[Hearts with double inlet ventricle. Morphological aspects in 20 cases]

The authors intended to verify the most common anatomical presentations of hearts with double inlet ventricle (DIV) and the associated lesions, in order to provide information regarding surgical palliation/correction.

Twenty hearts presenting DIV, from pathological collections. The sequential segmental analysis was applied.

Fourteen cases showed usual atrial arrangement, and six isomeric atria (4 with right isomerism and 2 with left isomerism). "Situs solitus" showed two atrioventricular (AV) valves, and the others a single valve. The main ventricle was morphologically left in 13 cases, right in one and of indeterminate morphology in six. The ventriculoarterial connexion was discordant in 10 cases, concordant in two, of double outlet type in eight (6 from an indeterminate ventricle, one from the main chamber and one from the rudimentary ventricle). Pulmonary obstructive lesions were common in the group with atrial isomerism (67%). AV valve stenosis was present in 4 cases, two of the left valve and two of the right one. Straddling of one AV valve was observed in 5 cases and aortic coarctation in three. The ventricular septal defect was restrictive in two cases, both with double inlet to a dominant left ventricle and a concordant AV connexion (Holmes'heart).

In those with atrial isomerism, the obstructive pulmonary lesions are the dominant feature and require early attention. For the other group, the atriopulmonary anastomosis procedures should take into account peculiarities such as AV valve dysfunctions and aortic coarctation.