Abstract

Background. In hearts having the atriums connected only to a dominant left ventricle, typified by double-inlet left ventricle but seen also in lesions such as tricuspid atresia, subaortic obstruction, when it exists, is usually found at the level of the ventricular septal defect when the aorta is supported by the rudimentary right ventricle. Methods. Heart specimens were examined to determine the nature and position of the ventricular septal defect existing between dominant left and rudimentary right ventricles when the ventriculoarterial connections are discordant. Results. Most commonly, the ventricular septal defect is positioned between the muscular apical trabecular septum and the muscular outlet septum. This type of defect is found not only in double-inlet left ventricle, but also in hearts with absence of either the right or left atrioventricular connection when the other atrium is connected to a dominant left ventricle, irrespective of the position of the rudimentary and incomplete right ventricle. Obstructive lesions within the aortic arch are commonly associated with restriction at the site of the ventricular septal defect. The atrioventricular conduction bundle takes a constant course relative to the margin of the septal defect. Conclusions. Because subaortic obstruction is almost always caused by a restrictive ventricular septal defect, relief of the obstruction can be achieved by surgical enlargement of the septal defect, bearing in mind the course of the atrioventricular conduction system.

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