Congenital Heart Disease Glossary


Tissue or organ transplanted to a new site within the same individual.

azygos continuation of the inferior vena cava

An anomaly of systemic venous connections wherein the inferior vena cava is interrupted distal to its passage through the liver, and IVC flow reaches the right atrium through an enlarged azygos vein connecting the IVC to the superior vena cava. Usually, only hepatic venous flow reaches the right atrium from below. see also isomerism.

Baffes operation

Anastomosis of the right pulmonary veins to the RA and the IVC to the LA by using an allograft aortic tube to connect the IVC and the LA. (Baffes TG. A new method for surgical correction of transposition of the aorta and pulmonary artery. Surg Gynecol Obstet 1956; 102:227-233). This operation provided partial physiologic correction in patients with complete TGA. Lillehei and Varco originally described such a procedure in 1953. (Lillehei CW, Varco RL. Certain physiologic, pathologic, and surgical features of complete transposition of great vessels. Surgery 1953; 34:376-400.)

cervical aortic arch

The arch is located above the level of the clavicle.

cleft A-V valve

A defect often involving the left A-V valve in AVSD formed by the conjunction of the superior and inferior bridging leaflets. A cleft may also be seen in the septal tricuspid leaflet. A similar but morphogenetically distinct entity may involve the anterior or rarely posterior leaflet of the mitral valve in otherwise normal hearts.

common A-V valve

Describes a 5-leaflet A-V valve in complete AVSD that is related to both ventricles.

coronary sinus ASD

Inferior and anterior location at the anticipated site of the orifice of the coronary sinus. May be part of a complex anomaly including absence of the coronary sinus and a persistent left superior vena cava.

double aortic arch

Both right and left aortic arches are present, i.e. the ascending aorta splits into two limbs encircling the trachea and esophagus; the two limbs join to form a single descending aorta. There are several forms such as widely open right and left arches or hypoplasia/atresia of one arch (usually the left). This anomaly is commonly associated with patent ductus arteriosus. Double aortic arch creates a vascular ring around the trachea and the esophagus. see also vascular ring.

interrupted aortic arch

Complete discontinuation between the ascending and descending thoracic aorta.

  • Type A: Interruption distal to the subclavian artery that is ipsilateral to the second carotid artery.
  • Type B: Interruption between second carotid artery and ipsilateral subclavian artery.
  • Interruption between carotid arteries.
ostium primum ASD

Part of the spectrum of atrio-ventricular septal defect (AVSD). Located anterior and inferior to the oval fossa such that there is no atrial septal tissue between the lower edge of the defect and the atrioventricular valves that are located on the same plane; almost always associated with a "cleft" in the "anterior mitral leaflet". This cleft is actually the separation between the left-sided portions of the primitive antero-superior and postero-inferior bridging leaflets. see also AVSD.