Congenital Heart Malformations (CHM) can be present together with Extracardiac Malformations (ECM) in a single individual, however the frequency and the patterns of associations are not well defined because the casistics of the literature are little comparable. The diagnosis of CHM has been demonstrated with cardiac catheterization in any case. Between January 1st, 1976 and December 31st, 1983 cardiac catheterization has been performed in 1012 children and 971 of them were affected by Congenital there was at least one ECM as well (group B); 311 ECM were present in group B. VSD (p less than 0.05) and Atrioventricular Septal Defects (AV.SD) (p less than 0.01) resulted associated with ECM while T.F. (p less than 0.05) and isolated TGA (p less than 0.01) showed the tendency to occur in isolation. VDS resulted associated with gastrointestinal anomalies (p less than 0.05), AV.SD with T21 (p less than 0.001), aortic valve (p less than 0.05) and supra-valve (p less than 0.001) stenosis with Nervous System abnormalities. Pulmonary stenosis showed little tendency to occur together with T21 (p less than 0.05).
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