Right ventricle to pulmonary artery conduit in a tetralogy of Fallot patient with situs inversus totalis

Case contributed by Vincent Tatco


Diagnosed to have Tetralogy of Fallot, S/P placement of a right ventricle to pulmonary artery conduit

Patient Data

Age: 5 years
Gender: Female

The heart is enlarged. There is mirror-image dextrocardia with the cardiac apex on the right side. The morphologic right atrium is left-sided and is mildly dilated. The morphologic right ventricle is anterior and is hypertrophied. The morphologic left atrium is posterior in location. The morphologic left ventricle is right-sided. There is atrio-ventricular and ventriculo-arterial concordance. The right ventricle communicates with the left ventricle via a membranous subaortic ventricular septal defect. The interatrial septum is intact.

There is a retrosternal conduit connecting the morphologic right ventricle and pulmonary artery. It is well-opacified and patent without abnormal thrombus formation or narrowing. A metallic ring is identified around the conduit at the level of the ascending aorta causing minimal beam hardening artifact.

Another smaller conduit is also noted connecting the aortic arch and pulmonary artery. There is however a central filling defect suggestive of thrombus formation.

There is severe stenosis at the proximal left pulmonary artery.  There is branch of the right pulmonary artery which crosses the midline to supply the left lung.

The aorta is overriding the ventricular septal defect. There is a single trunk for the coronary arteries emanating from posteriorly located coronary sinus. It then branches into the right and left main coronary arteries. No coronary arteries emanate from the other two coronary sinuses.  The arch and descending segment of the aorta are right-sided.

There are 2 superior and 2 inferior pulmonary veins draining into the left atrium. The left-sided inferior vena cava and a single left-sided superior vena cava drain into the right atrium.

Sternotomy wires are demonstrated. 

The liver is left-sided while the stomach and spleen are seen in the right.

Case Discussion

This is a case of situs inversus totalis, tetralogy of Fallot with pulmonary atresia, status-post unifocalization of the pulmonary arteries and construction of a conduit from the right ventricle to the pulmonary arteries.  The severe proximal left pulmonary artery stenosis demonstrated in this examination underwent stenting prior to closure of the ventricular septal defect.

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