Glossary for Adult Congenital Heart Disease, T—T

TAPVC

Total anomalous pulmonary venous connection. See also anomalous pulmonary venous connection.

TAPVD

Total anomalous pulmonary venous drainage. A term sometimes used to refer to the entity properly called total anomalous pulmonary venous connection. See also anomalous pulmonary venous connection.

Taussig-Bing anomaly

A form of double outlet right ventricle in which the great arteries arise side-by-side with the aorta to the right of the pulmonary artery and the ventricular septal defect in a sub-pulmonary position. Since the LV empties across the VSD preferentially into the PA, the physiology simulates complete transposition of the great arteries with a VSD.

tetralogy of Fallot

A congenital anomaly, the primary pathophysiologic components of which are obstruction to right ventricular outflow at the infundibular level and a large non-restrictive VSD. The other two components of the "tetralogy" are an over-riding aorta and concentric right ventricular hypertrophy. Valvar RVOTO (pulmonic stenosis) and distal pulmonary artery stenosis are often present. The essential morphogenetic anomaly is malalignment of the infundibular (outlet) septum such that it fails to unite with the trabecular septum (hence the VSD) due to anterior deviation (hence the RV outflow tract obstruction). Lillehei first described the repair in 1955. (Lillehei CW et al. Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; reports of first ten cases. Ann Surg 1955;142:418-445.)

• pentalogy of Fallot

Tetralogy of Fallot with an associated ASD or PFO.
• pink tetralogy of Fallot

Tetralogy of Fallot presenting with increased pulmonary blood flow and minimal cyanosis because of a lesser degree of RVOT obstruction. syn. acyanotic Fallot.

Thebesian valve

A remnant of the right valve of the sinus venosus guarding the opening of the coronary sinus.

total anomalous pulmonary venous connection (drainage, return)

See anomalous pulmonary venous connection and total anomalous pulmonary venous connection.

trabecular VSD

See ventricular septal defect.

transannular

Crossing the annulus. In connection with the RV outflow tract in tetralogy of Fallot, the term refers to the pulmonary valve annulus, which often must be enlarged by a transannular patch, with consequent obligatory pulmonary insufficiency. Transannular patching was first described in 1959. (Kirklin JW et al. Surgical treatment for tetralogy of Fallot by open intracardiac repair. J Thorac Surg 1959;37:22-51.)

transposition of the great arteries

See discordant ventriculo-arterial connections.

• simple transposition of the great arteries

Discordant connection of the great arteries and ventricles such that the pulmonary trunk arises from the left ventricle and the aorta arises from the right ventricle, without any associated abnormality.
• complex transposition of the great arteries

Discordant connection of the great arteries and ventricles such that the pulmonary trunk arises from the left ventricle and the aorta arises from the right ventricle, with associated abnormalities, most commonly a ventricular septal defect.

tricuspid atresia

A congenital anomaly in which there is no physiologic or gross morphologic connection between the right atrium and right ventricle and there is an interatrial connection allowing mixing of systemic and pulmonary venous return at the atrial level. There is a variable degree of hypoplasia of the RV. The LV and mitral valve are normal.

truncus arteriosus

A single artery (truncus) arises from the base of the heart because of failure of proximal division into the aorta and the pulmonary artery. Thus, both pulmonary and systemic arteries as well as the coronary arteries arise from the common trunk. Truncus arteriosus is divided into two types depending on whether there is a VSD or an intact ventricular septum. syn. common arterial trunk.

Turner syndrome

A clinical syndrome due to the 45 XO karyotype in about 50% of cases, with 45XO/45XX mosaicism and other X chromosome abnormalities comprising the remainder. There is a characteristic but variable phenotype, and association with congenital cardiac anomalies, especially post-ductal coarctation of the aorta and other left-sided obstructive lesions, as well as partial anomalous pulmonary venous drainage without ASD. The female phenotype varies with the age of presentation, and is somewhat similar to that of Noonan syndrome.