Surgical anatomy of double-outlet right ventricle with situs solitus and atrioventricular concordance.

Sixty-three hearts with situs solitus, concordant atrioventricular connection, and more than half of both great arteries connected to the right ventricle have been studied to delineate their surgically important features. Specifically noted were the relationship of the arterial valves to each other and the type and position of the ventricular septal defect with special reference to the conduction tissue location. Infundibular morphology was ascertained and other congenital abnormalities were recorded. An estimate was made of the surgical options available given the various anatomic abnormalities present. The aorta was most commonly in the right posterior position but could be found anywhere in the arc extending to left anterior position. The septal defects were most frequently in subaortic position (34/65) and were usually of perimembranous type (45/65). Three hearts had two defects each and one heart had an intact ventricular septum. Identifying the type of defect was helpful in locating the conduction tissues. In addition, there were numerous associated anomalies, including 19 hearts with coronary artery malformations. Retrospective analysis of the surgical options suggested that 36% of the hearts would not have been operable during life. Approximately another third could have been corrected by use of an intraventricular tunnel from the ventricular septal defect to the aorta, and the remaining third would have needed more complex procedures. In the final analysis it was concluded that hearts with the ventriculoarterial connection of double-outlet right ventricle, though so varied as to preclude "standardization," did permit anatomic analysis and description in clear and consistent terms.