[Pulmonary malformations associated with congenital cardiopathy]

The authors describe, in a series of 132 pediatric autopsies in the period January 1979-December 1994, the associations between congenital cardiopathies and pulmonary malformations. These latter were present in 13 of 46 cases (34.8%) in which cardiopathy was associated to a malformation of other discricts. In 9 of cases was exclusively interested the lung, while in the remaining 4 cases also other organs presented anomalies. The lung malformations observed were represented in 12 cases by supernumerary scissures with conjuncted cardiac septal system defects; in one case an alveolar dysplasia was associated to a previous Botallus duct. None of the 8 malformative syndromes, due to chromosomopathies, showed lung malformations. Examining the sequential chronological phases of the development of the two apparatus, the Authors hypothesize a common etiopathogenesis in both malformations, due to the isochronous action of the same intra-uterine pathogenic noxa, favoured by the topographic relationships of the two systems. Conversely, seems unlike the action of genetic damages, although either in genetically determined, or intra-uterine acquired, malformative syndromes, the pulmonary defects are almost exclusively represented by anomalies in the scissures formation.