Congenitally bicuspid aortic valves: study of a variant with fenestrated raphe

To illustrate and describe a variant of the congenitally bicuspid aortic valve (BAV) in the context of previous reports of the morphology of BAVs.

Retrospective review of surgically excised aortic valves at The University of Ottawa Heart Institute in Ottawa, Ontario.

The clinical, echocardiographic and pathological features of 17 cases with a variant of BAV were examined from patients who had valve replacements performed in the period from January 1, 1986 to December 31, 1992.

A total of 865 patients had native aortic valve replacements during the study period and 291 patients had BAVs. One hundred and eighty-one patients were male (62%) (mean age 60 years) and 110 were female (38%) (mean age 64 years). There were 17 BAVs (6%) where either: the raphe was fenestrated so that only a thin fibrous band (or bands) joined the valve cusp to aortic wall-type A valves (seven cases); or a similar fibrous band (or bands) was seen, but was associated with other usual appearing raphal tissue--type B valves (10 cases). These patients included 15 males (mean age 63 years) and two females (mean age 63 years); they presented with dyspnea and/or angina. Distinction of the number of cusps with this variant was incorrect in six of nine transthoracic echocardiograms, and three of four transesophageal echocardiograms.

This variant of BAV, with fenestrated raphe, is not well described in the literature, is difficult to diagnose clinically and may present in unusual ways.