Complex congenital heart disease: DORV, Malposed great arteries, VSD, PDA, PAPVR, Persistent left SVC & Interrupted IVC.
Diagnosis almost certain
Presentation
Cyanotic baby with congenital heart disease at echocardiography.
Patient Data
Age: 11 months
Gender: Male
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- Situs solitus with the apex to the left.
- Atrio-ventricular concordance.
- Great vessel relationship: Double outlet right ventricle (DORV) with malposed vessels: Aorta is anterior the main pulmonary artery (MPA).
- Large subaortic VSD.
- Right-sided aortic arch with normal three orderly branches.
- Cardiomegaly; predominantly right atrio-ventricular.
- A small tubular tortuous PDA is seen between the proximal descending aorta and proximal LPA.
- No evidence of aortic coarctation.
- Confluent good size main pulmonary artery and its branches. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
- Partial anomalous pulmonary venous return of the two right pulmonary veins that are seen draining into the right atrium. The two attenuated left sided pulmonary veins are draining into the left atrium.
- Double SVC. Right SVC opens into the right atrium and the persistent left SVC opens into the left atrium.
- Interrupted IVC with prominent hemi-azygous vein draining into the left SVC. Hepatic veins drain directly into the left atrium.
Case Discussion
Complex congenital heart disease: DORV, Malposed great arteries, VSD, PDA, PAPVR & persistent left SVC as well as interrupted IVC with prominent hemiazygous vein draining into the left SVC.
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