Congenital absence of the pulmonary valve associated with imperforate membrane type of tricuspid atresia, right ventricular tensor apparatus and intact ventricular septum: a curious developmental complex.

Abstract

We have presented the unique clinical and morphological features of 3 patients with an imperforate tricuspid valve and right ventricular tensor apparatus. Thus, despite valve tissue and apparatus, there was not a perforate atrioventricular connection. This most uncommon type of tricuspid atresia' was associated in all 3 cases with a congenitally absent pulmonary valve, an underdeveloped right ventricle, and a curious distortion of the ventricular septum. Indeed, 2 of these patients demonstrated severe disproportionate ventricular septal thickening, although histopathologic examination did not substantiate those features usually associated with a hypertrophic cardiomyopathy. Rather, microscopic examination revealed a sinusoidal malformation consisting of normal myocardial cells separated by branching ethothelial-lined channels which communicated with the right ventricular cavity. In addition, gross examination of these 3 specimens revealed an abnormally persistent right venous valve in 2, which subdivided the right atrium. Finally, these cases provide further evidence that the term 'tricuspid atresia' oversimplifies the observed morphological features.