Cardiac Surgery by Gerhard Ziemer & Axel Haverich

Author:Gerhard Ziemer & Axel Haverich
Language: eng
Format: epub
Publisher: Springer Berlin Heidelberg, Berlin, Heidelberg

17.2 Extracardiac Malformations

Congenital cardiac malformations are frequently combined with extracardiac malformations. For hypoplastic left heart syndrome, rates reported are between 12 and 37 %.

While one post mortem study shows an overall malformation rate in hypoplastic left heart syndrome (HLHS) of 28 % (Natowicz et al. 1988), in another autopsy series, cerebral malformations alone were found in 29 % of patients. However, severe cerebral malformations like agenesis of the corpus callosum were seen only in 10 % (Glauser et al. 1990). In more than half of the cases in Natowicz’s series, genetic disorders were described either chromosomal anomalies or autosomal dominant or recessive lesions. Malformations of all organ systems are described. Among our own series of 233 patients with hypoplastic left heart syndrome, we saw one Goldenhar syndrome and one VACTERL syndrome. There was one case each of anal atresia, intestinal malrotation, growth retardation, and several kidney malformations. The overall malformation rate in our own series is 5.6 % (unpublished data). Glauser’s study also demonstrated that the risk for cerebral malformations is higher if hypoplastic left heart syndrome is part of a malformations syndrome. Among our own patients, there was one with agenesis of the corpus callosum.

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