Author Affiliations: Division of Neonatology (Nelle M), Division of Pediatric Cardiology (Pavlovic M), Department of Cardiac Surgery (Carrel T), and Department of Obstetrics and Gynecology (Raio L, Surbek D, Meyer-Wittkopf M), University Hospital Berne, Switzerland

Corresponding Author: Mathias Nelle, MD, Division of Neonatology, University Children's Hospital Berne, Effingerstr. 102, CH 3010 Berne, Switzerland (Tel: 0041 (0) 31 6321401; Email: mathias.nelle@insel.ch)

Background: Newborns with hypoplastic left heart syndrome (HLHS) or right heart syndrome or other malformations with a single ventricle physiology and associated hypoplasia of the great arteries continue to be a challenge in terms of survival. The vast majority of these forms of congenital heart defects relate to abnormal morphogenesis during early intrauterine development and can be diagnosed accurately by fetal echocardiography. Early knowledge of these conditions not only permits a better understanding of the progression of these malformations but encourages some researchers to explore new minimally invasive therapeutic options with a view to early pre- and postnatal cardiac palliation.

Data sources: PubMed database was searched with terms of "congenital heart defects", "fetal echocardiography" and "neonatal cardiac surgery".

Results: At present, early prenatal detection has been applied for monitoring pregnancy to avoid intrauterine cardiac decompensation. In principle, the majority of congenital heart defects can be diagnosed by prenatal echocardiography and the detection rate is 85%-95% at tertiary perinatal centers. The majority, particularly of complex congenital lesions, show a steadily progressive course including subsequent secondary phenomena such as arrhythmias or myocardial insufficiency. So prenatal treatment of an abnormal fetus is an area of perinatal medicine that is undergoing a very dynamic development. Early postnatal treatment is established for some time, and prenatal intervention or palliation is at its best experimental stage in individual cases.

Conclusion: The upcoming expansion of fetal cardiac intervention to ameliorate critically progressive fetal lesions intensifies the need to address issues about the adequacy of technological assessment and patient selection as well as the morbidity of those who undergo these procedures.

Key words: congenital heart disease; fetal echocardiography; in utero cardiac palliation; postnatal intervention; prenatal diagnosis

                  World J Pediatr 2009;5(1):18-22