Single Ventricle

Single ventricle is a birth defect in which one lower chamber (ventricle) of the heart is abnormally small (hypoplastic). The other lower chamber (ventricle), which is normal sized, is referred to as the single ventricle. This defect is usually accompanied by a hole in the wall between the upper chambers (atrial septal defect or ASD). Single ventricle often has narrow or blocked pulmonary artery. There are many forms of single ventricle, resulting in different types of cardiac functional abnormalities.

This condition can be surgically corrected. First, when there is pulmonary artery blockage a modified Blalock-Taussig shunt can be placed during infancy . This shunt allows blood to flow from the innominate artery into the pulmonary artery and to the lungs. A bi-directional Glenn shunt is then performed later in the first year of life to suture the end of the superior vena cava to the right pulmonary artery. The modified Blalock-Taussig shunt is tied off Still later, a Fontan procedure is performed to connect the inferior vena cava to the right pulmonary artery. In this illustration, a graft directs blood from the inferior vena cava outside the heart (extra-cardiac) and then connects to the pulmonary artery. Blood flow to the lungs is then restored.


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