Dextrocardia, DORV, Malposed great arteries, VSD, severe PS, TAPVR & Interrupted IVC with azygos continuation of the IVC
Diagnosis almost certain
Presentation
A 4 months old cyanotic baby with complex congenital heart disease at echocardiography.
Patient Data
Age: 4 months
Gender: Male
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- Situs solitus with isolated dextrocardia.
- Atrio-ventricular disconcordance with the right atrium is attached to a right-sided morphologically left ventricle and the left atrium is attached to a left-sided morphologically right ventricle.
- Great vessel relationship: Double outlet left-sided morphologically right ventricle (DORV) with malposed vessels: Aorta is anterior the main pulmonary artery (MPA).
- Large subaortic VSD.
- Left-sided aortic arch with normal three orderly branches.
- Cardiology.
- A small tubular tortuous PDA is seen between the proximal descending aorta and proximal LPA.
- No evidence of aortic coarctation.
- Confluent small-sized main pulmonary artery and its branches with severe valvular and subvalvular pulmonary stenosis. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
- Total anomalous pulmonary venous return with the pulmonary veins are seen draining into the right atrium.
- Interrupted IVC with prominent azygous vein draining into the SVC. Hepatic veins drain directly into the right atrium.