Abstract

One hundred specimens from subjects with coarctation of the aorta were studied with regard to the nature and occurrence of additional cardiovascular anomalies. Seventy-seven were less than 6 months of age at the time of death (group I) and 23 were 6 months of age or older (group II). Exclusive of cases of bicuspid aortic valve, there were one or more associated anomalies in 87 cases, that is 91% of the patients under 6 months of age and in 74% of those in the older group. The various types of additional anomalies in order of decreasing frequency were as follows: (1) tubular hypoplasia of the aortic arch (49 cases); (2) abnormal communications (mainly ventricular septal defect and patent ductus arteriosus) (49 cases); (3) left ventricular outflow obstruction (mainly subaortic stenosis) (37 cases); (4) left ventricular inflow obstruction (26 cases); and (5) positional anomalies of the great vessels (mainly transpositions) (13 cases). More than one anomaly in addition to the coarctation was commonly present. The overall incidence of congenital bicuspid aortic valve was 46%. Among 71 cases in which the relationship to the ductus could be determined, the coarctation was proximal or opposite the ductus in 62 cases and distal to the ductus in nine cases.

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