Uncommon Conal Pathology in Complete Dextrotransposition of the Great Arteries with Ventricular Septal Defect

¹ Servicio de Cardiología Pediátrica, Clínica Infantil La Paz, Madrid, Spain

The anatomic and clinical findings are reported in five cases of complete dextrotransposition of the great arteries with an uncommon and varied conal pathology, different from the usual conal architecture of classic complete dextrotransposition of the great arteries. The following points summarize the most important findings: (1) The ventricular septal defect was due to a positional anomaly or a deficiency of the conal septum in all of these cases. (2) The conal free wall distribution was different from that designated as characteristic of classic complete dextrotransposition of the great arteries, ie, in three cases, a persistence of the subpulmonary conal free wall preventing the pulmonary-mitral fibrous continuity. (3) A positional deviation of the conal septum inside the conus resulted in obstructive anomalies of the subpulmonary outflow tract in two cases and of the subaortic in another. (4) There was overriding of the conal septum of the ventricular septum in two cases. (5) In keeping with item 4, the observation of the pulmonary outflow tract of three additional cases of classic complete dextrotransposition of the great arteries with intact ventricular septum suggests a possible origin of the fibromuscular subpulmonary stenosis in these patients: the abnormal fusion line between the ventricular septum and a malorientated conal septum. Some important angiocardiographic characteristics of these cases are also shown. The surgical implications of these particular anatomic characteristics are discussed.

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