The frequency of a ‘double proximal axial triradius’ (DPAT), which results in a palmar parathenar pattern, has been reported to be increased in those with ventricular septal defect (VSD). We scored the presence of this pattern in 313 patients with congenital heart defects (including 86 with isolated VSD), 176 with cystic fibrosis (CF) or relatives of those with CF, and 333 with no known clinical disease who were unrelated to individuals with CF. The frequencies in these three groups were 1.6, 2.3, and 0.6%, respectively. None of those with isolated VSD defect had a DPAT. All five individuals in the heart defect group with a DPAT had pulmonic stenosis. Of the total of eleven individuals in our series with DPAT patterns, eight were affected only on the right hand. The frequency of DPAT in individuals with no known disease in this series was 4/406 = 1.0%, more than double the frequency in a control series, published previously by David, 5/1,129 = 0.4%. If the 73 relatives of those with CF are excluded from our own ‘control’ group, the frequency of DPAT in this group is then 0.6 %, closer to the results in David’s control series.

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