Single ventricle lesions are congenital heart anomalies in which one of the 2 ventricular chambers is either absent or so severely hypoplastic that a biventricular repair is impossible. Typically, the atrioventricular valve associated with the absent or hypoplastic ventricle is also absent or hypoplastic. The arterial outflow valve associated with the absent/hypoplastic ventricle is also frequently affected. Single ventricle congenital heart defects cover a wide spectrum including the following: hypoplastic left heart syndrome, tricuspid atresia, unbalanced atrioventricular, pulmonary atresia with intact ventricular septum, Ebstein anomaly, and double outlet left ventricle. The repair of this entity involves 3-staged reconstruction to establish a circulatory system wherein all the deoxygenated blood flows directly into the lungs, bypassing the heart, while the oxygenated blood goes to the functional single ventricle to be pumped out to the systemic circulation. Stage 1 of this repair involves optimizing the pulmonary arterial blood flow, sometimes with a systemic-pulmonary arterial shunt or pulmonary artery band, and is performed shortly after birth. This procedure allows postoperative recovery and growth until stage 2 palliation can be performed. The stage 2 procedure, otherwise known as superior cavopulmonary connection, involves establishing a connection between the systemic venous return from the upper half of the body and the pulmonary arteries to allow deoxygenated blood flow into the pulmonary circulation. Some procedures in stage 2 are bidirectional Glenn, hemi-Fontan, and Kawashima operations. This procedure is typically performed within 6 months of birth. The primary goals of the imaging evaluation before the stage 2 operation include an assessment for obstruction in the pulmonary arteries and veins, the atrial septal defect, ventricular outflow tract, and aortic arch; for the degree and mechanism of valve regurgitation; and ventricular size and function.
Congenital or Acquired Heart Disease. Single ventricle lesions are congenital heart anomalies in which one of the 2 ventricular chambers is either absent or so severely hypoplastic that a biventricular repair is impossible. Typically, the atrioventricular valve associated with the absent or hypoplastic ventricle is also absent or hypoplastic. The arterial outflow valve associated with the absent/hypoplastic ventricle is also frequently affected. Single ventricle congenital heart defects cover a wide spectrum including the following: hypoplastic left heart syndrome, tricuspid atresia, unbalanced atrioventricular, pulmonary atresia with intact ventricular septum, Ebstein anomaly, and double outlet left ventricle. The repair of this entity involves 3-staged reconstruction to establish a circulatory system wherein all the deoxygenated blood flows directly into the lungs, bypassing the heart, while the oxygenated blood goes to the functional single ventricle to be pumped out to the systemic circulation. Stage 1 of this repair involves optimizing the pulmonary arterial blood flow, sometimes with a systemic-pulmonary arterial shunt or pulmonary artery band, and is performed shortly after birth. This procedure allows postoperative recovery and growth until stage 2 palliation can be performed. The stage 2 procedure, otherwise known as superior cavopulmonary connection, involves establishing a connection between the systemic venous return from the upper half of the body and the pulmonary arteries to allow deoxygenated blood flow into the pulmonary circulation. Some procedures in stage 2 are bidirectional Glenn, hemi-Fontan, and Kawashima operations. This procedure is typically performed within 6 months of birth. The primary goals of the imaging evaluation before the stage 2 operation include an assessment for obstruction in the pulmonary arteries and veins, the atrial septal defect, ventricular outflow tract, and aortic arch; for the degree and mechanism of valve regurgitation; and ventricular size and function.