Ventriculoarterial Malconnections


Anomalies of ventriculoarterial connection includes those lesions associated with abnormal connection between the ventricular mass and the great arteries. Lesions in this category include truncus arteriosus, tetralogy of Fallot, double outlet ventricles, and transposition of the great vessels.

To understand the morphology of ventriculoarterial malconnections, it is important to first understand the anatomy of the normal ventriculoarterial connection, which is dealt with separately.

A classification of ventriculoarterial malconnections includes:

Truncus Arteriosus
Tetralogy of Fallot
Tetralogy of Fallot and Pulmonary Stenosis
Tetralogy of Fallot and Pulmonary Atresia
Tetralogy of Fallot and Pulmonary Insufficiency
Double Outlet Right Ventricle
Transposition of the Great Arteries
d-Transposition of the Great Arteries
l-Transposition of the Great Arteries

A summary of the morphologic features of  ventriculoarterial malconnections is included in the following table:

Comparison of morphologic features of the major ventriculoarterial malconnections
  Truncus Arteriosus Tetralogy of Fallot DORV d-TGA
Subaortic conus -- -- ++ / - ++
Subpulmonary conus NA ++ + / - --
TC-PV distance distant distant highly variable fibrous continuity
AV-MV distance fibrous continuity fibrous continuity highly variable distant
LVOTO +/- rare +/- not uncommon
RVOTO +/- yes +/- rare
VSD subaortic subaortic variable subpulmonary