Congenital Heart Disease Glossary

ostium secundum ASD

Located at the level of the oval fossa.

overriding A-V valveoverriding A-V valve

Describes an A-V valve that empties into both ventricles. It overrides the interventricular septum above a VSD.

partial anomalous pulmonary venous connection (PAPVC)

One or more but not all the pulmonary veins connect to the right atrium directly, or via a vena cava. This anomaly is frequently associated with sinus venosus atrial septal defect. see also scimitar syndrome.

persistent 5th aortic arch

Double-lumen aortic arch with both lumina on the same side of the trachea. Degree of lumen patency varies from full patency of both lumina to complete atresia of one of them.

right aortic arch

In right aortic arch the descending thoracic aorta crosses the right mainstem bronchus. It is often associated with tetralogy of Fallot, pulmonary atresia, truncus arteriosus and other cono-truncal anomalies. Types of right aortic arch branching include:

  • Mirror image branching (left innominate artery, right carotid artery, right subclavian artery).
  • Retroesophageal left (aberrant) subclavian artery with a normal calibre. Sequence of branching: left carotid artery, right carotid artery, right subclavian artery, then left subclavian artery.
  • Retroesophageal diverticulum of Kommerell. see diverticulum of Kommerell.
  • Right aortic arch with left descending aorta i.e. retroesophageal segment of right aortic arch. The descending aortic arch crosses the midline toward the left by a retroesophageal route.
  • Isolation of contralateral arch vessels: an aortic arch vessel arises from the pulmonary artery via the ductus arteriosus without connection to the aorta. This anomaly is very uncommon. Isolation of the left subclavian artery is the most common form.
sinus venosus ASD

see sinus venosus defect.

straddling A-V valve

Describes an A-V valve with anomalous insertion of tendinous cords or papillary muscles into the contralateral ventricle (VSD required).

total anomalous pulmonary venous connection (TAPVC)

All pulmonary veins connect to the right side of the heart, either directly or via venous tributaries. The connection may be supradiaphragmatic, usually via a vertical vein to the innominate vein or the SVC. The connection may also be infradiaphragmatic via a descending vein to the portal vein, the IVC or one of its tributaries. Pulmonary venous obstruction is common in supradiaphragmatic connection, and almost universal in infradiaphragmatic connection.

Z-score, Z-value

A way of expressing a physiologic variable in a form corrected for age and body size. Important in pediatrics. This is the number of standard deviations a measurement departs from mean normal. (Rimoldi HJA, et al. A note on the concept of normality and abnormality in quantitation of pathologic findings in congenital heart disease. Pediatr Clin North Am 1963;10:589-591.) (Daubeney PEF et al. Relationship of the dimension of cardiac structures to body size: an echocardiographic study in normal infants and children. Cardiol Young 1999;9:402-410.)