Echocardiographic-morphologic correlations in tricuspid atresia

Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in two-dimensional echocardiograms.

Traditionally, tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle.

We examined 39 autopsy specimens catalogued as having tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of tricuspid atresia.

Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass.

Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.