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The anatomy of hearts with double inlet ventricle

In the past, hearts with double inlet ventricle have been amongst the most contentious of congenital cardiac malformations. This is because, although most examples found with this particular atrioventricular connection have one big and one small chamber within the ventricular mass, for many years the variant most frequently encountered, with a dominant left ventricle, was usually described as exhibiting a single ventricle.1 With the recognition that, in this particular variant, the small chamber is an incomplete right ventricle, and is never capable of supporting independently the pulmonary circulation, the anatomic situation has now been clarified, as explained in the previous review,2 by recognising that the arrangement produces a functionally single ventricle, and that almost always patients with this lesion, if treated surgically, will be converted to the Fontan circulation. Even though, nonetheless, most patients with all variants of double inlet ventricle will likely end up with the Fontan circulation, it remains necessary to identify the functionally significant variants, namely those to be found in ventricular morphology, atrioventricular valvar morphology, ventriculo-arterial connections, and associated malformations.3