[Nosologic accuracy or semantic impasse?].

Neonatal cardiosurgery is nowadays an integral part of treatment of congenital heart disease. At present it is possible to correct completely with a feasible risk heart diseases in the majority of neonates (73%) with a critical congenital disease. Most frequently these disease defects are operated during the first two weeks of life (78% of all operations). In the strategy of surgery optimal timing and selection of the most suitable operation is essential to ensure in time favourable conditions for child growth and development and a long high-quality life. The tactics and therapeutic results in contemporary neonatal cardiosurgery are illustrated by the authors' experience with 174 neonates operated on for congenital critical heart defect in 1991-1993 in the Pediatric Cardiocentre, University Hospital Prague-Motol.

Heart Defects, Congenital↗

PubMed1995

Aspects of the aetiology of congenital heart disease.

A concise overview of current knowledge on the aetiology of congenital heart disease is provided. At present, only 10 to 20% of the cases occurring in neonates can be attributed to known risk factors. Recurrence within relatives, chromosomal anomalies, genetic disorders, maternal disease and teratogen exposure are addressed briefly; contemporary research models and methods, e.g. embryology and genetics and molecular biology, are referred to. A major innovation has been the introduction of the concept of common pathogenetic pathways. Thus, different teratogenic factors or risk-factors may affect normal development at an identical stage and cause similar malformations. Also, the importance of timing of an event is stressed. If the time frame of exposure does not coincide with embryogenesis any teratogenic effect may be missed. Large-scale epidemiological studies on fetuses and neonates with congenital heart disease are introduced as a third mode of research on the aetiology, although this approach is not used efficiently at present; cases of intra-uterine death can be considered a valuable source of information that needs further attention. Combined, the above three lines of research may prove productive, but the design of a comprehensive research project would need to be handled carefully. Possibilities for prevention of the occurrence of cardiovascular malformations are reported. Through lack of knowledge of causality, at present, only secondary prevention may be possible and hence deserves attention. However, there appears to be no provision for thorough pre-natal screening tests for congenital heart disease in an unselected population.

Heart Defects, Congenital↗