Congenital Heart Disease Glossary

A-V septal defect (AVSD)

see atrio-ventricular septal defect (AVSD).

A-V valve

see atrio-ventricular valve.

aberrant innominate artery

A rare abnormality associated with right aortic arch wherein the sequence of arteries arising from the aortic arch is: right carotid artery, right subclavian artery, then (left) innominate artery. The latter passes behind the esophagus. This is in contrast to the general rule that the first arch artery gives rise to the carotid artery contralateral to the side of the aortic arch (i.e.: right carotid artery in left aortic arch and left carotid artery in right aortic arch). syn. retro-esophageal innominate artery.

aberrant subclavian artery

The right subclavian artery arises from the aorta distal to the left subclavian artery. Left aortic arch with (retroesophageal) aberrant right subclavian artery is the most common aortic arch anomaly, first described 1735 by Hunauld, and occurring in 0.5% of the general population.

abnormal left aortic arch

Left aortic arch with minor branching anomalies; Left aortic arch with retroesophageal right subclavian artery.

absent pulmonary valve syndrome

Pulmonary valvular tissue is absent, resulting in pulmonary regurgitation. This rare anomaly uncommonly may be isolated; or it may be associated with ventricular septal defect, obstructed pulmonary valve annulus and massive dilation and distortion of the pulmonary arteries. Absent pulmonary valve may also occur in association with other simple or complex congenital heart lesions.


Adult Congenital Heart Disease

Alagille syndrome

see arteriohepatic dysplasia


Anomalous left coronary artery arising from the pulmonary artery. see Bland-White-Garland syndrome.


With reference to cardiac situs, neither right- nor left-sided (indeterminate). see situs.