Glossary for Adult Congenital Heart Disease, V—V

VACTERL association

Describes a spectrum of defects including vertebral abnormalities, anal atresia, tracheo-esophageal fistula, radial dysplasia, renal abnormalities and congenital heart defects (atrial and ventricular septal defect, tetralogy of Fallot, truncus arteriosus, aortic coarctation, patent ductus arteriosus, etc.).

vascular ring

A wide spectrum of aortic arch anomalies including double aortic arch and other vascular structures that surround the trachea and the esophagus resulting in their compression. The vascular structures may or may not be patent. Vascular rings may be isolated (in 1% to 2% of CHD) or associated with other CHD malformations, such as  tetralogy of Fallot. See aortic arch anomalies.

velo-cardio-facial syndrome

Syndrome of cleft palate, abnormal facies (square nasal root, long nose with narrow alar base, long face with malar hypoplasia, long philtrum, thickened helix, low set ears), velopharyngeal incompetence and congenital cardiac defects (cono-truncal anomalies, isolated VSD, tetralogy of Fallot) due to microdeletion at chromosome 22q11. syn. Shprintzen syndrome. See also CATCH-22.

venous (or pulmonary) A-V valve

The A-V valve guarding the inlet to the venous, or pulmonary, ventricle.

ventricle repair:

• 1-ventricle repair

See Fontan procedure (operation).

• 1.5-ventricle repair (one and one-half ventricle repair)
  A term used to describe operations for cyanotic congenital heart disease performed when the pulmonary ventricle is insufficiently developed to accept the entire systemic venous return. A bi-directional cavo-pulmonary connection is constructed to divert superior vena cava flow directly to the lungs, while inferior vena cava flow is directed to the lungs via the functioning but small pulmonary ventricle.
• 2-ventricle repair
  A term used to describe operations for cyanotic congenital heart disease with common ventricle wherein functioning systemic and pulmonary ventricles are created by means of surgical septation of the common ventricle.

ventricular imbalance

In the setting of atrio-ventricular septal defect, ventricular imbalance refers to relative hypoplasia of one or the other of the ventricles in association with small size of the ipsilateral component of the atrio-ventricular annulus.

ventricular septal defect (VSD)

A defect in the ventricular septum, such that there is direct communication between the two ventricles.

• doubly-committed VSD
  A defect in the outlet septum such that there is fibrous continuity between the aortic and pulmonary valves, with the VSD situated directly beneath both semi-lunar valves.
• inlet VSD
  A defect in the lightly trabeculated inlet portion of the muscular interventricular septum, typically seen as part of an atrio-ventricular septal defect.
• muscular VSD
  A defect entirely surrounded by muscular interventricular septum.
• non-restrictive VSD
  A ventricular septal defect of such a size that there is no significant pressure gradient between the ventricles. Hence the pulmonary artery is exposed to systemic pressure unless there is RV outflow tract obstruction.
• outlet VSD
  A defect in the non-trabeculated outlet portion of the muscular interventricular septum, hence above the crista ventricularis. syn. supracristal VSD. Sometimes also described as subpulmonary, subarterial, or doubly committed subarterial VSD.
• perimembranous VSD

A VSD located in the membranous portion of the interventricular septum with variable extension into the contiguous portions of the inlet, trabecular, or outlet portions of the muscular septum, but not involving the atrio-ventricular septum. syn. membranous VSD; infracristal VSD.
• restrictive VSD
  A ventricular septal defect of small enough size that there is a pressure gradient between the ventricles, such that the pulmonary ventricle (hence pulmonary vasculature) is protected from the systemic pressure of the contralateral ventricle.
• trabecular VSD
  A defect in the heavily trabeculated central or trabecular portion of the muscular interventricular septum. May be multiple.

ventriculo-arterial concordance

See concordant ventriculo-arterial connections.

ventriculo-arterial discordance

See discordant ventriculo-arterial connections.