Presentation
18 months old cyanotic baby with complex congenital heart disease at echocardiography.
Patient Data
Age: 18 months
From the case:
Dextrocardia, DORV, VSD, persistent left SVC & PAPVR
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- Situs solitus with isolated dextrocardia.
- Atrio-ventricular disconcordance.
- 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.
- Cardiomegaly; predominantly right atrio-ventricular.
- No evidence of aortic coarctation.
- Confluent rather average calibered main pulmonary artery and its branches. No major aorto-pulmonary collaterals (MAPCAs) or peripheral pulmonary stenosis.
- A very small patent ductus arteriosus (PDA) is seen just distal to the left subclavian artery origin and coursing anteroinferiorly to join the main pulmonary artery bifurcation.
- Partial anomalous pulmonary venous return with the left superior pulmonary vein is seen draining via a persistent left superior vena cava. The rest of the pulmonary veins are draining into the left atrium.
- Normal both coronary arteries arising from their corresponding cusps.
- Normal hepatic veins and the inferior vena cava draining into the IVC.
Case Discussion
This is a case of complex congenital heart disease with Dextrocardia, DORV, VSD, persistent left SVC & partial anomalous pulmonary venous return (PAPVR).
Double outlet right ventricle (DORV) is a rare congenital cardiac anomaly where the aorta and the pulmonary artery arise from the right ventricle. VSD is invariably present.
The morhological right ventricle criteria are:
- Triangular configuration.
- Moderator band at its apex.
- Coarse trabecular pattern at its apical septum.
- Muscular band around its out ventricular tract with the its atrioventricular and ventriculo-arterial valves separated by muscle.
The septal surface of the morphological left ventricle is smooth.
The left atrial appendage is finger-like while the right atrial appendage is triangular.
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