical vein catheter with its distal tip overlying T6-T7 mid hemivertebral disk space
From the case:
Heterotaxy syndrome
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- levocardia
- large atrioventricular septal defect
- severe pulmonary stenosis with small confluent pulmonary arteries
- infracardiac total anomalous pulmonary venous connection to the portal vein
- aorta and main pulmonary trunk arise from the morphologic right ventricle (double outlet right ventricle)
- major aortopulmonary collateral arteries (MAPCA's)
- bilateral trilobed lungs
- midline liver
- right-sided stomach
- asplenia
Case Discussion
This is a case of heterotaxy syndrome with complex congenital heart disease.
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