Background: This study was conducted to determine a morphologic spectrum of ventriculoarterial connection in double inlet left ventricle and implications for surgical procedures.
Methods: Examined were 54 autopsied heart specimens and 43 consecutive clinical patients.
Results: The hypoplastic and incomplete morphologic right ventricle was located leftward to the dominant ventricle in 62 and adjacent to the right atrium in 35. Common patterns were seen in 46 of 62 (74%) with the right ventricle leftward (discordant ventriculoarterial connection with the aorta left-anteriorly located ["SLL" type]) and in 28 of 35 (80%) with the right ventricle rightward (either the normally connected great arteries in 13 or discordant connections with the aorta right-anteriorly located in 15). In the remaining 23 hearts, the great arteries were unusually oriented in 7, the outlet septum was malaligned in 9, or the pulmonary trunk was atretic in 7. In those with malalignment, the ventriculoarterial connections were double outlet from the right ventricle, from the left ventricle, or were transitional with overriding of one of the great arteries. In the clinical series, 19 of 35 patients (54%) in whom the aorta arose from the morphologically right ventricle underwent either myectomy to enlarge the interventricular communication or a Damus-Kaye-Stansel anastomosis was fashioned to treat existing or potential subaortic stenosis. Only 1 of 8 patients with the aorta arising from the dominant ventricle needed similar surgical procedures.
Conclusions: Ventriculoarterial connection in double inlet left ventricle demonstrated a morphologic spectrum and needs precise recognition to provide an unobstructed ventricular outflow after operation.