Concordance for congenital heart disease in monozygotic twins is uncommon, but when it occurs, the specific defects are most often identical.
Low concordance informs genetic counseling in monozygotic twins; leaves open mechanisms of identical defects when present.
A pair of monozygotic twin girls is reported with concordance for 3 congenital cardiac abnormalities: (1) secundum atrial septal defect, (2) aneurysm of the membraneous ventricular septum, and (3) electrocardiographic frontal plane left axis deviation. A review of the published materials shows a 9.5 per cent incidence of concordance for congenital heart disease among monozygotic twins. In those in whom a precise cardiological diagnosis was made, 15/16 pairs (95%) were concordant for a specific defect, 2 had an additional defect, and only 1 pair had completely dissimilar defects. Concordance for congenital heart disease in monozygotic twins is uncommon, but when it occurs the defects will most often be identical.
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Seides et al. (1979) studied this question.
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